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PubMed1982

Influence of oral contraceptive therapy on the activity of systemic lupus erythematosus.

Since harmful effects of estrogens in murine lupus are well established, we studied the influence of oral contraceptive therapy on systemic lupus erythematosus activity in 26 female patients with lupus nephropathy. Combined preparations containing either 50 micrograms (14 patients) or 30 micrograms (7 patients) of ethinyl-estradiol were used in 21 courses in 20 patients. Initial manifestations or exacerbations of systemic lupus activity appeared within 3 months of beginning hormonal therapy in 9 patients, an overall incidence of lupus flare-up of 43%; there was major renal involvement in 4 patients. Conversely, evidence of lupus exacerbation did not develop in any of 11 patients who received pure progestogen oral contraceptive therapy with either continuous low-dose norsteroids (6 patients) or discontinuous progestogens at normal dosage (5 patients). These patients were followed for 5--30 months. Our data indicated that oral contraceptive therapy that used estrogens, even at low doses, often induced exacerbation of systemic lupus erythematosus activity. Pure progestogens, which were effective and devoid of such unfavorable effects, may be preferred in these patients.

Adolescent↗
PubMed1980

Unusual cutaneous manifestations of systemic lupus erythematosus: I. Urticaria-like lesions. Correlation with clinical and serological abnormalities.

Ten of 143 systemic lupus erythematosus patients demonstrated urticaria-like lesions. Lesional biopsies in 7 of 9 patients tested revealed a leukocytoclastic angiitis and in 2, a mononuclear perivascular infiltrate. Direct immunofluorescent studies in 2 of 6 patients tested revealed IgM and C3 deposition in and about dermal blood vessels. Nine of the 10 systemic lupus erythematosus, patients displayed active clinical disease (e.g., arthritis, renal disease, etc.), a positive lupus band test, antibodies against deoxyribonucleic acid or Sm macromolecules, serum hypocomplementemia and markedly elevated quantities of serum immune complexes as determined by an immunoradiometric assay employing Raji cells. Similar lesions were not detected in 35 discoid lupus erythematosus patients. These studies strongly suggest: (1) urticaria-like lesions are uncommon cutaneous manifestations of systemic lupus erythematosus. (2) These urticaria-like lesions do not represent a classic IgE mediated urticaria. (3) These urticaria-like lesions generally occur in lupus erythematosus patients demonstrating clinical and/or serological evidence of systemic disease activity. (4) These lesions are probably secondary to immune complex deposition. We, therefore, conclude that all urticarial lesions in lupus erythematosus patients should be biopsied and the patient evaluated for active systemic disease.

Antibodies, Antinuclear↗
PubMed1991

Autoantibodies against all the phospholipids: a comparative systematic study with systemic lupus erythematosus and healthy sera.

The sera of systemic lupus erythematosus patients were tested by ELISA for the presence of autoantibodies against all the phospholipids: cardiolipin, phosphatidic acid, phosphatidylcholine, phosphatidylethanolamine, phosphatidylglycerol, phosphatidylinositol and phosphatidylserine. The quantity of phospholipid coated (in comparison with that initially deposited) on the microtitration plates was precisely evaluated in order to determine if the results (absorbance values) obtained for each phospholipid could be compared directly. The systemic lupus erythematosus sera tested gave positive results for all the phospholipids. The highest level of autoantibodies was observed with phosphatidic acid followed by phosphatidylserine, phosphatidylinositol, cardiolipin, phosphatidylglycerol, phosphatidylcholine and phosphatidylethanolamine. The sera seemed to contain antibodies directed either against all the 8 phospholipids tested or more specifically against one or two phospholipids. The results were compared with those obtained with 17 healthy sera. Much lower values were obtained for the sera of healthy subjects, the majority of which showed a weak binding, similar for all the phospholipids. These results suggest that the anti-phospholipid autoantibodies present in systemic lupus erythematosus sera are significantly higher than those of healthy subjects. It is concluded that in the investigation of anti-phospholipid antibodies, tests should be carried out for all the phospholipids.

