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Systemic Lupus Erythematosus
I.
Lupus Eritematoso Sistémico I.
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****** DATA-MÉDICOS **********
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LUPUS ERITEMATOSO SISTÉMICO I
SYSTEMIC LUPUS ERYTHEMATOSUS
I
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****** DERMAGIC-EXPRESS No.50 *******
****** 07 MAYO DE 1.999 ***********
07 MAY 1.999
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EDITORIAL ESPAÑOL:
=====================
Hola amigos de la RED,, DERMAGIC una vez mas con ustedes, el tema: EL LUPUS ERITEMATOSO
SISTÉMICO I
Temida enfermedad y TODO un reto para tratar. Sigo en esta onda de enfermedades conectivas con motivo de la
Reunión del 22 de Mayo en Valencia cuyo tema central serán estas enfermedades,,
Encontré 90 interesantes referencias al respecto que enviare en 2 correos para no hacerlo tan pesado.
Dr. Carlos Fachin (Valencia, Venezuela) Bienvenido a Dermagic...
Nos volveremos a ver en la red...
Dr. José Lapenta R.,,,
EDITORIAL ENGLISH:
=====================
Hello friends of the NET, DERMAGIC once again with you. The topic: SYSTEMIC LUPUS ERYTHEMATOSUS
I.
Feared illness and an entire challenge to try. I continue in this wave of connective tissue diseases with reason of the Meeting of May 22 in Valencia (Venezuela), whose central topic will be these illnesses, I Found 90 interesting references in this respect, that I will send in 2 mail for not making it so heavy.
Dr. Carlos Fachin (Valencia, Venezuela) welcome to Dermagic...
We will see in the net again...
Greetings to ALL, !!
Dr. José Lapenta R.,,,
================================================================
DERMAGIC/EXPRESS(50)
================================================================
LUPUS
ERITEMATOSSO SISTEMICO I /
SYSTEMIC LUPUS ERYTHEMATOSUS I
================================================================
1.) [A case of lupus myocarditis and nephritis with transient foramen
jugular syndrome]
2.) [Two cases with SLE and MCTD developed after a long period of chronic
arthritis that was initially diagnosed as JRA]
3.) An increased prevalence of Epstein-Barr virus infection in young
patients suggests a possible etiology for systemic lupus erythematosus.
4.) [Newer approach of screening test for antinuclear antibodies: an
enzyme-linked immunosorbent assay detecting antinuclear antibodies
characteristic of connective tissue diseases]
5.) Development of systemic lupus erythematosus in a rheumatoid arthritis
patient with anti-ribosomal P protein antibody.
6.) Elevated soluble fas production in SLE correlates with HLA status not with disease activity.
7.) Lack of NK cells in lupus patients with renal involvement.
8.) Systemic lupus erythematosus in India.
9.) Systemic lupus erythematosus (SLE) lymphadenopathy presenting with
histopathologic features of Castleman' disease: a clinicopathologic study
of five cases.
10.) [A case of systemic lupus erythematosus associated with minimal change
nephrotic syndrome]
11.) T cell receptor clonotypes in skin lesions from patients with systemic
lupus erythematosus.
12.) Circulating plasma levels of nucleosomes in patients with systemic
lupus erythematosus: correlation with serum antinucleosome antibody titers
and absence of clear association with disease activity.
13.) Autoantibodies to human recombinant erythropoietin in patients with
systemic lupus erythematosus: correlation with anemia.
14.) A promoter polymorphism of tumor necrosis factor alpha associated with
systemic lupus erythematosus in African-Americans.
15.) Histone-specific Th0 and Th1 clones derived from systemic lupus
erythematosus patients induce double-stranded DNA antibody production.
16.) Hepatitis C virus antibodies in systemic lupus erythematosus.
17.) Cutaneous lupus mucinosis: a review of our cases and the possible
pathogenesis.
18.) The immunofluorescent profile of dermatomyositis: a comparative study
with lupus erythematosus.
19.) [A man with systemic lupus erythematosus presenting with spastic
paraplegia]
20.) Cyclosporine and therapeutic plasma exchange in treatment of
progressive autoimmune diseases.
21.) [The incidence of the antiphospholipid syndrome in the clinical and
22.) Emotional and physical intimacy in coping with lupus: women's dilemmas
of disclosure and approach.
23.) [A study on interval of intravenous cyclophosphamide pulse in the
treatment of severe systemic lupus erythematosus]
24.) Autonomic nervous dysfunction in systemic lupus erythematosus (SLE) and
rheumatoid arthritis (RA): possible pathogenic role of autoantibodies to
autonomic nervous structures.
25.) [Cyclophosphamide therapy in systemic lupus erythematosus]
26.) Pulmonary involvement in systemic lupus erythematosus.
27.) Renal vein thrombosis in Chinese patients with systemic lupus
erythematosus.
28.) Usefulness of antinuclear antibody testing to screen for rheumatic
diseases.
29.) [Human parvovirus B19 infection mimicking systemic lupus erythematosus:
case report]
30.) SLE and Sjogren's syndrome associated with unilateral moyamoya vessels in cerebral arteries.
31.) SLE with death from acute massive pulmonary hemorrhage caused by
disseminated strongyloidiasis.
32.) A fatal case of severe SLE complicated by invasive aspergillosis.
33.) Methotrexate use in miscellaneous inflammatory diseases.
34.) Renal vascular lesions in lupus nephritis.
35.) Antibodies to C1q in systemic lupus erythematosus: characteristics and
relation to Fc gamma RIIA alleles.
36.) Heredity and systemic lupus erythematosus: dissecting a complex genetic
37.) Massive uncomplicated vascular immune complex deposits in the kidney of a patient with systemic lupus erythematosus.
38.) [Oral manifestations in patients with systemic lupus erythematosus]
39.) Vasculitis and bacteraemia with Yersinia enterocolitica in late-onset
systemic lupus erythematosus.
40.) Neoral--new cyclosporin for old?
41.) Successful therapy with danazol in refractory autoimmune
thrombocytopenia associated with rheumatic diseases.
42.) Predisposing factors in sulphasalazine-induced systemic lupus
erythematosus.
43.) Clinical features of lupus myositis versus idiopathic myositis: a
review of 30 cases.
44.) Serological characteristics of systemic lupus erythematosus from a
hospital-based rheumatology clinic in Kuwait.
45.) Elevated anticardiolipin antibodies in autoimmune haemolytic anaemia
irrespective of underlying systemic lupus erythematosus.
46.) A young woman with SLE: diagnostic and therapeutic challenges.
47.) Binding characteristics of SLE anti-DNA autoantibodies to modified DNA
analogues.
48.) Circulating interleukin-6 type cytokines in patients with systemic
lupus erythematosus.
49.) [Osteonecrosis in systemic lupus erythematosus. Report of 3 cases]
50.) Association of an insertion polymorphism of angiotensin-converting enzyme gene with the activity of systemic lupus erythematosus.
=====================================================================
=====================================================================
1.) [A case of lupus myocarditis and nephritis with transient foramen
jugular syndrome]
=====================================================================
AU: Kohro-Kawata-J; Nakamura-H; Yamamoto-T; Fukuta-S; Matsuzaki-M
SO: Ryumachi. 1997 Oct; 37(5): 709-13
AB: A 46-year-old man was admitted to our clinic because of acute heart
failure. Six years before admission he was pointed out cardiomegary and
hematuria. One year later, he was diagnosed as having jugular foramen
syndrome. On admission, he had a fever and dyspnea. Pansystolic blowing
murmur was audible at the apex. The chest ratio on his chest X-ray was
52.5%. An electrocardiogram showed left ventricular hypertrophy. An
echocardiogram showed marked dilatation and severe dysfunction of left
ventricle. Radionuclide scanning with technetium 99 m pyrophosphate
identified inflammatory change in the apex.
Myocardial biopsy showed
fibrotic degeneration and IgG deposits in myocardium. Blood examination
showed anemia, lymphopenia. positive anti-nuclear antibody (1000 times,
shaggy pattern), positive anti ds-DNA antibody and hypocomplementemia.
Furthermore, proteinuria was pointed out. Renal biopsy showed focal
segmental glomerulonephritis with active necrotizing lesion (type III
nephritis). Lupus myocarditis and nephritis was diagnosed. After
prednisolone (80 mg/day) was administered. left ventricular function and
hypocomplementemia improved. The ACE inhibitor was also used for
proteinuria. In spite of a little amount of blood transfusion, he showed
hepatic hemosiderosis. We suspect that the cause of hemosiderosis was
related chronic inflammation of active lupus. It was treated with
Erythropoietin.
=====================================================================
2.) [Two cases with SLE and MCTD developed after a long period of chronic
arthritis that was initially diagnosed as JRA]
=====================================================================
AU: Takei-S; Maeno-N; Shigemori-M; Nakae-Y; Mori-H; Nerome-Y; Imanaka-H;
Hokonohara-M; Miyata-K
SO: Ryumachi. 1997 Oct; 37(5): 702-8
AB: In order to discuss the diversity of clinical features and the
difficulty in diagnosis of children with juvenile rheumatoid arthritis
(JRA), we present two cases who have documented the development of systemic
lupus erythematosus (SLE) and mixed connective tissue disease (MCTD) after
a long period of disease characterized only by arthritis that was initially
diagnosed as JRA. The first case was a girl diagnosed for her arthritic
joints as polyarticular JRA at 15 years of age. At onset, she had Raynaud
phenomenon but autoantibodies such as anti-nuclear antibody (ANA), anti-DNA
antibody, and rheumatoid factor were negative. Five years after onset, she
became ANA positive and 3 years later she became pregnant.
During her
pregnancy, she became positive for anti-DNA antibody without any signs of
nephritis. One month after the delivery, however, she developed butterfly
rash, carditis, nephritis, and was diagnosed as SLE. No destructive changes
were observed in her joints though arthritis continued for 8 years form
onset to pregnancy. The second case was a 3 years old girl who was
diagnosed as polyarticular JRA.
Treatment by aspirin induced complate
remission after one year from the onset. However, 10 years after that
remission, she developed Raynaud phenomenon and arthralgia in her knees and
hip joints. Her laboratory findings showed hypergammaglobulinemia, positive
ANA, positive anti-DNA antibody, positive anti-RNP antibody. She was
eventually diagnosed as MCTD when she was found to have polymyositis by EMG
and serum CK. In the present paper, two cases imply the difficulty in
diagnosing JRA and diversity of rheumatic diseases such as JRA, SLE and
MCTD. Closer and longer period of observation is essential for the JRA
patients with nondestructive arthritis.
=====================================================================
3.) An increased prevalence of Epstein-Barr virus infection in young
patients suggests a possible etiology for systemic lupus erythematosus.
