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PubMedDate not supplied

Deficiency of C4A is a genetic determinant of systemic lupus erythematosus in three ethnic groups.

Systemic lupus erythematosus (SLE) has shown associations with the major histocompatibility complex (MHC) class II DR antigens and class III complement components C2 and C4 in previous studies. The primary susceptibility locus has been difficult to identify, however, on account of linkage disequilibrium within the MHC. We have studied C4A and C4B distributions in 63 Caucasoid, 75 Chinese and 51 Japanese SLE patients. All three populations showed a statistically significant increase in C4A*Q0 (null) alleles when compared with 323 ethnically matched controls. We conclude that complete or partial deficiency of C4A is a genetic determinant of SLE common to these three ethnically distinct populations.

Alleles↗
PubMed1999

Hormonal regulation of tumor necrosis factor-alpha, interleukin-12 and interleukin-10 production by activated macrophages. A disease-modifying mechanism in rheumatoid arthritis and systemic lupus erythematosus?

Rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) frequently develop and progress in settings in which sympathoadrenomedullary and gonadal hormone levels are changing, e.g., during pregnancy, postpartum period, menopause, estrogen administration. This paper addresses the view that adrenal and gonadal hormonal deficiency facilitates excessive macrophage production of TNF-alpha and IL-12 that characterizes RA, whereas excessive estrogen action is suggested to play an essential role in the production of IL-10 in patients with SLE. Disease activity in SLE, in contrast to RA, appears to be associated with high-level production of IL-10, relative to the proinflammatory cytokines, TNF-alpha and IL-12. Accumulating data suggest that novel therapeutic approaches may ultimately be developed from continued investigation of the role of the neuroendocrine factors in RA and SLE.

Animals↗
PubMed1987

Serological and virological evidence of human T-lymphotropic virus in systemic lupus erythematosus.

We present evidence that systemic lupus erythematosus (SLE) patients, but not other mixed connective tissue disease patients, have been exposed to a retrovirus similar or identical to known human T-lymphotropic viruses (HTLV). Serological studies demonstrated a high incidence of seropositivity in SLE patients to HTLV-I antigens by indirect immunofluorescence and Western blot analysis, and to HTLV-III by Western blot analysis. Furthermore, peripheral blood mononuclear cells from four patients with SLE cultivated in vitro expressed HTLV-I antigens after 3 days or more in culture, and reverse transcriptase activity was detected in supernatants from short-term cultures.

Antibodies, Viral↗
PubMed1983

Ethnic difference in the prevalence of systemic lupus erythematosus.

A retrospective study of systemic lupus erythematosus (SLE) was carried out in Auckland, New Zealand, for the years 1975 to 1980 inclusive. One hundred and fifty-one patients were found of which 106 fulfilled the American Rheumatism Association criteria. There were 15 deaths. Age-adjusted prevalence rates per 100 000 were estimated for all cases at the end of 1980 as follows: white 14.6, Polynesian 50.63, and other 19.11. There was a preponderance of females in each ethnic group, average 87% of all cases. Age-adjustment mortality rates per million patient years were: white 2.5 and Polynesians 13.0. Survival curves for both ethnic groups were similar. The prevalence rate in New Zealand Polynesian is significantly higher than in white counterparts which confirms the observation that the disease is commoner in the pigmented races.

Adolescent↗
PubMed2004

Pulmonary disease in systemic lupus erythematosus and the antiphospholpid syndrome.

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by disturbances in innate and adaptive immune mechanisms. Multiple systems and organs may be involved. Tissue damage and dysfunction are mediated by autoantibodies and immune complex formation. The lungs are among the organ systems commonly involved. The pulmonary manifestations usually occur in patients with multisystem disease and include: pleural involvement, parenchymal disease, pulmonary vascular disease and diaphragmatic dysfunction. Manifestations may range from sub-clinical abnormalities to life threatening disorders. Many of the pulmonary manifestations characteristic of SLE are seen in the antiphospholipid syndrome (APS) as well, in both the primary and secondary syndrome. In this review the diverse pulmonary manifestations are described as well as the diagnostic modalities available, including the use of induced sputum evaluation for early diagnosis and follow up. New treatment modalities are referred to.

Antibodies, Antiphospholipid↗
PubMed1988

Subacute cutaneous lupus erythematosus associated with breast carcinoma.

A case of subacute cutaneous lupus erythematosus associated with breast carcinoma is presented. The patient had not been aware of her malignant disease and had consulted us after her dermatosis developed. To date only one case of subacute cutaneous lupus erythematosus associated with malignancy has been described in the literature. The relationship between a dermatosis and a malignant internal disease is discussed.

Adenocarcinoma↗
PubMed2003

Abdominal pain as the initial and sole clinical presenting feature of systemic lupus erythematosus.