Antibody Specificity↗
PubMed2026

Gut microbiota dysbiosis and host metabolite-immune crosstalk drives the pathogenesis of neonatal lupus erythematosus: a multi-omics analysis.

BACKGROUND: Neonatal lupus erythematosus (NLE) is a rare autoimmune condition triggered by the transplacental transfer of maternal antibodies. Despite its recognized clinical manifestations, the underlying pathogenesis remains incompletely understood. This study seeks to explore the disruption of the gut microbiota-host metabolism-immune axis in anti-Ro/La-positive neonates, and to assess its potential role in the development of NLE. METHODS: This multicenter, cross-sectional study included 90 neonates, divided into three groups: 30 with neonatal lupus erythematosus (NLE), 30 with positive antibodies but without clinical manifestations (No-NLE), and 30 healthy controls. We performed 16 S rRNA sequencing to analyze gut microbiota composition, untargeted plasma metabolomic profiling, and proteomic analysis to identify alterations associated with the pathogenesis of NLE. RESULTS: We identified significant alterations in the gut microbiota, plasma metabolome, and proteome profiles of anti-Ro/La-positive neonates. NLE infants exhibited marked enrichment of Enterobacteriaceae and depletion of Bifidobacterium and Clostridium butyricum. Metabolomic analysis revealed hyperactivation of β-alanine and purine metabolism, along with impaired α-linolenic acid metabolism and endocannabinoid signaling. Proteomic profiling indicated aberrant protein expression that modulated IFN signaling, particularly within the C-type lectin receptor pathway. Dysregulation of the spleen tyrosine kinase (SYK) and high-affinity immunoglobulin epsilon receptor subunit gamma (FCER1G) decoupling was observed, correlating with elevated IFN-α and NF-κB p65 levels. Integrated correlation analysis revealed significant associations among differential microbial taxa, plasma metabolites, and proteins. Notably, E. coli-associated metabolites and proteins displayed inverse relationships with those associated with C. butyricum. CONCLUSIONS: These findings represent comprehensive evidence of dysregulation along the "gut microbiota-host metabolism-immune" axis in neonatal lupus erythematosus (NLE), providing novel insights into the disease's underlying heterogeneity.

Humans↗
PubMed1992

Clinical manifestations of systemic lupus erythematosus, measures of disease activity, and long-term complications.

Studies of renal involvement in systemic lupus erythematosus continue to dominate the clinical literature. Reports of the prognostic significance of both clinical and histologic parameters at the time of renal biopsy are discussed. The potential impact of anticardiolipin antibodies on the development of renal insufficiency is described. The outcome of renal transplantation in patients with systemic lupus erythematosus is assessed in a study concerned with both allograft survival and recurrence of active nephritis in the transplanted kidney. The incidence and prognosis of various features of neuropsychiatric systemic lupus erythematosus are discussed, while the search for an accurate indicator of lupus involvement of the central nervous system continues. Magnetic resonance imaging and single-photon-emission computed tomography are considered. Abnormalities of pulmonary gas exchange are featured in several reports. Features of the antiphospholipid antibody syndrome are presented, stressing predisposition to thrombosis. The definition and characteristics of systemic lupus erythematosus disease flares is discussed, in relation to several recently developed disease activity indexes. The attempt to distinguish systemic lupus erythematosus activity from infection and preeclampsia is also considered. Finally, the association of systemic lupus erythematosus with the development of cancer is discussed.

Antiphospholipid Syndrome↗
PubMed1996

Acute myocardial infarction in a young woman with systemic lupus erythematosus.