=====================================================================
AU: James-JA; Kaufman-KM; Farris-AD; Taylor-Albert-E; Lehman-TJ; Harley-JB
SO: J-Clin-Invest. 1997 Dec 15; 100(12): 3019-26
AB: An unknown environmental agent has been suspected to induce systemic
lupus erythematosus (lupus) in man. Prompted by our recent immunochemical
findings, we sought evidence for an association between Epstein-Barr virus
infection and lupus. Because the vast majority of adults have been infected
with Epstein-Barr virus, we chose to study children and young adults.
Virtually all (116 of 117, or 99%) of these young patients had
seroconverted against Epstein-Barr virus, as compared with only 70% (107 of
153) of their controls (odds ratio 49.9, 95% confidence interval 9.3-1025,
P < 0. 00000000001).
The difference in the rate of Epstein-Barr virus
seroconversion could not be explained by serum IgG level or by
cross-reacting anti-Sm/nRNP autoantibodies. No similar difference was found
in the seroconversion rates against four other herpes viruses. An assay for
Epstein-Barr viral DNA in peripheral blood lymphocytes established
Epstein-Barr virus infection in the peripheral blood of all 32 of the lupus
patients tested, while only 23 of the 32 matched controls were infected
(odds ratio > 10, 95% confidence interval 2.53-infinity, P < 0.002). When
considered with other evidence supporting a relationship between
Epstein-Barr virus and lupus, these data are consistent with, but do not in
themselves establish, Epstein-Barr virus infection as an etiologic factor
in lupus.
=====================================================================
4.) [Newer approach of screening test for antinuclear antibodies: an
enzyme-linked immunosorbent assay detecting antinuclear antibodies
characteristic of connective tissue diseases]
=====================================================================
AU: Asanuma-H; Miyake-J; Miyawaki-S
SO: Nihon-Rinsho-Meneki-Gakkai-Kaishi. 1997 Oct; 20(5): 417-27
AB: An enzyme-linked immunosorbent assay (ELISA) has been developed for the
detection of antinuclear antibodies (ANAs) previously established as
diagnostic and/or prognostic marker ANAs for various connective tissue
diseases. The antigen used in ELISA is a mixture of purified recombinant or
natural antigens including single-and double-stranded DNA, RNP, Sm,
SS-A/Ro, SS-B/La, centromere, topoisomerase I and Jo-1 antigens. Thirty
hundred and fifty nine patients sera from a variety of connective tissue
diseases and 113 normal human sera (NHS) were examined. ELISA ANAs were
positive in 3.5% of NHS and 80.2% of patients sera at cut off index 11.5,
whereas indirect immunofluorescent antinuclear antibodies (FANAs) using
HEp-2 cells were positive in 9.7% of NHS and 92.5% of patients sera at
1:160 serum dilution.
More than 80% of sera from systemic lupus
erythematosus, mixed connective tissue disease and primary Sjogrens disease
were ELISA ANAs positive. Mean value of ELISA ANAs was highest in sera of
patients with MCTD. ELISA ANAs were positive in 92.5% of sera with marker
ANAs for connective tissue diseases. Mean value of ELISA ANAs was higher in
sera with more than two marker ANAs than in sera with a single ANA or in
sera without marker ANAs. In contrast incidence and mean value of ELISA
ANAs were low in sera positive for anti topoisomerase I antibody or anti
Jo-1 antibody.
Sensitivity, specificity and agreement (accuracy) for
connective tissue diseases with marker ANAs were as follows: ELISA ANAs (at
index 11.5): 92.5%, 88.3% and 90.9%: FANAs (at 1:160 serum dilution):
99.0%, 70.4% and 88.1%, respectively. ELISA ANAs, thus, are specific for
connective tissue diseases when compared to FANAs and previous ELISA for
the detection of total ANAs. Moreover, ELISA ANAs are able to measure
precise ANAs titers and are much less labor intensive when screening a
large number of clinical specimens.
=====================================================================
5.) Development of systemic lupus erythematosus in a rheumatoid arthritis
patient with anti-ribosomal P protein antibody.
=====================================================================
AU: Hamasaki-K; Mimura-T; Kanda-H; Morino-N; Yazaki-Y; Nojima-Y
SO: Lupus. 1997; 6(9): 734-6
AB: We describe a patient with rheumatoid arthritis (RA) whose sera
contained a high titre of an antibody targeting cytoplasmic ribosomal P
proteins (anti-P). This association preceded by 6 years the development of
serological and clinical manifestations of systemic lupus erythematosus
(SLE). The clinical significance of anti-P for the diagnosis of SLE is
discussed.
=====================================================================
6.) Elevated soluble fas production in SLE correlates with HLA status not with disease activity.
=====================================================================
AU: Rose-LM; Latchman-DS; Isenberg-DA
SO: Lupus. 1997; 6(9): 717-22
AB: Evidence from animal models of lupus suggests that disruption of
Fas-mediated apoptotic events may play a role in systemic lupus
erythematosus (SLE). The recently described secreted from of Fas (sFas)
could interfere with apoptotic events by blockading Fas/Fas ligand
interactions. We describe elevated secreted Fas protein in sera from 60
patients with SLE compared with controls but neither sFas protein nor sFas
mRNA levels correlated with disease activity. At the mRNA level there is
strong evidence that individuals with human leucocyte antigens common in
SLE patients have a genetic predisposition for increased secreted Fas
production.
=====================================================================
7.) Lack of NK cells in lupus patients with renal involvement.
=====================================================================
AU: Erkeller-Yuksel-FM; Lydyard-PM; Isenberg-DA
SO: Lupus. 1997; 6(9): 708-12
AB: We have previously shown that patients with SLE have significantly
lower percentages and absolute numbers of NK(CD3-/CD16+56) cells in their
peripheral blood compared with normals. Patients with active disease had
very low levels of NK cells and the reduction was also associated with
patients who had renal involvement. We have now performed a serial study
immunophenotyping 11 patients with SLE and renal involvement using dual
colour immunofluorescence and flow cytometry.
Patients were tested every
three months on an average of three occasions. As a control, nine SLE
patients without renal involvement were immunophenotyped for similar
intervals; 11 normal controls were also tested. Major lymphocyte subsets
(T, B and NK) remained very stable during serial bleeds. However, the NK
cell populations were decreased significantly in patients with renal
involvement both as percentages (5 +/- 6 vs 9 +/- 5, P < 0.0001) and
absolute counts (75 +/- 108 vs 109 +/- 52, P < 0.001) in comparison to
non-renal patients. Analysis of disease activity using BILAG score showed
an inverse correlation between renal system activity and percentage and
absolute number of NK cells (P < 0.002 and 0.01, respectively). In this
study we have also analysed a CD8 T cell subset which we have not studied
before.
We have found a significantly increased percentage of CD38+CD8+ T
cells(activated CD8 subset) in patients with SLE in comparison to normal
controls. We did not find any association with the CD38+CD8+ T cells and
disease activity as measured by BILAG or renal involvement. NK cells are
important factors in immunity against virus infections and tumour cells.
CD38+CD8+T cells are increased in viral infections. We speculate that the
lack of NK cells in SLE patients might have an association with increased
CD38 expression.
=====================================================================
8.) Systemic lupus erythematosus in India.
=====================================================================
AU: Malaviya-AN; Chandrasekaran-AN; Kumar-A; Shamar-PN
SO: Lupus. 1997; 6(9): 690-700
AB: The first case of systemic lupus erythematosus (SLE) was reported from
India in 1995 followed by two more case reports and further, a series of
eight cases, till 1969. Since the establishment of a clinical immunology
laboratory at a major teaching institution in New Delhi in 1968, SLE was
extensively studied and reported from that centre. From mid-1980 onwards
several other centres in different regions in India including Chennai (old
name Madras), Mumbai (old name Bombay), Calcutta and Hydrabad, also
published their regional experience on SLE.
Based on these data, the
present report describes the clinical and laboratory characteristics of
1366 SLE patients seen in different regions of India. Arthritis, rash,
photosensitivity, seizures and psychosis were seen in comparable
proportions to other racial groups.
Similarly, ANA and anti-DNA antibody
positivity was also within the range seen in other racial groups. When
compared with other series, however, alopecia, renal lupus, oral ulcers and
neurological involvement was seen in higher proportions, reaching
statistically significant figures in comparison to some racial groups. In
contrast, haematological manifestations were seen in significantly less
proportions in comparison to some of the racial groups. Serositis and
discoid lesions were also seen in lower proportions than in most of other
races. The proportion of those with anti-Sm antibodies was in between two
extremes of highest among Africans and Israelis and lowest among Chinese
and Europeans.
Other manifestations were comparable to most other racial
groups. Compared to North American and European reports, significantly low
5 and 10 year survival was observed among patients from India. This could
be related to the general public health situation in the country including
less than optimal management facilities in hospitals, delay in diagnosis
due to lack of awareness of the disease, referral bias where only serious
patients reach major city hospitals, or a truly severe disease among
Indians, or a combination of these genetic, environmental and/or
sociocultural factors. The
Main causes of death were irreversible renal
damage, infections and neurological involvement. Despite a comparable
prevalence of anticardiolipin antibodies (aCL) and lupus anticoagulants
(LAC), clinical antiphospholipid syndrome was significantly less common.
Genetic studies showed appreciable increase of HLA DR4 (37.5%) among
patients compared with controls (18%). Additionally the haplotype B8-DR3
was encountered frequently in the patient group.
=====================================================================
9.) Systemic lupus erythematosus (SLE) lymphadenopathy presenting with
histopathologic features of Castleman' disease: a clinicopathologic study
of five cases.
=====================================================================
AU: Kojima-M; Nakamura-S; Itoh-H; Yoshida-K; Asano-S; Yamane-N;
Komatsumoto-S; Ban-S; Joshita-T; Suchi-T
SO: Pathol-Res-Pract. 1997; 193(8): 565-71
AB: Lymph node enlargement is common in active systemic lupus erythematosus
(SLE), a disease characterized by well defined clinical criteria. Although
numerous reports have described the characteristic histology of SLE
lymphadenopathy to include necrotizing lesions and hematoxylin bodies, no
detailed description has examined the histopathologic features that are
similar to Castleman's disease (CD) in SLE patients. In this report, we
describe the clinicopathologic findings of CD-like peripheral
lymphadenopathy, which was identified in five (26%) of 19 SLE patients.
These five patients were all female with an age range of 24 to 44 years,
and four of them presented with multicentric lymphadenopathy. They also had
systemic symptoms and abnormal laboratory findings, indicating active
disease, although two patients had not fulfilled the diagnostic criteria of
SLE at the initial disease.
The size of the enlarged lymph nodes seldom
exceeded 2.0 cm in diameter, and biopsies revealed histopathologic features
similar to CD, of intermediate type in three patients and hyaline vascular
type in two according to the classification of Flendrig [7].
Immunohistochemical studies demonstrated polyclonal plasma cell populations
in all five cases. Epstein-Barr virus genomes were detected in the small
lymphocytes of two of the three cases examined by in situ hybridization
studies. Recently, the histopathologic findings of CD have been associated
with a disrupted immune response, and the present data suggest that SLE
should be listed as one of the diseases showing the histopathologic
features similar to CD.