Classically, a diagnosis of systemic lupus erythematosus (SLE) is dependent on renal, rheumatological, cutaneous and neurological target organ damage with supporting serological markers. A previously healthy 26-year-old Japanese woman whose only manifestation of otherwise occult SLE was severe abdominal pain is reported. A computed tomographic scan of the abdomen revealed thickened loops of small bowel, endoscopic findings were nonspecific and jejunal biopsy revealed a nonspecific enteritis. Laboratory studies revealed lymphopenia, hypocomplementemia, a positive antinuclear antibody, a weakly positive anti-Smith and a strongly positive anti-double stranded DNA. There was a prompt symptomatic recovery with immunosuppressive therapy. The authors' experiences, and a review of the literature suggest that a diagnosis of SLE should be considered in young Asian women who present with significant but clinically enigmatic gastrointestinal illness.

Abdominal Pain↗
PubMed2003

Degos' disease: a distinctive pattern of disease, chiefly of lupus erythematosus, and not a specific disease per se.

Degos' disease, known confusingly as malignant strophic papularis, is an uncommon condition of unknown cause characterized by distinctive infarctive lesions in the skin, gastrointestinal tract, and central nervous system; the lesions at the two latter sites often result in death. We deem Degos' disease to be analogous to lupus erythematosus in the sense that each is fundamentally a systemic pathologic process involving several organs, among them the skin, but, moreover, we regard Degos' disease, in most instances, to be an actual manifestation of lupus erythematosus. Histopathologically, the findings in sections of tissue of skin lesions of Degos' disease are indistinguishable from those of one expression of cutaneous lupus erythematosus; immunopathologically, some patients with morphologic findings stereotypical of Degos' disease display signs characteristic of lupus erythematosus. For these reasons, we consider Degos' disease to be a distinctive pattern of disease, rather than a specific disease per se, just as are erythema multiforme, erythema nodosum, leukocytoclastic vasculitis, Sweet's syndrome, and pyoderma gangrenosum, to name but five of scores of them. The singular pattern that is designated Degos' disease usually is an expression of lupus erythematosus, but, episodically, of conditions like dermatomyositis and rheumatoid arthritis.

Adolescent↗
PubMed2002

Trends in deaths from systemic lupus erythematosus--United States, 1979-1998.

Systemic lupus erythematosus (SLE) is a serious autoimmune disease of unknown etiology that can affect several organs. Because SLE affects connective tissues and because painful joints and arthritis are among its most common manifestations, this disease is classified with arthritis and other rheumatic diseases. SLE is one of the more fatal forms of rheumatic diseases and non-Causcasian race is a risk factor for death from SLE; however, trends in death from SLE have not been analyzed recently. To characterize deaths from SLE, CDC reviewed SLE deaths during 1979-1998. This report presents the results of that analysis, which indicate that marked age-, sex-, and race-specific disparities exist in SLE death rates and that death rates have increased by approximately 70% during the study period among black women aged 45-64 years. Prevention of deaths requires early recognition and diagnosis of SLE and appropriate therapeutic management.

Adolescent↗
PubMedDate not supplied

[Serum factors inhibiting cellular immunity in systemic lupus erythematosus].

Forty three patients with systemic lupus erythematosus (SLE) were divided into three groups, inactive, active without treatment and active on treatment with steroids. T lymphocytes from peripheral blood were determined and the results compared with those of twenty five normal individuals. In further experiments, the mitogenic capacity of SLE lymphocytes to Con A and the serum's activity of these patients upon normal lymphocytes were analized. Decreased T lymphocytes were found in all groups of patients with SLE. There were no difference between the mitogenic response of SLE patients and controls when the lymphocytes were incubated with normal human AB serum. When normal lymphocytes were incubated with serum from SLE patients with active disease, and inhibition of mitogenic response to Con A was observed. These results suggests that the decrease cellular immunity observed in SLE is due to serum factors rather than to intrinsic T cell abnormality.

Adult↗
PubMed1998

Late onset systemic lupus erythematosus in southern Chinese.