A young woman was diagnosed with systemic lupus erythematosus at the age of 7 years and incurred an acute myocardial infarction at the age of 17 years. Her risk factors for coronary artery disease include hypertension, hypercholesterolemia, a relatively long disease duration, a fairly active disease as evidenced by the history of nephrotic syndrome and other organ system involvement, and a long history of prednisone use. It is difficult to determine the etiology of this patient's acute myocardial infarction without coronary artery histopathology, but aspects of her presentation (a history of virulent systemic lupus erythematosus, and the angiographic findings of ectasia and aneurysm) suggest that coronary arteritis was the etiology of her accelerated coronary artery disease and subsequent myocardial infarction. Acute myocardial infarction is an uncommon occurrence in premenopausal women less than 30 years old.35 These patients are typically found to have an associated systemic disease such as diabetes mellitus or familial hypercholesterolemia. Systemic lupus erythematosus is a less common systemic disease associated with premature coronary artery disease. Mechanisms of acute coronary syndromes in these patients include accelerated atherosclerosis, active coronary vasculitis, and/or vasospasm with superimposed thrombosis.

Adolescent↗
PubMed1999

Generalized lupus erythematosus profundus in a patient with genetic partial deficiency of C4.

Lupus erythematosus profundus (LEP) is an unusual variant of cutaneous lupus erythematosus (CLE)that is characterized by chronic, recurrent inflammation of the subcutaneous tissue leading to fibrosis. It is found in the settings of both discoid and systemic lupus erythematosus. Generalized forms are extremely rare. We present a case of generalized LEP associated with genetic partial C4-deficiency.

Adult↗
PubMed1984

Successful treatment of lupus erythematosus cystitis with DMSO.

Systemic lupus erythematosus patients sometimes present with pathologically confirmed lupus interstitial cystitis. Treatment with prednisone has not been observed to be successful. Two patients are presented who were successfully treated with intravesical dimethyl sulfoxide (DMSO).

Adult↗
PubMed1979

[Neuropsychic manifestations in systemic lupus erythematosus (author's transl)].

Thirty-three cases of systemic lupus erythematosus in women are studied in retrospect. Eight patients (24 percent) presented a total of ten neuropsychiatric episodes that could be attributed to the underlying disease. The neuropsychiatric manifestation that was most often observed was cerebrovascular stroke (40 percent) followed by psychic disturbances and convulsions. A facial paralysis coinciding with an hypertensive crisis was noticed in one case. Another patient presented a peripheral sensitive neuropathy. Five episodes occurred prior to diagnosis or during the first year of the disease. In two patients the neuropsychiatric manifestations constituted the initial symptom of systemic lupus erythematosus. ECG proved to be one of the most valuable diagnostic tools among the complementary studies. It was altered in cases of organic pathology and normal when disturbances were of psychic origin. The C3 fraction of the serum complement revealed a definite difference between patients with and without neuropsychiatric manifestations. Concentrations were lower in persons which psychic disturbances than in patients without them. The pathogenesis of neuropsychiatric manifestations of systemic lupus erythematosus is discussed. They cannot be attributed exclusively to coagulation disturbances nor to treatment with corticosteroids. The immediate prognosis of neuropsychiatric episodes was good: there was partial or total recuperation in eight of the ten episodes. However, the long-term course of systemic lupus erythematosus shows that these manifestations represent an unfavourable prognostic factor in this disease.

Adolescent↗
PubMed2004

The use of the British Isles Lupus Assessment Group (BILAG) index as a valid tool in assessing disease activity in childhood-onset systemic lupus erythematosus.