=====================================================================
10.) [A case of systemic lupus erythematosus associated with minimal change
nephrotic syndrome]
=====================================================================
AU: Horita-Y; Nazneen-A; Cheng-M; Razzaque-MS; Namie-S; Tadokoro-M;
Taura-K; Miyazaki-M; Ozono-Y; Kohno-S; Harada-T; Taguchi-T
SO: Nippon-Jinzo-Gakkai-Shi. 1997 Oct; 39(7): 759-64
AB: A case of systemic lupus erythematosus (SLE) associated with minimal
change nephrotic syndrome (MCNS) in a 25-year-old female is described. The
patient suddenly manifested butterfly rash and proteinuria was first
pointed out on March, 1994. On admission, her skin biopsy indicated SLE.
Subsequently, she developed nephrotic syndrome. Urinalysis showed heavy
proteinuria (4.1 g/day), with no other abnormalities in the urinary
sediment. Immunological examination revealed positive antinuclear antibody
at a titer of 1:80 with a speckled pattern. Anti-ssDNA and anti-SS-A
antibodies were positive, but other antibodies were negative. Serum
complement (CH50) was within the normal range (30.5 U/ml).
The renal biopsy
showed no apparent cellular proliferation or increase of extracellular
matrices in glomeruli by light microscopy. Slight deposition of IgG, IgM,
C3 and C1q was focally seen in the mesangium and capillary wall by
immunofluorescence. Electron microscopic examination revealed small and
scattered dense deposits in the mesangium, subepithelium and
subendothelium, associated with diffuse fusion of the foot processes of
epithelial cells along the glomerular basement membrane.
According to the
WHO classification, the histological features were compatible with those of
lupus nephritis (LN), class Ib. The patient was treated with PREDNISOLONE,
Mizorbine and Dilazep, resulting in the disappearance of proteinuria and a
normal serum level of total protein. The association of LN and MCNS is very
rare. We also investigated the relationship between the intensity of
proteinuria and histological types of 53 cases with LN examined in our
laboratory. The cases with heavy proteinuria were mostly classified as
WHO-Class IV and Class V. We report here a case of LN associated with MCNS
and also review the literatures.
=====================================================================
11.) T cell receptor clonotypes in skin lesions from patients with systemic
lupus erythematosus.
=====================================================================
AU: Kita-Y; Kuroda-K; Mimori-T; Hashimoto-T; Yamamoto-K; Saito-Y;
Iwamoto-I; Sumida-T
SO: J-Invest-Dermatol. 1998 Jan; 110(1): 41-6
AB: Systemic lupus erythematosus is an autoimmune disease characterized by
the presence of autoantibodies and by lymphocytic infiltration into lesions
at several sites such as skin, kidney, and other organs. Immunohistologic
studies have clarified that the majority of lymphocytes in the skin are
CD4+ alphabeta T cells.
In the present work, to clarify the pathologic role
of T cells in the skin of systemic lupus erythematosus patients, we
analyzed T cell receptor (TCR) clonotypes of T cells infiltrating into skin
lesions. TCR Vbeta gene transcripts from T cells from discoid lesions of
the skin and peripheral blood lymphocytes of four systemic lupus
erythematosus patients were amplified by reverse transcriptase polymerase
chain reaction. Southern blot analysis of polymerase chain reaction product
demonstrated the heterogeneous TCR Vbeta repertoire of T cells in the skin
of systemic lupus erythematosus.
Single-strand conformation polymorphism
analysis showed several distinct bands for smears of most TCR Vbeta genes
from T cells infiltrating the skin, whereas smears with few bands were
found for all TCR Vbeta genes from peripheral blood lymphocytes of the same
patients.
The number of bands encoding each TCR Vbeta gene from the skin
was significantly higher compared with peripheral blood lymphocytes.
Sequencing analysis showed a Leucine-X-Glycine amino acid motif at position
96-98 in the CDR3 region at the frequency of 23-24% in skin-accumulated T
cells from two patients, whereas the frequency of this motif in peripheral
T cells was only 0-3%, indicating limited T cell epitopes. In conclusion, T
cells infiltrating into the skin of systemic lupus erythematosus patients
might recognize restricted T cell epitopes on autoantigens and trigger the
autoimmune reaction in skin lesions.
=====================================================================
12.) Circulating plasma levels of nucleosomes in patients with systemic
lupus erythematosus: correlation with serum antinucleosome antibody titers
and absence of clear association with disease activity.
=====================================================================
AU: Amoura-Z; Piette-JC; Chabre-H; Cacoub-P; Papo-T; Wechsler-B; Bach-JF;
Koutouzov-S
SO: Arthritis-Rheum. 1997 Dec; 40(12): 2217-25
AB: OBJECTIVE. To assess nucleosome plasma levels in patients with systemic
lupus erythematosus (SLE) and to study the correlations with serum
antinucleosome, anti-double-stranded DNA (anti-dsDNA), and antihistone
antibody activities, as well as with disease activity (by the SLE Disease
Activity Index [SLEDAI]).
METHODS. In a cross-sectional study, we assessed
58 SLE patients for their plasma nucleosome levels. Plasma nucleosome
levels as well as serum antinucleosome, anti-double-stranded DNA, and
antihistone antibody activities were assessed by enzyme-linked
immunosorbent assay. SLE activity was evaluated using the SLEDAI.
RESULTS.
The mean (+/-SD) plasma nucleosome concentration in SLE patients was 52 +/-
159 ng/ml (range 5-1,180), and was significantly higher than that of the
controls (16 +/- 8.8 ng/ml, range 8-52; P = 0.03). Thirteen of the 58 lupus
patients had levels over the range of normal (defined as the control mean +
3 SD, or 42 ng/ml).
An inverse correlation was found between nucleosome
plasma levels and serum antinucleosome antibody activity in the entire
group of SLE patients, those with active disease, and those with inactive
disease, respectively. No correlation was found between the SLEDAI and
nucleosome plasma concentrations.
CONCLUSION. Nucleosome plasma levels may
be normal or increased in SLE, and found in patients with active or
inactive SLE. Longitudinal studies are needed to further establish whether
high levels of circulating nucleosomes may predict the occurrence of an SLE
flare.
=====================================================================
13.) Autoantibodies to human recombinant erythropoietin in patients with
systemic lupus erythematosus: correlation with anemia.
=====================================================================
AU: Tzioufas-AG; Kokori-SI; Petrovas-CI; Moutsopoulos-HM
SO: Arthritis-Rheum. 1997 Dec; 40(12): 2212-6
AB: OBJECTIVE. To investigate the existence of circulating autoantibodies
to erythropoietin (EPO) in sera from patients with systemic lupus
erythematosus (SLE), and to correlate their presence with anemia and
clinical activity.
METHODS. Ninety-two consecutive patients with SLE, 80
patients with rheumatoid arthritis, and 42 normal individuals were studied.
The patients with SLE were categorized into 3 groups according to
hemoglobin (Hgb) level: group A (45 patients with Hgb > 12 gm/dl), group B
(26 patients with Hgb 10.1-12 gm/dl), and group C (21 patients with Hgb <
or = 10 gm/dl). In all patients with SLE, the disease activity was
evaluated using the European Consensus Lupus Activity Measurement scale.
Antibodies to EPO were detected using an enzyme-linked immunosorbent assay
and purified recombinant human EPO as antigen. The specificity of the
method was evaluated with homologous and cross-reactive inhibition assays.
RESULTS. Antibodies to EPO were found in 15.2% of the SLE patient sera. The
distribution of these antibodies among the 3 groups of SLE patients was as
follows: 8.8% (4 of 45) from group A, 15.4% (4 of 26) from group B, and
28.6% (6 of 21) from group C. The prevalence of antibodies to EPO in
patients with severe anemia (group C) was statistically significantly
higher compared with patients without anemia (chi(2) = 4.31, P < 0.05).
Patients with antibodies to EPO had higher disease activity scores (P <
0.005) and lower levels of the C4 component of complement (P < 0.05)
compared with patients without antibodies to EPO.
CONCLUSION. In this
study, the presence of antibodies to EPO in the sera of SLE patients is
demonstrated for the first time. The presence of these antibodies is
associated with severe anemia and active disease.
=====================================================================
14.) A promoter polymorphism of tumor necrosis factor alpha associated with
systemic lupus erythematosus in African-Americans.
=====================================================================
AU: Sullivan-KE; Wooten-C; Schmeckpeper-BJ; Goldman-D; Petri-MA
SO: Arthritis-Rheum. 1997 Dec; 40(12): 2207-11
AB: OBJECTIVE. The polymorphic tumor necrosis factor alpha (TNFalpha) gene
encodes a cytokine involved in inflammation, angiogenesis, and apoptosis.
One polymorphic variant is associated with increased production of
TNFalpha. This study examined the frequency of this polymorphic variant in
African-American patients with systemic lupus erythematosus (SLE) compared
with controls.
METHODS. We determined the gene frequency of the polymorphic
variant of TNFalpha in an African-American SLE patient population and in a
geographically matched African-American control population.
RESULTS. The
gene frequency of the TNFalpha -308A polymorphism was higher in the
African-American SLE population than in the control population. This
relationship was independent of major histocompatibility complex DR
alleles.
CONCLUSION. The TNFalpha -308A polymorphism is associated with an
increased risk of SLE in African-Americans.
=====================================================================
15.) Histone-specific Th0 and Th1 clones derived from systemic lupus
erythematosus patients induce double-stranded DNA antibody production.
=====================================================================
AU: Voll-RE; Roth-EA; Girkontaite-I; Fehr-H; Herrmann-M; Lorenz-HM; Kalden-JR
SO: Arthritis-Rheum. 1997 Dec; 40(12): 2162-71
AB: OBJECTIVE. To investigate whether histone-specific T helper (Th) cells
that are able to induce anti-double-stranded DNA (anti-dsDNA) antibodies
can be isolated from patients with systemic lupus erythematosus (SLE) and
to characterize the cytokine secretion pattern of such Th clones.
METHODS.
Peripheral blood mononuclear cells from SLE patients and healthy donors
were stimulated with autologous apoptotic cell material or purified
histones, expanded with interleukin-2 (IL-2), and cloned by limiting
dilution. Histone reactivity of clones was examined by histone-specific
proliferation and cytokine release. Cytokines were determined by
enzyme-linked immunosorbent assay (ELISA) and CTLL-2 bioassay. Induction of
anti-dsDNA antibodies was measured in cocultures of autologous B cells and
Th clones by ELISA.
RESULTS. Numerous histone-specific T cell receptor
(TCR) alpha/beta+ Th clones were established from 2 of 3 patients with
active SLE and from 1 of 2 healthy individuals. Most Th clones secreted
IL-2, interferon-gamma (IFNgamma), and IL-4, whereas some produced
predominantly IL-2 and IFNgamma. Th clones that could stimulate the
production of anti-dsDNA antibodies were derived from SLE patients and from
a healthy individual.