OBJECTIVE: Systemic lupus erythematosus (SLE) is a multisystem disorder that predominately affects women of the reproductive age. Onset of the disease beyond the age of 50 years is unusual. This study was undertaken to compare retrospectively the clinical and laboratory features between early and late onset (onset of disease beyond the age of 50 years) SLE patients in a Chinese population. METHODS: Case records of all SLE patients who attended our rheumatology clinics between 1971 and 1997 were reviewed. Patients with a disease onset beyond the age of 50 years were identified. One hundred consecutive SLE patients who had their disease onset before the age of 50 were recruited as controls. The presenting clinical features, autoantibody profile, number of major organs involved, number of major relapses, and the use of cytotoxic agents in the two groups of patients were obtained and compared. RESULTS: 25 patients with late onset SLE were identified. All the female patients in the late onset group were postmenopausal. The female to male ratio was 3.2 to 1, compared with 13.3 to 1 in the control group (p < 0.02). Both groups had a comparable duration of disease. There were no significant differences in the presenting features between the two groups except for a lower prevalence of malar rash (24% v 86%, p < 0.0001) and a higher prevalence of rheumatoid factor (32% v 1%, p < 0.0001) in the late onset patients. On subsequent visits, the late onset group had a lower prevalence of lupus nephritis (4% v 51%, p < 0.001), fewer major organs involved (mean number of major organs involved; 0.3 v 0.9, p < 0.02), fewer major relapses (mean number of major relapses/patient; 0.08 v 0.47, p < 0.002, number of major relapses/patient year; 0.009 v 0.12, p < 0.001), and required fewer cytotoxic agents for disease control (percentage of patients on cytotoxic agents; 32% v 79%, p < 0.002). CONCLUSION: Late onset SLE in Chinese tends to run a more benign course with fewer major organ involvement and fewer major relapses. The significantly higher incidence of male sex in late onset SLE and the milder disease course in the postmenopausal female patients suggest that oestrogen status may influence disease activity.

Adolescent↗
PubMed2006

Involvement of a tissue-specific autoantibody in skin disorders of murine systemic lupus erythematosus and autoinflammatory diseases.

Human systemic lupus erythematosus (SLE) and its murine model, MRL lpr/lpr mice, are well known to develop a wide range of symptoms, such as glomerulonephritis, dermatitis, and arthritis, as an immune-complex disease. However, the deposition of circulating immune complex does not fully explain the tissue specificity of disease. Tissue-specific autoantigens may also be involved in tissue inflammation. In this study, desmoglein 3 (Dsg3), a major component of epidermal desmosomes, was identified as a skin-specific autoantigen. Several murine models of skin inflammation were found to develop autoantibodies to Dsg3 tightly correlated with disease aggravation, especially in MRL lpr/lpr mice. Furthermore, SLE-prone skin disease-free FcgammaRIIb-deficient mice developed skin inflammation upon immunization with Dsg3. Taken together with histological studies, we concluded that skin-specific Dsg3 serves as an autoantigen in chronic skin inflammatory diseases accompanied by mast cell degranulation, including both murine SLE and other autoinflammatory diseases.

Animals↗
PubMed1991

The time course of acute psychiatric episodes in systemic lupus erythematosus.

Acute psychotic manifestations in systemic lupus erythematosus (SLE) have been considered usually to occur early in the course of illness, although these findings are primarily based on studies with few patients and limited lengths of observation. To further investigate the chronology of acute psychiatric disturbances in SLE, the time course of these episodes was examined in 36 patients over a median duration of observation of 72 months. Twenty-two of 36 (61.1%) initial psychiatric episodes occurred within the first year of diagnosis of SLE, although several patients experienced the initial onset of psychiatric manifestations several years after the onset of SLE. Recurrent episodes occurred in 10 of 36 patients at a median of 8 months after the initial episode. Although tending to occur early in SLE, primary acute psychiatric events may first occur in patients with long durations of illness, and the time of onset of these episodes does not appear helpful in their differential diagnosis.

Acute Disease↗
PubMed1987

[Diagnostic and prognostic significance of complement fixing anti-n-DNA antibodies in systemic lupus erythematosus].

Four patients with active systemic lupus erythematosus and eleven patients at quiescent stage were subjected to research in order to determine the serum concentration of C3 and to estimate the presence of anti-n-DNA antibodies and complement-fixing anti-n-DNA antibodies by the indirect immunofluorescent technique on the Crithidia luciliae. The medium title of anti-n-DNA antibodies was significantly more present in the sera of patients with active disease rather than in patients with quiescent disease and only in the first ones were found complement-fixing anti-n-DNA antibodies. Their title is expression of the title of total anti-n-DNA antibodies. Only sera with titles greater than 1:80 had antibodies able to fix the complement and at the same time they showed an important fall of C3. So the course of complement-fixing anti-n-DNA antibodies seems to have a certain prognostic meaning on the disease development.

Antibodies↗
PubMed2006

Heart involvement in systemic lupus erythematosus, anti-phospholipid syndrome and neonatal lupus.

Cardiac involvement is one of the main complications substantially contributing to the morbidity and mortality of patients suffering from systemic autoimmune diseases. All the anatomical heart structures can be affected, and multiple pathogenic mechanisms have been reported. Non-organ-specific autoantibodies have been implicated in immune complex formation and deposition as the initial triggers for inflammatory processes responsible for Libman-Sacks verrucous endocarditis, myocarditis and pericarditis. Anti-phospholipid antibodies have been associated with thrombotic events in coronary arteries, heart valve involvement and intra-myocardial vasculopathy in the context of primary and secondary anti-phospholipid syndrome. Antibodies-SSA/Ro and anti-SSB/La antigens play a major pathogenic role in affecting the heart conduction tissue leading to the electrocardiographic abnormalities of the neonatal lupus syndrome and have been closely associated with endocardial fibroelastosis.