OBJECTIVES: The British Isles Lupus Assessment Group (BILAG) index is a standardized systemic lupus erythematosus (SLE) disease activity assessment. The main aim of this study was to correlate the BILAG index with laboratory measures of disease activity in childhood-onset SLE with and without biopsy-proven lupus nephritis. METHOD: Prospective observational comparison study of the BILAG index in 21 SLE patients under 18 yr of age over a 12-month period in a tertiary referral paediatric outpatient clinic. RESULTS: Eleven patients with lupus non-nephritis and 10 patients with lupus nephritis were reviewed. The lupus nephritis patients had significantly (P<0.001) more admissions over a similar time interval since diagnosis. The renal BILAG disease activity scores were significantly greater (P = 0.013) in the lupus nephritis group (range 1-9, median 3.0, compared with 0-3 and 1.0 in the lupus non-nephritis group). The total BILAG scores and patient visual analogue scores (VAS) were higher in the lupus nephritis groups, unlike the lower physician VAS, but these differences were not statistically significant compared with other laboratory indices of disease activity. CONCLUSIONS: The BILAG index is a useful tool in monitoring disease activity in children and adolescents with SLE. The data collected for the BILAG index can be used serially and effectively by different clinicians over time to enable recording of disease status at sequential assessments. The lower patient VAS in the lupus non-nephritis group was not significant and may reflect the patients' own perception of lethargy at times of increased disease activity.

Adolescent↗
PubMed1981

Systemic lupus erythematosus in pregnancy.

A retrospective analysis of the course of systemic lupus erythematosus in pregnant patients hospitalized at UCLA during a 15-year period provided important prognostic information. Women with systemic lupus erythematosus became pregnant if renal function was reasonably well preserved. Pregnancy infrequently posed a serious threat to the mother; the frequency of nonrenal complications in patients with systemic lupus erythematosus was very low, and the frequency of permanent deterioration of renal function was less than 10%. In contrast, the fetus was at high risk and was adversely affected even if mild renal involvement was detected in the mother. The prevalence of neonatal complications did not increase in the newborn; however, growth retardation did occur in the immediate neonatal period. The pharmacologic management of systemic lupus erythematosus does not require any important modifications in pregnant patients. During labor and in the postpartum period, however, an increase in the dosage of glucocorticoids may reduce postpartum exacerbations.

Female↗
PubMed1991

Hodgkin's disease associated with systemic lupus erythematosus.

We report on the rare association of Hodgkin's disease with systemic lupus erythematosus. Two years after the diagnosis of systemic lupus erythematosus, the patient developed upper abdominal pain, jaundice, splenomegaly, and fever of unknown origin. He had a rapidly fatal clinical course, despite being treated for systemic lupus erythematosus, cholecystitis, and possible sepsis. Autopsy revealed Hodgkin's disease, lymphocyte-depletion type, involving lymph nodes, liver, spleen, and bone marrow. The awareness of the association of Hodgkin's disease with systemic lupus erythematosus and its modes of presentation will help in the early diagnosis and management of such patients.

Hodgkin Disease↗
PubMed1976

[Headache in the pathology of small cerebral blood vessels: study of patients with systemic lupus erythematosus].

Seventy-two records of patients with systemic lupus erythematosus were reviewed retrospectively. Sixty-one fulfilled the criteria for the disease. Forty-six percent had clinical evidence of central nervous system involvement. The incidence of headache was compared in those with and without central nervous system lupus. No difference in the frequency of headache in the two groups was found and the incidence of hypertension, renal disease and steroid therapy was equally distributed among those with and without headache. A significantly higher incidence of hypertension was found in patients with central nervous system lupus erythematosus. We conclude that headache in systemic lupus erythematosus in the absence of neurologic symptoms or signs is no indication of central nervous system involvement by the process and that small vessel disease of the brain is not a cause of headache.

Adolescent↗
PubMed1985

The role of diet in animal models of systemic lupus erythematosus: possible implications for human lupus.

Studies of diet in the mouse model of SLE have established the beneficial effects of a low calorie, low fat diet in these animals as well as the importance of the specific source of dietary fat. The role of zinc in murine and human SLE is less clear. The reported improvement of patients with SLE and other related diseases on a low phenylalanine and tyrosine diet high in fish content, and the lupus inducing capacity of a nonphysiologic amino acid present in alfalfa are also reviewed. The need for carefully controlled prospective studies of diet in patients with SLE is noted, and a diet of potential therapeutic benefit is described.

Animals↗