CONCLUSION. Th cells specific for histones may play
an important role in the pathogenesis of SLE by inducing autoantibodies to
dsDNA. Both Th1 and Th2 cytokines may be involved in the pathogenesis of
SLE. The presence of histone-specific Th cells in a healthy individual
indicates the importance of peripheral tolerance for preventing
autoimmunity to nuclear antigens.
=====================================================================
16.) Hepatitis C virus antibodies in systemic lupus erythematosus.
=====================================================================
AU: Kowdley-KV; Subler-DE; Scheffel-J; Moore-B; Smith-H
SO: J-Clin-Gastroenterol. 1997 Sep; 25(2): 437-9
AB: To determine the prevalence and significance of serum antibody to
hepatitis C virus (HCV) in patients with systemic lupus erythematosus
(SLE), we measured serum antibodies to HCV by enzyme-linked immunosorbent
(ELISA) and by Abbott MATRIX Immunoblot assays in 42 patients with SLE, a
condition associated with hypergammaglobulinemia.
We used the polymerase
chain reaction (PCR) to identify patients with HCV viremia. Five of 42
(11.9%) patients were seropositive for anti-HCV by ELISA; of these only two
were positive by PCR; only one of three patients seropositive by Immunoblot
assay was also positive by PCR. Both ELISA and the Immunoblot assays may be
falsely positive for ongoing HCV infection in patients with SLE. Suspected
HCV infection should be confirmed with PCR for serum HCV RNA in these
patients.
=====================================================================
17.) Cutaneous lupus mucinosis: a review of our cases and the possible
pathogenesis.
=====================================================================
AU: Kanda-N; Tsuchida-T; Watanabe-T; Tamaki-K
SO: J-Cutan-Pathol. 1997 Oct; 24(9): 553-8
AB: Cutaneous lupus mucinosis (CLM) is a rare variant of lupus
erythematosus eruptions. Our 5 cases with CLM were reviewed. All were men
with systemic lupus erythematosus (SLE). CLM occurred as asymptomatic
cutaneous papules, nodules, or plaques on the trunk, upper and lower
extremities, and face. Histopathology of CLM mainly revealed abundant mucin
deposits among splayed collagen bundles throughout the dermis. However,
some CLM lesions showed discoid lupus erythematosus-like epidermal and
dermal changes and/or lupus profundus. Vasculitis was also revealed in the
CLM lesions of 2 cases. The pathogenesis of CLM may be closely related to
its two important features, the male preponderance and the association with
SLE. Vasculopathy may also be involved in the development of CLM.
=====================================================================
18.) The immunofluorescent profile of dermatomyositis: a comparative study
with lupus erythematosus.
=====================================================================
AU: Magro-CM; Crowson-AN
SO: J-Cutan-Pathol. 1997 Oct; 24(9): 543-52
AB: We have demonstrated a role for microvascular injury mediated by the
membrane attack complex of complement (C5b-9) in the genesis of cutaneous
lesions of dermatomyositis (DM) (1). The purpose of this study is to
revisit the immunofluorescent (IF) profile of DM, to further investigate
the role of C5b-9 in the pathogenesis of cutaneous lesions, and to see if
any features of the IF profile reliably distinguish DM from LE. Lesional
skin biopsies from 24 patients with clinical findings characteristic of DM
were received in formalin and in Michel's transport medium. Conventional
light microscopy, and IF studies with antibodies monospecific for IgG, IgA,
IgM, C3, fibrin and C5b-9 were performed. The control group comprised
biopsies from 31 patients with well-documented LE.
A positive lupus band
test (LBT) correlated highly with a diagnosis of LE, with a sensitivity of
64.5% and a specificity of 95.6% (p=0.001). The LBT was most sensitive in
the setting of DLE and SLE and was least sensitive in the setting of SCLE.
The finding of vascular C5b-9 deposition correlated with a diagnosis of DM
versus LE (p=0.001) although the false positive rate was 21.4%.
The false
negative rate was reduced when vascular C5b-9 was seen in the absence of
antibodies to Ro, La, or RNP. While a negative LBT correlated with a
diagnosis of DM (p=0.001), the specificity was only 64.5%. However, when it
was seen in concert with C5b-9 along the DEJ, specificity was increased to
80.6% (p=0.001).
The presence of C5b-9 in vessels and along the DEJ in
concert with a negative LBT was predictive of DM (p=0.001) with a
specificity of 93.5%, sensitivity of 78.3%, a false positive rate of 10%
and a false negative rate of 14.7%. The combination of a negative LBT,
vascular C5b-9 deposition and negative serology for Ro, La, and RNP was a
predictor of DM versus LE with a sensitivity of 90.5%, a specificity of
96.8%, a false positive rate of 5% and a false negative rate of 6.2%
(p=0.001). The IF profile of DM in lesional skin comprises a negative LBT,
deposition of C5b-9 within vessels and along the DEJ, and variable
keratinocyte decoration for IgG and C5b-9.
The most statistically powerful
predictor of DM is the combination of a negative LBT with vascular C5b-9
deposition and negative serology for antibodies to Ro, La, Sm, and RNP.
Demonstration of a negative LBT in all but 1 case of DM suggests that the
DEJ is not a primary site for antigen-antibody interaction. We postulate
that the aforementioned IF findings reflect humorally mediated injury of
endothelium and keratinocytes, effected by C5b-9.
=====================================================================
19.) [A man with systemic lupus erythematosus presenting with spastic
paraplegia]
=====================================================================
AU: Kashihara-K; Shiro-Y; Shohmori-T; Nomura-A; Hara-H
SO: No-To-Shinkei. 1997 Oct; 49(10): 915-8
AB: We report a 38-year-old man systemic lupus erythematosus who presented
with an acute onset of paraplegia and urinary retention. The man had a
12-year history of nodular cutaneous mucinosis and arthralgia. In 1994, he
was admitted to our hospital with a sudden onset of weakness and numbness
of the right leg followed by an emergence of similar symptoms in the left
leg. His elder sister had died at 16 years of age after suffering from
systemic lupus erythematosus for 6 years.
On examination, the patient had
skin rash on his chest, back, head, forehead, and extremities. The
neurological examination revealed that his tongue deviated to the right on
protrusion. The muscle power was reduced to 2-3/5 in the right leg and to
4/5 in the left leg. The sensory disturbance was noted in the lower
extremities with predominant involvement of the right leg. Reflexes were
increased in the right biceps, triceps, both patellas, and Achilles
tendons. Babinski sign was noted bilaterally. Urinary retention and
constipation were also noted. The results of the blood cell count and
hepatic and renal function tests were normal. Serum levels of C-reactive
protein and complements (C3, C4, CH50) were also normal.
Serological
examinations showed increased anti-DNA antibody (14 U/ml, [normal, < 6]).
Antinuclear antibody was positive at a titer of 1:1380. CSF study showed an
increased protein concentration of 83 mg/dl and an IgG level of 14 mg/dl
with a normal number of cells. MR images revealed a T1-low, T2-high signal
lesion at the upper part of the left ventral medulla. MR images of the
brain and spinal cord were normal.
The patient was diagnosed as having SLE.
High-dose intravenous methylprednisolone (1 g/day) pulse treatment that was
started 25 days after the onset of neurological symptoms, produced partial
relief. Our case presented with paraplegia with a focal lesion in the left
upper ventral part of the medulla on MR images. The incidence of male SLE
is low, and paraplegia is a rare complication of SLE. Thus, the medullary
lesion in SLE observed in our case appears to be rare. SLE should be
considered as a cause of acute onset paraplegia or myelopathy.
=====================================================================
20.) Cyclosporine and therapeutic plasma exchange in treatment of
progressive autoimmune diseases.
=====================================================================
AU: Schiel-R; Bambauer-R; Latza-R; Klinkmann-J
SO: Artif-Organs. 1997 Sep; 21(9): 983-8
AB: Despite treatment with intensive immunosuppressive drug regimens, the
prognosis of patients suffering from severe progressive autoimmune diseases
like systemic lupus erythematosus (SLE), nephrotic syndrome (NS), and
Behcet's disease is poor. Side effects (infections and malignant tumors)
often occur.
In the present trial, 35 patients suffering from autoimmune
diseases (SLE, n = 21; NS, n = 10; and Behcet's disease, n = 4) were
treated for 3.7 +/- 2.0 years with 2.5 +/- 0.6 mg cyclosporine/kg body
weight/day in addition to corticosteroids alone or in combination with
azathioprine and/or cyclophosphamide. In active stages of the diseases with
extremely high concentrations of anti-ds-DNA-antibodies, antinuclear
antibodies, circulating immunocomplexes, and reduced complement
concentrations, therapeutic plasma exchange (TPE) has been applied.
Compared with previous treatment modalities, significantly (p < 0.05) more
effective and rapid reductions of the antibodies were reached. Clinical
disorders improved within 1-6 weeks. All patients reported increased
performance and a better quality of life.
After 1-12 months, the previously
required doses of immunosuppressive drugs and the frequency of TPE could be
reduced by 40-100%. After 13.4 +/- 11.8 months in 17 of 35 patients (8 with
SLE, 5 with NS, 4 with Behcet's disease), cyclosporine was established as
the monotherapy. No severe side effects were registered. In treating active
stages of severe progressive autoimmune diseases and forms with persistent
high antibody levels, the addition of TPE to conventional therapy was very
effective, as observed in both clinical and laboratory parameters.
=====================================================================
21.) [The incidence of the antiphospholipid syndrome in the clinical and biological manifestations of systemic lupus erythematosus]
=====================================================================
AU: Sanchez-Rodriguez-A; Martin-Oterino-JA; Fidalgo-Fernandez-MA;
Araoz-Sanchez-P; Alonso-Garcia-P; Fernandez-Navarro-P; Chimpen-Ruiz-V;
Portugal-Alvarez-J
SO: Rev-Clin-Esp. 1997 Oct; 197(10): 669-74
AB: In the present investigation a study was made on the incidence of
antiphospholipid syndrome (APS) on clinical and biological manifestations
in a series or 32 patients (28 females and 4 males with a mean age of 25
years) diagnosed of SLE (ARA criteria) and APS (Harris criteria) compared
with a group of 25 patients (19 females and 6 males with a mean age of 38
years) diagnosed of SLE without APS. This entails a selection from 124
patients diagnosed of SLE, and an incidence of 25.8% and 9.7% for ACA and
LA, respectively.
After a clinical protocol was filled, a complete
immunological profile was obtained, with lymphocyte subsets, IL-2 receptor,
coagulation study, isotype determination for anticardiolipin antibody
(ACA), lupus anticoagulant (LA), serology for syphilis and imaging
diagnostic techniques. Comparative results, with an statistic assessment,
are shown in tables. It is concluded that SLE + APS population can be
considered as definite for a peculiar SLE subtype.