Antibodies, Antiphospholipid↗
PubMed1987

Large speckle-like thready and thready antinuclear antibody patterns as markers for different clinical presentations in lupus erythematosus.

Fifty-one patients with lupus erythematosus were studied retrospectively. They were chosen on the basis of their antinuclear antibody (ANA) immunofluorescent pattern. Only those with the thready or the large speckle-like thready patterns were studied. Autoantibody profiles consisting of ANA, anti-single-stranded deoxyribonucleic acid (ssDNA) antibody, and anti-extractable nuclear antigen (ENA) antibody determinations were obtained. The patients with the thready ANA pattern and anti-ENA (Sm) antibodies had a significantly higher incidence of pulmonary, joint, and renal involvement than the anti-ENA negative patients with the large speckle-like thready pattern. There was also a significantly higher incidence of Raynaud's phenomenon in patients with the thready pattern than in those with the large speckle-like thready pattern. Photosensitivity was seen significantly more frequently in the patients with the large speckle-like thready pattern than in those with the thready pattern.

Antibodies, Antinuclear↗
PubMed2005

Association between discoid lupus erythematosus and cigarette smoking.

BACKGROUND: Discoid lupus erythematosus (DLE) is a chronic cutaneous disease affecting photoexposed areas and has also been associated with cigarette smoking. OBJECTIVE: To evaluate the association between smoking and DLE. METHODS: A case-control study was performed involving 57 cases diagnosed with DLE and 215 healthy controls. RESULTS: A higher smoking prevalence was noted in DLE cases (84.2%) than controls (33.5%), and the odds ratio adjusted for gender, age and ultraviolet index in the city of origin was 14.4 (95% confidence interval 6.2-33.8; multiple logistic regression, p < 0.01). The cumulative smoking exposure was not related to premature DLE development. At the beginning of the disease, smokers had more extensive involvement than nonsmokers; compromise of the upper arms was statistically related to smoking. CONCLUSION: Cigarette smoking was statistically associated with DLE development. Other studies are needed in order to evaluate the effects of smoking cessation on the course of disease.

Adolescent↗
PubMed2000

Comparison of the validity and sensitivity to change of 5 activity indices in systemic lupus erythematosus.

OBJECTIVE: To compare the construct validity and sensitivity to change of the Systemic Lupus Activity Measure (SLAM), Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), Lupus Activity Index (LAI), British Isles Lupus Assessment Group index (BILAG), and the European Consensus Lupus Activity Measure (ECLAM). METHODS: Twenty-three patients with systemic lupus erythematosus (SLE) were examined prospectively every 2 weeks for up to 40 weeks. Nineteen patients completed all 20 assessments. At each assessment, each of the 5 activity indices was scored, along with physicians' and patients' global assessments of SLE activity. Construct validity was determined by the strength of correlations between changes over time in each activity index and changes in physician and patient global assessments. Sensitivity to change was determined by the magnitude of change in each index over the 2 week interval of greatest change in the physician or patient global assessments, and calculated as standardized response means (SRM; mean change/standard deviation of change). Thirteen patients were also examined monthly by a second physician who was blinded to previous scores on the activity indices and to the patient global assessments. RESULTS: Patients had substantial changes in SLE activity during the study. Changes in each activity index were correlated with changes in the physician global assessment (SLAM r = 0.54; SLEDAI r = 0.52; LAI r = 0.75; BILAG r = 0.61; ECLAM r = 0.65; all p < 0.0001). Correlations were somewhat lower with the blinded physician assessment (SLAM r = 0.42; SLEDAI r = 0.12; LAI r = 0.30; BILAG r = 0.28; ECLAM r = 0.32). The SLAM was the only index that was positively correlated with changes in the patient global assessment (r = 0.22; p < 0.0001). Sensitivity to change was greatest for the LAI (SRM = 0.74) and the ECLAM (SRM = 0.75) and smallest for the SLEDAI (SRM = 0.48) when the physician global assessment was used as the standard. Sensitivity to change was greatest for the SLAM (SRM = 0.61) and the BILAG (SRM = 0.57) and smallest for the SLEDAI (SRM = -0.01) when the patient global assessment was used as the standard. CONCLUSION: Each index is a valid measure of SLE activity. The SLAM captures patients' assessments better than the other indices, perhaps because it assesses the patients' subjective complaints to a greater extent. Estimates of sensitivity to change varied with the standard used, but the SLEDAI was least sensitive to change. Larger studies are indicated to further compare the sensitivity to change of these indices.

Adult↗

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