=====================================================================
22.) Emotional and physical intimacy in coping with lupus: women's dilemmas
of disclosure and approach.
=====================================================================
AU: Druley-JA; Stephens-MA; Coyne-JC
SO: Health-Psychol. 1997 Nov; 16(6): 506-14
AB: This study examined whether self-rated physical and emotional intimacy
of 74 women with their heterosexual partner, during an illness episode of
lupus, was related to their affect and relationship satisfaction. It was
predicted that greater intimacy would be related to better psychosocial
adjustment.
Women who engaged in physically intimate behavior with their
partner more often reported greater relationship satisfaction. Women who
frequently avoided or who were often the initiators of physical intimacy,
however, reported greater negative affect. Concerning emotional intimacy,
women who disclosed more information about illness symptoms and women who
concealed more information about their symptoms and feelings experienced
the highest levels of negative affect. Results identify dilemmas that women
with recurrent illness may face when trying to maintain intimacy during
illness periods.
=====================================================================
23.) [A study on interval of intravenous cyclophosphamide pulse in the
treatment of severe systemic lupus erythematosus]
=====================================================================
AU: Yang-X; Yin-P; Gao-X
SO: Chung-Hua-Nei-Ko-Tsa-Chih. 1996 Apr; 35(4): 257-60
AB: The aim of this study was to compare the clinical efficacy and adverse
effects of intravenous cyclophosphamide pulse therapy between 2-week and
4-week intervals in the treatment of severe systemic lupus erythematosus.
52 patients were involved in the study and were equally divided into two
groups.
The pulse intervals were 2 weeks for group I and 4 weeks for group
II. Each pulse dose of cyclophosphamide was 15 mg/kg. Every patient took
prednisone 1 mg/kg daily for first 8 weeks and then tapered. Most of
disease indices recovered quicker in group I than in group II. The group
with short interval was better than that of long one on total efficacy and
remission rate.
The cyclophosphamide cumulative doses were not different
between two groups as disease activity index dropped 50%. The pulse
interval had to be prolonged from 2 weeks to 4 weeks to six patients in
group I (23.1%) because of leukopenia, and be shortened from 4 weeks to 2
weeks to five cases in group II (19.2%) because of inefficacy.
There was no
statistical difference in adverse effects between two groups. Intravenous
cyclophosphamide pulse therapy with two-weeks interval is advantageous to
recovery of severe systemic lupus erythematosus provided blood test prior
to each pulse. We suggest that pulse interval should be individualized.
=====================================================================
24.) Autonomic nervous dysfunction in systemic lupus erythematosus (SLE) and
rheumatoid arthritis (RA): possible pathogenic role of autoantibodies to
autonomic nervous structures.
=====================================================================
AU: Maule-S; Quadri-R; Mirante-D; Pellerito-RA; Marucco-E; Marinone-C;
Vergani-D; Chiandussi-L; Zanone-MM
SO: Clin-Exp-Immunol. 1997 Dec; 110(3): 423-7
AB: Autonomic nervous dysfunction has been previously reported in SLE, RA
and systemic sclerosis, but the pathogenesis of such a complication is
poorly understood. In the present study, four standard cardiovascular
autonomic function tests were performed in 34 female patients with
connective tissue diseases and in 25 healthy control subjects, and results
expressed as cardiovascular (CV) test scores.
Moreover, in each subject the
presence of circulating complement-fixing autoantibodies directed against
sympathetic and parasympathetic nervous structures, represented by superior
cervical ganglia and vagus nerve, respectively, was simultaneously assessed
by an indirect immunofluorescent complement-fixation technique, using
rabbit tissue as substrate. None of the patients reported autonomic
symptoms.
However, an abnormal CV test score (> or = 5) was detected in 15%
of the patients and in none of the healthy control subjects, approaching
statistical significance (P = 0.07).
No correlation was found between CV
test results and disease duration, type of therapy or presence of
conventional autoantibodies. One or two autoantibodies to autonomic nervous
structures were detected in six patients (18%) and not in the control
subjects (P < 0.05). Values of deep breathing test were significantly lower
in autoantibody-positive patients compared with those amongst the control
subjects (P < 0.05), and an abnormal CV test score was significantly
associated with the presence of autoantibodies to autonomic nervous
structures (P < 0.05).
In conclusion, we confirm that autonomic nervous
function can be impaired in patients with connective tissue diseases, and
suggest that autoantibodies directed against autonomic nervous system
structures may play a role in the pathogenesis of the autonomic dysfunction.
=====================================================================
25.) [Cyclophosphamide therapy in systemic lupus erythematosus]
=====================================================================
AU: Backhaus-M; Hiepe-F
SO: Z-Rheumatol. 1997 Jul-Aug; 56(4): 178-89
AB: Cyclophosphamide is a potent immunosuppressive drug which is widely
used to treat renal and central nervous system manifestation of Systemic
Lupus Erythematosus (SLE). It is employed especially to prevent renal
failure in lupus nephritis. Within the last decade intravenous
cyclophosphamide bolus therapy has become the standard therapy in severe
SLE due to its tendency towards a higher efficacy and fewer side effects as
compared to oral application.
Studies on the slightly more cost-effective
oral cyclophosphamide bolus therapy have recently been published, however,
there are no data on long-term results yet. Here, we review the effects and
side effects of cyclophosphamide as well as recent clinical studies on
cyclophosphamide bolus therapy in SLE. Because of the possible side
effects, especially the high risk of malignancies, which sharply increases
at a cumulative dose of approximately 60 g, cyclophosphamide should be used
cautiously. Cyclophosphamide bolus therapy should only be performed in
hospitals with special experience.
=====================================================================
26.) Pulmonary involvement in systemic lupus erythematosus.
=====================================================================
AU: Fishback-N; Koss-MN
SO: Curr-Opin-Pulm-Med. 1995 Sep; 1(5): 368-75
AB: Pulmonary disorders in systemic lupus erythematosus involve a variety
of clinical presentations and pathologic patterns, which can be difficult
to diagnose due to systemic dysfunction, infection, or complications of
therapy. The causes of dyspnea in systemic lupus erythematosus are
multifactorial, and the clinical manifestations of lung disease widely
vary.
Biopsy is frequently relied on to evaluate and diagnose pulmonary
disease in systemic lupus erythematosus. The patient who has systemic lupus
erythematosus-associated lung disease is effectively treated with various
immunosuppressive drugs, in conjunction with careful evaluation of the
patient's systemic involvement, drug-induced complications, and the
ever-present threat of infection.
=====================================================================
27.) Renal vein thrombosis in Chinese patients with systemic lupus
erythematosus.
=====================================================================
AU: Lai-NS; Lan-JL
SO: Ann-Rheum-Dis. 1997 Sep; 56(9): 562-4
AB: OBJECTIVES: To evaluate the risk factors associated with renal vein
thrombosis (RVT) in Chinese patients with systemic lupus erythematosus
(SLE).
METHODS: Data on clinical symptoms, renal biopsy, antiphospholipid
antibody syndrome profile, and serological examinations of lupus features
were examined retrospectively in six patients with RVT confirmed by
angiography from a total of 625 patients with SLE over a 14 year period
(1982-1996).
RESULTS: The lupus patients with RVT did not have acute
symptoms of severe flank pain, haematuria, and oligouria. In contrast, most
patients were suspected to have RVT because of peripheral oedema and
worsening proteinuria. Roentgenological examinations (including renal
sonography, renal computer tomography, or renal Doppler, or all three) were
positive only in some patients.
Positive antiphospholipid antibody profiles
were found in four of six lupus patients. By renal biopsy, only two samples
were confirmed as World Health Organisation (WHO) class V lupus membranous
glomerulonephritis. The others were class IV in three patients, and class
III in the remaining one. No RVT was found in lupus patients without
nephrotic syndrome. Peripheral thrombophlebitis was, however, noted in only
one patient.
CONCLUSION: Nephrotic syndrome could be a distinct risk factor
in the development of RVT in Chinese SLE patients, in contrast with that
reported in white populations in whom the peripheral thrombotic events were
recognised as a determining factor.
=====================================================================
28.) Usefulness of antinuclear antibody testing to screen for rheumatic
diseases.
=====================================================================
AU: Malleson-PN; Sailer-M; Mackinnon-MJ
SO: Arch-Dis-Child. 1997 Oct; 77(4): 299-304
AB: OBJECTIVE: To assess the usefulness of the indirect immunofluorescence
antinuclear antibody test (FANA) using human laryngeal epithelial carcinoma
cells as nuclear substrate, to screen for childhood rheumatic diseases.
STUDY DESIGN: A review of all FANA tests performed on children at British
Columbia's Children's Hospital between 7 March 1991 and 31 July 1995.
RESULTS: FANA tests were positive at titres of 1:20 or greater in 41% of
all subjects tested, and in 65% of all subjects in whom the diagnosis was
obtained. FANA positivity occurred in 67% of those with a rheumatic
disease, compared with 64% of those with a non-rheumatic disease (p = 0.4).
More girls had high titre FANA positivity than boys independent of whether
or not they had a rheumatic disease (p = 0.05). At a screening serum
dilution of 1:40 a positive test has a sensitivity of only 0.63, and a
positive predictive value of only 0.33 for any rheumatic disease. For
systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD),
or overlap syndrome at a screening dilution of 1:40 the test has a very
high sensitivity of 0.98, but a very low positive predictive value of only
0.10, the test having slightly better characteristics for boys than girls.
CONCLUSION: Although a negative FANA test makes a diagnosis of SLE or MCTD
extremely unlikely, a positive test even at moderately high titres of 1:160
or higher is found so frequently in children without a rheumatic disease
that a positive result has little or no diagnostic value. It is suggested
that a screening serum dilution of 1:160 or 1:320 would increase the
usefulness of the test, by decreasing false positive tests, without
significantly increasing false negative tests for SLE or MCTD, and would
have the potential for considerable cost savings.
=====================================================================
29.) [Human parvovirus B19 infection mimicking systemic lupus erythematosus:
case report]
=====================================================================
AU: Banno-S; Matsumoto-Y; Sugiura-Y; Ueda-R
SO: Ryumachi. 1997 Aug; 37(4): 581-6
AB: Human parvovirus B19 (HPV-B19) has been known as the etiologic agents
of erythema infectiosum in normal childhood, and chronic anemia and
thrombocytopenia in immuno-compromised patients. Recently, this virus has
been reported as the association with rheumatic manifestation such as
rheumatoid arthritis and systemic lupus erythematosus (SLE).
We described
here a patient whose HPV-B19 infection was mimiking atypical symptoms of
SLE at diagnosis, and was persistent because of immuno-suppressive therapy
for SLE. A 34-year-old female was admitted to our hospital on 22 June 1995,
presenting fever episode and cervical lymph node swelling. Before eighteen
months, she was received methyl-predonisolone pulse therapy and plasma
exchange by fresh frozen plasma for the treatment of Stevens-Johnson
syndrome, and after several weeks these therapy she was suffered from viral
infection with lymphadenopathies with a transient appearance of atypical
lymphocytes in her peripheral blood smear.
On laboratory examination at the
present admission, her peripheral blood showed anemia, thrombocytopenia
with atypical lymphocytes. Throughout her hospitalization, anti-nuclear
antibody (ANA) suspected SLE including anti-DNA and anti-Sm antibody were
all negative except of transient week positive ANA screening test. Her
physical condition presented poor clinical course with fever elevation,
increased ascites and renal dysfunction showing the elevation of CRP and
circulating immune-complex (Clq binding method). Her serum was positive for
IgM and IgG antibody against VP-1 and VP-2 antigen of HPV-B19 by ELISA in
April 1996.
And then, HPV-B19 DNA by polymerase chain reaction (PCR) was
positive in bone marrow sample in March 1996, and also positive in spleen
necropsy at death. We confirmed persistent chronic HPV-B19 infection by
measurement of HPV-B19 IgM and IgG antibody by ELISA and HPV-B19 DNA by
PCR.
The plasmapheresis and administration of intravenous immunoglobulin
showed the possible efficacy for her symptom throughout this clinical
course. Moreover, bone marrow smear showed the finding of virus-associated
hemophagocytic syndrome, and finally, she was died of cervical hemorrhage
accompanied with disseminated intravascular coagulation syndrome on July
1996. HPV-B19 infection can present an atypical clinical picture that is
highly suggestive of SLE.
We suggest that the therapy of steroids and
immuno-suppressive agents should be cautious, because these may potentially
cause persistent chronic HPV-B19 infection and induced life-threatening
clinical course.
=====================================================================
30.) SLE and Sjogren's syndrome associated with unilateral moyamoya vessels
in cerebral arteries.
=====================================================================
AU: Matsuki-Y; Kawakami-M; Ishizuka-T; Kawaguchi-Y; Hidaka-T; Suzuki-K;
Nakamura-H
SO: Scand-J-Rheumatol. 1997; 26(5): 392-4
AB: Moyamoya disease is a rare clinical entity, diagnosed by cerebral
angiography and characterized by occlusion of the internal carotid artery
system and the development of collateral arteries. A 30-year-old woman with
systemic lupus erythematosus and Sjogren's syndrome recurrently presented
transient right homonymous hemianopsia. Cerebral angiography showed
occlusion of the left posterior cerebral artery associated with the
development of collateral circulation ("moyamoya vessels").
In a young
adult, as in this case, the unilaterality of the lesion and the
presentation of transient ischemic attacks rather than subarachnoid
hemorrhage are rare features for Moyamoya disease. Antiphospholipid
syndrome was absent.
=====================================================================
31.) SLE with death from acute massive pulmonary hemorrhage caused by
disseminated strongyloidiasis.
=====================================================================
AU: Setoyama-M; Fukumaru-S; Takasaki-T; Yoshida-H; Kanzaki-T
SO: Scand-J-Rheumatol. 1997; 26(5): 389-91
AB: We describe a case of disseminated strongyloidiasis involving a female
patient with active systemic erythematosus (SLE). The cause of death was
massive pulmonary hemorrhage induced by a filariform larvae infection. This
was initially diagnosed during examination of the bronchoalveolar lavage
fluid just 2 days before her death. The case indicated that
immunosuppressed individuals would be paid attention to possible parasitic
infection prior to starting therapy even in non-endemic areas as well as
other microorganisms.
=====================================================================
32.) A fatal case of severe SLE complicated by invasive aspergillosis.
=====================================================================
AU: Zuber-M; Daus-H; Koch-B; Pfreundschuh-M
SO: Rheumatol-Int. 1997; 17(3): 127-30
AB: We report on the case of a 25-year-old female with severe systemic
lupus erythematosus (SLE) who presented with pancytopenia, fever,
arthralgia and abdominal pain. After antibiotic treatment, the patient was
afebrile for 3 days before her temperature rose again. Dyspnoea and cough
pointed towards pneumonia which was confirmed by X-ray. Different
antibiotics and the antimycotic agent fluconazol were given. The lupus
flare was treated with high-dose prednisolone.
After a couple of days, the
dyspnoea increased and mechanical ventilation became necessary.
Bronchoscopy and transbronchial biopsy revealed the diagnosis of invasive
aspergilloses. Despite of an immediate treatment with amphotericin B, the
patient died because of respiratory insufficiency. The literature on
aspergillosis in SLE is reviewed and prophylactic, diagnostic and
therapeutic options are discussed for this infectious complication which
has an 80% mortality in patients with SLE.
=====================================================================
33.) Methotrexate use in miscellaneous inflammatory diseases.
=====================================================================
AU: Wilke-WS
SO: Rheum-Dis-Clin-North-Am. 1997 Nov; 23(4): 855-82
AB: Methotrexate has proven to be a safe, effective, long-term therapy for
rheumatoid arthritis. Its property as a corticosteroid-sparing drug in
rheumatoid arthritis has been recognized and its potential has been
explored in other inflammatory and autoimmune diseases.
This article
describes and analyzes the use of methotrexate for a wide variety of
diseases, some of which are not the usual province of rheumatologists, to
provide some guidance concerning its role for treatment. Methotrexate
therapy seems promising for systemic lupus erythematosus, inflammatory
myopathy, inflammatory eye disease, inflammatory bowel disease, and some
manifestations of sarcoidosis. Its role in other diseases is not as well
defined.
=====================================================================
34.) Renal vascular lesions in lupus nephritis.
=====================================================================
AU: Descombes-E; Droz-D; Drouet-L; Grunfeld-JP; Lesavre-P
SO: Medicine-Baltimore. 1997 Sep; 76(5): 355-68
AB: We retrospectively studied the prevalence, histologic features,
clinical correlations, and long-term outcome of the intrarenal vascular
lesions of lupus nephritis (LN) in a series of 169 renal biopsies performed
between 1980 and 1994 in 132 patients with systemic lupus erythematosus.
The most common vascular lesions were nonspecific sclerotic changes, found
in 37% of the biopsies (24% if only the cases with moderate to severe
changes are considered). The other common vascular lesions were
"immunoglobulin microvascular casts," found in 24% of the biopsies.
Vasculitis and thrombotic microangiopathy were rare lesions and were seen
in only 4 (2.4%) and 1 (0.6%) cases, respectively. Isolated sclerotic
vascular changes were present in biopsies from older patients with a longer
duration of LN, compared with the group with no vascular lesions, and were
associated with a significantly higher prevalence of hypertension. Overall,
however, the long-term renal and patient survival of this group did not
differ significantly from that of the patients without vascular changes.
Immunoglobulin microvascular casts (IMCs) ("lupus vasculopathy") were
characterized by the presence of immunoglobulin deposition within the
glomerular capillaries and small arterioles. In the present study we
extensively investigated the morphologic and immunologic features of this
lesion.
The lesions were notable for the absence of endothelial or parietal
vascular lesions and of fibrin, platelets, and leukocytes, which indicates
that thrombosis is not involved in the vascular obstruction. According to
our data immunoglobulin precipitation in the microvasculature seems to play
a central role in the pathogenesis of this lesion, which is why we propose
the term "immunoglobulin microvascular casts." In general, IMCs were
associated with the most severe and active forms of diffuse proliferative
lupus nephritis (World Health Organization [WHO] class IV).
However our
data show that, in contrast to previous studies, the long-term outcome of
patients with IMCs is not worse than that of other patients with class IV
LN. It may even be somewhat better, suggesting that this type of lesion may
reverse with immunosuppressive therapy. In addition, we did not find any
association between the presence of IMCs and the lupus anticoagulant, IgG
anticardiolipin antibodies, or extrarenal vascular manifestations.
Concerning vasculitis and thrombotic microangiopathy, our results confirm
that their occurrence is quite rare in-lupus nephritis.
The outcome of our
4 patients with vasculitis was not particularly poor, which could be
related to early and/or aggressive treatment. Taken as a whole, our data
confirm that the presence of active and severe forms of diffuse
proliferative LN (WHO class IV) carries a worse prognosis compared with the
other forms of LN. In our study, and in agreement with previous reports
(23), the long-term renal survival of patients with class IV LN was
significantly worse than that of patients with other forms of LN, with a
10-year renal survival of 70% compared with 85%, respectively. However our
data do not support the conclusions of some previous studies that the
presence of intrarenal vascular lesions is a marker of poor renal prognosis
in lupus nephritis.
More precisely, our data show that the somewhat poorer
renal outcome observed in patients with IMCs is related to the fact that in
most cases these lesions are associated with class IV lupus nephritis, and
not related to the presence of the vascular lesion per se.
=====================================================================
35.) Antibodies to C1q in systemic lupus erythematosus: characteristics and
relation to Fc gamma RIIA alleles.
=====================================================================
AU: Haseley-LA; Wisnieski-JJ; Denburg-MR; Michael-Grossman-AR; Ginzler-EM;
Gourley-MF; Hoffman-JH; Kimberly-RP; Salmon-JE
SO: Kidney-Int. 1997 Nov; 52(5): 1375-80
AB: Autoantibodies to the collagen-like region of the first complement
component (C1qAB) are found in patients with systemic lupus erythematosus
(SLE), particularly those with renal disease. In a cohort of 46 SLE
patients with diffuse proliferative glomerulonephritis, we found declining
C1qAB titers in 77% of treatment responders and in only 38% of treatment
non-responders (P < 0.03). To further characterize this autoantibody, we
tested 240 SLE patients for the presence of C1qAB. Positive titers were
found in 44% of patients with renal disease and 18% of patients without
renal disease (chi2 P < 0.0003).
Analysis of IgG subclass revealed IgG2
C1qAB alone in 34%, IgG1 C1qAB alone in 20%, and both IgG1 and IgG2 in 46%
of patients. Fewer than 10% of patients had measurable titers of IgG3 or
IgG4 C1qAB. The pathogenic role of these IgG2-skewed C1qAB may relate to
impaired immune complex clearance by the mononuclear phagocyte system: IgG2
antibodies are efficiently recognized by only one IgG receptor, the H131
allele of Fc gamma RIIa (Fc gamma RIIa-H131).
In contrast, Fc gamma
RIIa-R131, which is characterized by minimal IgG2 binding, has recently
been associated with lupus nephritis. In our C1qAB positive patients, the
presence of Fc gamma RIIA-R131 was associated with an increased risk for
renal disease. Autoantibodies to C1q may have pathogenic significance in
SLE patients with genetic defects in the ability to clear IgG2 containing
immune complexes.
=====================================================================
36.) Heredity and systemic lupus erythematosus: dissecting a complex genetic diease
=====================================================================
AU: Shaver-TS; Harley-JB; Moser-KL
SO: Kans-Med. 1997 Winter-Spring; 97(3-4): 18-22
AB: The etiology of SLE appears to be exceedingly complex and possibly
heterogeneous, with genetics and environment both making substantial
contributions. A schematic representation of potential mechanisms is
depicted in Figure 2. We may not fully understand the pathogenesis of this
disease until we unravel the relative contributions of each component to
the development of SLE. While genetic mechanisms involved in SLE remain
obscure, we now have available elegant laboratory techniques for analysis
of genetic loci as well as computer technology which permits simulation and
analysis of the transmission of complex genetic traits among multiple
families and demographic groups. What remains is the painstaking task of
collecting families multiplex for SLE and analyzing multiple sets of
clinical, serologic, and genetic data within and between these pedigrees.
Such studies are currently underway and will hopefully increase
understanding of this enigmatic and complex autoimmune disorder.
=====================================================================
37.) Massive uncomplicated vascular immune complex deposits in the kidney of a patient with systemic lupus erythematosus.
=====================================================================
AU: Takazoe-K; Shimada-T; Nakano-H; Kawamura-T; Utsunomiya-Y; Kanai-T;
Kitajima-T; Yamaguchi-Y; Joh-K; Mitarai-T; Sakai-O
SO: Clin-Nephrol. 1997 Sep; 48(3): 195-8
AB: The case of a patient with systemic lupus erythematosus (SLE) is
reported which was accompanied by renal dysfunction and massive vascular
immune deposits in the kidney without active glomerular lesions. The renal
biopsy showed arterioles and small arteries with circumferential periodic
acid-Schiff (PAS) and Masson trichrome-positive homogenous material in the
subendothelial area in the absence of thrombotic, necrotizing or
inflammatory lesions. Immunofluorescence and electron microscopy
examination demonstrated immune deposits in the vascular walls.
Glomeruli
showed only minor abnormalities with a trend to collapse. There was no
improvement in renal dysfunction over a 4-year period until the patient's
death, despite steroid therapy producing a decrease in disease activity.
The autopsy showed similar vascular changes to those seen in the biopsy,
however; glomeruli were either sclerotic or showed a trend to collapse.
Massive uncomplicated vascular immune complex deposition without active
glomerular lesions is rare. The present case indicates that this type of
lupus vasculopathy may be a prognostic factor for the loss of renal
function in SLE mediated by hemodynamic glomerular injury.
=====================================================================
38.) [Oral manifestations in patients with systemic lupus erythematosus]
=====================================================================
AU: Meyer-U; Kleinheinz-J; Gaubitz-M; Schulz-M; Weingart-D; Joos-U
SO: Mund-Kiefer-Gesichtschir. 1997 Mar; 1(2): 90-4
AB: Forty-six patients with systemic lupus erythematosus underwent thorough
dental examination to determine the frequency and severity of oral lesions
and periodontal diseases. According to clinical criteria, disease was
classified as severe (n = 26) or less severe (n = 20). The overall rate of
mucosal involvement in the studied patients was 48%-from 54% in patients
with severe disease, 40% in those with less severe disease.
Patients with
severe disease were found to have a higher rate of tooth loss and an
increased rate of gingival inflammation. The severity of periodontal
lesions correlated with alterations in the immunoglobulin pattern,
particularly with an increase in gamma-immunoglobulins. Thus it is
suspected that complex immunodysregulation in combination with
immunosuppressive therapy is responsible for the high rate of oral and
periodontal lesions in patients with systemic lupus erythematosus.
=====================================================================
39.) Vasculitis and bacteraemia with Yersinia enterocolitica in late-onset
systemic lupus erythematosus.
=====================================================================
AU: Gauthier-T; So-AK
SO: Br-J-Rheumatol. 1997 Oct; 36(10): 1122-4
AB: We report a case of Yersinia enterocolitica 0.9 septicaemia
complicating systemic lupus erythematosus in an elderly male patient. The
infection gave rise to digital vasculitis, fevers and general malaise on
top of pre-existing articular symptoms. Features of Yersinia septicaemia
may mimic some of the signs of lupus.
=====================================================================
40.) Neoral--new cyclosporin for old?
=====================================================================
AU: Somerville-MF; Scott-DG
SO: Br-J-Rheumatol. 1997 Oct; 36(10): 1113-5
AB: Cyclosporin A is now well established as an effective second-line drug
to treat rheumatoid arthritis. In April 1995, the microemulsion-based
formulation of cyclosporin (Neoral) was introduced based on its increased
bioavailability at 'no extra cost'.
There may have been concerns that with
increased bioavailability of Neoral, some patients might experience
increased toxicity, particularly if transferring from Sandimmun to Neoral
at the same dose. We describe our experience of 51 patients treated with
Neoral--39 with rheumatoid arthritis, six with psoriatic arthritis and the
remainder with a variety of diseases, including Behcet's, systemic lupus
erythematosus and juvenile chronic arthritis.
All patients continued their
other medication including non-steroidal anti-inflammatory drugs and
analgesics. Five continued low dose prednisolone (average 7.5 mg per day)
all patients were monitored for safety and efficacy throughout their
treatment according to standard protocol.
Five patients were enrolled in a
study of efficacy and safety where the dose of cyclosporin was reduced to
2.5 mg/kg/day at the time of conversion, i.e. to Neoral 2.5 mg/kg/day; 19
patients were converted dose for dose, cyclosporin A dose range 2.5-4
mg/kg/day converted to Neoral dose range 2.5-4 mg/kg/day and 27 patients
started Neoral de novo.
We conclude that cyclosporin is a useful disease
modifying anti-rheumatic agent, and our experience suggests that the new
formulation, Neoral, has a similar safety and efficacy profile to the
original preparation (Sandimmun). Neoral was relatively easy to manage and
we noted a slight reduction in dose when compared to Sandimmun. With dose
adjustments over 18 months the mean dose for patients with RA fell from 3.2
to 2.7 mg/kg/day and of the 27 patients starting Neoral de novo only seven
required an increased dose above 2.5 mg/kg/day in order to establish efficacy.
=====================================================================
41.) Successful therapy with danazol in refractory autoimmune
thrombocytopenia associated with rheumatic diseases.
=====================================================================
AU: Blanco-R; Martinez-Taboada-VM; Rodriguez-Valverde-V; Sanchez-Andrade-A;
Gonzalez-Gay-MA
SO: Br-J-Rheumatol. 1997 Oct; 36(10): 1095-9
AB: The objective was to assess the efficacy of therapy with danazol in
refractory immune thrombocytopenia associated with different rheumatic
diseases. Patients with severe immune thrombocytopenia (platelet counts <
40 x 10(9)/l) with a bone marrow biopsy showing megakaryocytes in normal or
increased number and normal morphology were included if they fulfilled at
least one of the following criteria:
(a) thrombocytopenia refractory to
prednisone (> or = 1 mg/kg/day during > or = 4 weeks);
(b) patients
requiring an unacceptably high dose of prednisone for > 2 months
(prednisone dose > or = 20 mg/day);
(c) no response to at least another
drug besides corticosteroids. Other causes of thrombocytopenia were
excluded.
They were treated with danazol (100-200 mg q.i.d.) and followed
for at least 12 months. Four patients diagnosed with systemic lupus
erythematosus, two with rheumatoid arthritis and one with primary
antiphospholipid syndrome met the inclusion criteria.
All of them achieved
acceptable platelet counts within the first 4 weeks of danazol therapy that
allowed the prednisone dosage to be tapered. No important side-effects
related to danazol therapy were observed. Danazol therapy seems to be a
useful and well-tolerated treatment for refractory immune thrombocytopenia
associated with different rheumatic diseases.
=====================================================================
42.) Predisposing factors in sulphasalazine-induced systemic lupus
erythematosus.
=====================================================================
AU: Gunnarsson-I; Kanerud-L; Pettersson-E; Lundberg-I; Lindblad-S; Ringertz-B
SO: Br-J-Rheumatol. 1997 Oct; 36(10): 1089-94
AB: The aim of this study was to define predisposing factors in patients
with sulphasalazine-induced systemic lupus erythematosus (SLE). Eleven
patients with onset of SLE or SLE-like syndromes during sulphasalazine
treatment are reported. Before the onset of SLE, five of the patients
suffered from rheumatoid arthritis (RA), one from psoriatic arthropathy
(PsoA), two from juvenile chronic arthritis (JCA) and three from ulcerative
colitis (UC).
At the time of diagnosis of drug-induced SLE, analysis of
antinuclear antibodies (ANA), anti-double-stranded DNA antibodies
(anti-dsDNA), anti-histone antibodies (anti-histones), acetylator status of
the enzyme N-acetyltransferase 2 (NAT2) and HLA classification were
performed.
All patients were anti-DNA positive at disease onset and were
determined to be slow acetylators. HLA A1 occurred in 4/10 patients, B8 in
5/10. HLA DR 3 was represented in one patient and DR 3(17) in five
patients. The DQA1* 0501 allele was observed in 7/10 patients and DQB1
0201* in 6/10. Persistent SLE and development of nephritis were noted in
patients with long duration of treatment and high cumulative dose of
sulphasalazine (> 1000 g). In sulphasalazine-induced SLE, slow acetylator
genotype and HLA haplotypes associated with idiopathic SLE seem to predict
disease induction.
Further, as the risk of developing persistent SLE and
nephritis increases with long-standing sulphasalazine medication, it is of
importance to monitor the patients with regard to signs of SLE during the
entire treatment period.
=====================================================================
43.) Clinical features of lupus myositis versus idiopathic myositis: a
review of 30 cases.
=====================================================================
AU: Garton-MJ; Isenberg-DA
SO: Br-J-Rheumatol. 1997 Oct; 36(10): 1067-74
AB: Myositis is a rare but well-recognized complication of systemic lupus
erythematosus (SLE). It is reputed to be milder than primary myositis in
terms of morbidity and treatment response. This study compares clinical and
laboratory features of idiopathic inflammatory myositis in patients with
and without evidence of SLE overlap. We performed a case note review of 30
patients with probable or definite polymyositis/dermatomyositis of whom 11
also had definite or probable SLE.
Lupus patients were slightly younger at
diagnosis than those with primary disease, and more likely to be female. At
presentation, quadriceps strength (expressed as a percentage of expected)
was significantly reduced in both the lupus (48.9%; 95% CI 29.0-70.4%) and
primary (52.0%; 95% CI 43.6-59.4%) myositis groups, and serum creatine
phosphokinase (expressed as a multiple of the upper limit of normal) was
significantly elevated (11.2; 95% CI 5.3-29.1 vs 10.7; 95% CI 6.1-17.6).
During a mean (S.D.) follow-up period of 7.4 (4.1) yr, both groups tended
to follow either a relapsing and remitting, or a chronic persistent course,
and when last seen quadriceps muscle strength remained significantly
depressed. One of the lupus patients and two of the primary myositis
patients died due to direct complications of the disease, and one further
death was attributable to a complication of therapy. Our results suggest
that lupus myositis is often as severe as primary disease and should be
treated with equal vigour.
=====================================================================
44.) Serological characteristics of systemic lupus erythematosus from a
hospital-based rheumatology clinic in Kuwait.
=====================================================================
AU: al-Mekaimi-A; Malaviya-AN; Serebour-F; Umamaheswaran-I; Kumar-R;
al-Saeid-K; Sharma-PN
SO: Lupus. 1997; 6(8): 668-74
AB: Thirty-one consecutive patients with SLE were screened for antinuclear
antibody (ANA), anti-DNA antibodies, extractable nuclear antigen antibodies
(anti-ENAs) including anti-Sm, anti-RNP, anti-SSA (anti-Ro), anti-SSB;
(anti-La), anti-Scl-70, rheumatoid factor (RF), C-reactive protein (CRP),
C3 and C4 levels, anti-cardiolipin antibodies (aCL), biologically false
positive serological test for syphilis (BF-STS) using VDRL test and Coombs'
test. The age of the patients ranged from 11 to 52 year with a median of 29
year; female to male ratio of 5:1.
There were 21 Kuwaitis, four Egyptians,
three from the Indian subcontinent, two Filipinos and one Syrian. Main
clinical categories of SLE were: mild cutaneous SLE in 12 (38.7%), clinical
antiphospholipid syndrome (APS) secondary to SLE in 8 (25.8%),
haematological manifestations of SLE in 5 (16.1%), renal lupus in four
(12.9%), neuropsychiatric in three (9.7%), others (6.4%).
Clinical features
overlapped in several patients. ANA was positive in 96.8% (mean value
891.61 units/ml), anti-DNA in 35.5% (mean value 56.4 units) that was lower
than expected and could be due to selection bias as the patients were from
a rheumatology clinic, anti-ENA in 42%, anti-Sm 13% that was lower than
other non-Caucasian populations, anti-RNP 13%, anti-SS-A in 35.5%,
anti-SS-B in 19.4%, Scl-70 in 13%, CRP in 71% (moderate 58%, very high
13%); C3 mean 1.52 mg/ml (3.2% low levels), C4 mean 0.35 mg/ml (32% low
levels), anticardiolipin mean GPL 35.35 units (high 58%), mean MPL 10.61
units (high 26%), BF-STS in 6%, Coombs' test in 6%, RF positive in 36%.
The
only significant positive clinical associations observed were those of
renal involvement with anti-DNA antibodies (P = 0.042), and clinical
antiphospholipid antibody syndrome with aCL antibodies (P = < 0.05).
=====================================================================
45.) Elevated anticardiolipin antibodies in autoimmune haemolytic anaemia
irrespective of underlying systemic lupus erythematosus.
=====================================================================
AU: Lang-B; Straub-RH; Weber-S; Rother-E; Fleck-M; Peter-HH
SO: Lupus. 1997; 6(8): 652-5
AB: In patients with systemic lupus erythematosus (SLE) and concomitant
Coombs positive autoimmune haemolytic anaemia (AIHA) anticardiolipin
antibodies (aCL) are found more frequently and at higher titres than in SLE
patients without AIHA. In order to assess if aCL elevation is primarily
associated with the underlying SLE, or with AIHA itself, we examined AIHA
patients with and without underlying SLE for the presence of aCL. aCL (IgG
and IgM) were determined by ELISA in 74 SLE patients without AIHA, 22 SLE
patients with AIHA, 50 patients with idiopathic AIHA (warm-reactive
autoantibodies), 52 patients with idiopathic AIHA (cold-reactive
autoantibodies) and 50 healthy controls.
The mean IgG and IgM aCL titres in
SLE patients without AIHA (IgG 37.0 U/ml, IgM 8.9 U/ml) were significantly
elevated compared with the values in healthy controls (IgG 9.1 U/ml, IgM
3.2 U/ml; P < 0.005).
The mean aCL levels in SLE patients with AIHA (IgG
53.2 U/ml, IgM 28.2 U/ml) were higher than in SLE patients without AIHA (P
= 0.09 for IgG, P < 0.005 for IgM). Interestingly, the mean aCL levels of
patients with idiopathic AIHA (warm-reactive autoantibody type: IgG 29.2
U/ml, IgM 19.3 U/ml; cold-reactive autoantibody type: IgG 27.4 U/ml, IgM
18.9 U/ml) were also significantly elevated compared with healthy controls
P < 0.001).
As aCL are elevated not only in SLE (with and without
concomitant AIHA) but also in idiopathic AIHA it can be speculated that aCL
are involved in the pathomechanism of autoantibody-induced erythrocyte
destruction per se irrespective of an underlying SLE.
=====================================================================
46.) A young woman with SLE: diagnostic and therapeutic challenges.
=====================================================================
AU: McDonagh-JE; Khamashta-MA; Menon-S; Rahman-A; Isenberg-DA
SO: Lupus. 1997; 6(8): 633-7
AB: The presence of antiphospholipid antibodies, especially of the IgG
isotype and in high titre, is associated with additional complications in
patients with SLE. The thrombocytopaenia and cerebral events in the patient
described are likely to have been linked to her lupus anticoagulant.
However, the antibody and anticardiolipin antibodies are not necessarily
synonymous and indeed we did not detect anticardiolipin antibodies in the
patient, although her sister had them. It is likely that they represent
overlapping sets of immunoglobulins. The production and analysis of further
such antibodies, as described in this review, is awaited urgently.
Much
effort is also being expanded on identifying the precise targets for these
antibodies. In a very recent report Horkko et al have shown that these
antibodies may be directed against epitopes of oxidized phospholipids. The
management of patients with complex disorders such as described here remain
a challenge, although in the short term the patient's major locomotor,
neurological, dermatological and haematological problems have been
controlled. Long-term problems including impaired fertility and
osteoporosis remain to be faced.
=====================================================================
47.) Binding characteristics of SLE anti-DNA autoantibodies to modified DNA
analogues.
=====================================================================
AU: Arjumand-S; Moinuddin; Ali-A
SO: Biochem-Mol-Biol-Int. 1997 Oct; 43(3): 643-53
AB: Native calf thymus DNA was modified with furocoumarins and reactive
oxygen species (ROS). The modifications were probed by UV & Fluorescence
spectroscopy, thermal denaturation and hydroxyapatite chromatography. In CD
spectroscopy changes in the ellipticity of the DNA molecule were observed
as a result of modification. The binding specificity of naturally occurring
anti-DNA antibodies with modified DNA molecules was assessed by ELISA and
quantitative precipitin titration. The affinity of anti-DNA antibodies for
modified conformers was found to be quite high.
=====================================================================
48.) Circulating interleukin-6 type cytokines in patients with systemic
lupus erythematosus.
=====================================================================
AU: Robak-E; Sysa-Jedrzejowska-A; Stepien-H; Robak-T
SO: Eur-Cytokine-Netw. 1997 Sep; 8(3): 281-6
AB: We investigated the serum concentration of interleukin-6 (IL-6) and the
IL-6 family of cytokines (leukemia inhibitory factor (LIF), oncostatin M
(OSM) and ciliary neurotrophic factor (CNTF) using an enzyme-linked
immunosorbent assay (ELISA) in 64 patients with systemic lupus
erythematosus (SLE) and 15 healthy controls.
We also examined a possible
association between the serum levels of these proteins and SLE activity as
well as correlations between the IL-6 concentration and the levels of LIF,
OSM and CNTF. IL-6 was detectable in all 64 patients with SLE and normal
individuals, and the level of this cytokine was significantly higher in
patients than in the control group (p < 0.002 ). LIF, OSM and CNTF were
detectable in 9 (14.1%), 6 (9.4%) and 51 (78%) patients, respectively, and
undetectable in the majority of healthy individuals.
We found positive
correlation between the serum concentrations of IL-6, LIF, OSM and CNTF and
SLE activity. IL-6 and OSM serum levels were also correlated but not IL-6
and LIF or CNTF. In conclusion, an increase in the serum levels of IL-6
and, to a lesser extent of LIF, OSM and CNTF concentrations may be useful
markers for SLE activity.
=====================================================================
49.) [Osteonecrosis in systemic lupus erythematosus. Report of 3 cases]
=====================================================================
AU: Marron-A; Formiga-F; Valverde-J; Mitjavila-F; Pac-M; Moga-I
SO: An-Med-Interna. 1997 Jun; 14(6): 307-9
AB: We present three cases of patients with systemic lupus erythematosus
(SLE) and osteonecrosis or avascular necrosis (AV). Although, the
pathogenesis of osteonecrosis is controversial and multifactorial, the
glucocorticoids therapy is the most important factor contributing to the
lesion. We report the clinical presentation of the three patients. We
comment the characteristics of AV, the diagnosis and the treatment of this
uncommon complication in SLE patients.
=====================================================================
50.) Association of an insertion polymorphism of angiotensin-converting enzyme gene with the activity of systemic lupus erythematosus.
=====================================================================
Author
Sato H; Akai Y; Iwano M; Kurumatani N; Kurioka H; Kubo A; Yamaguchi T; Fujimoto T; Dohi K
Address
First Department of Internal Medicine, Nara Medical University, Kashihara, Japan.
Source
Lupus, 7(8):530-4 1998
Abstract
Systemic lupus erythematosus (SLE) shows various clinical manifestations, which are characterized by inflammation in many different organ systems. The cause of SLE is still unclear; however, the immunological abnormalities are considered to be responsible for the pathogenesis of SLE. As angiotensin I-converting enzyme (ACE) has been reported to be associated with various immunological phenomena, we investigated the correlation between insertion (I)/deletion (D) polymorphism of the ACE gene and the disease activity of SLE.
Ninety-three patients with newly diagnosed SLE were enrolled in this study. ACE genotype was determined by the polymerase chain reaction (PCR). We measured serum levels of anti-double-stranded (ds) DNA antibody (Ab) and serum levels of total complements (CH50) as the parameter for lupus activity.
Moreover, we evaluated the clinical disease activity by calculating SLE disease activity index (SLEDAI). Individuals with II genotype showed a significant increase in SLE activity. Patients with the ACE II genotype showed a higher serum level of anti-dsDNA Ab (14.3 IU/ml (5.475, 74.6, median (25th centile, 75th centile)) than those with the DD genotype (4.65IU/ml (4.05, 6.8)) (P<0.01).
Moreover, patients with the 11 genotype also showed lower levels of serum CH50 than those with the DD genotype (P < 0.01). Patients with the II1 or DI genotype had significantly higher SLEDAI score than those with the DD genotype (P < 0.01). These results suggest that the ACE genotype could be associated with the disease activity of SLE. ACE insertion polymorphism might be used as one of predictive factors for the activity of lupus.
======================================================================
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DATA-MÉDICOS/DERMAGIC-EXPRESS No (50) 07/05/99 DR. JOSÉ LAPENTA R.
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Produced by Dr. José Lapenta R.
Dermatologist
Maracay Estado Aragua Venezuela 1999-2026
Telf.:
04142976087 - 04127766810
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