Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Anemia occurs in more than one half of patients with systemic lupus erythematosus and is usually attributed to "chronic disease." Approximately 10% of patients with a positive Coombs' test manifest clinically significant hemolysis. Leukopenia affects both granulocytic and lymphocytic lines and may be caused by autoantibodies. Nevertheless, enhancement of B lymphocyte function occurs in active disease, perhaps due to a loss of regulatory T cells. Most patients have increased production and increased peripheral destruction of thrombocytes, with a normal circulation platelet count. Thrombocytopenia is usually caused by increased destruction. Qualitative abnormalities of platelet aggregation also occur. Circulation anticoagulants are not rare; however, spontaneous bleeding is uncommon. The anticoagulants, immunoglobulins directed against clotting factors, assume importance for invasive procedures. Most clinically significant hematopoietic abnormalities can be suppressed by corticosteroids; however, splenectomy, or immunosuppressive agents, or both, may be indicated for patients who respond inadequately to corticosteroids.
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PMID:Hematologic aspects of systemic lupus erythematosus. Current concepts. 83 48

Since cellular and humoral immune mechanisms were shown to be involved in the pathogenesis of rheumatic diseases, efforts have been made to influence these systems therapeutically. Aside from suppression of the immune response, studies have been conducted to evaluate to what extent immunostimulation might be of value in the treatment of rheumatic diseases. Levamisole, chemically a simple synthetic agent recently shown to have immunorestorative capacities in anergic cancer patients, has been administered in several rheumatic diseases. The drug was administered either continuously or intermittently in a daily dose of 150 mg over several months. In about half of the rheumatoid arthritis patients a significant improvement was noted. Beneficial effects and sometimes even dramatic improvement lere observed in some patients with systemic lupus erythematosus, ankylosing spondylitis and Reiter's syndrome. Levamisole did not alter the course of psoriatic arthritis. Significant adverse reactions were leukopenia and allergic skin reactions. Other side effects were mild and did not require interruption of drug intake. The use of levamisole in rheumatic diseases is still experimental, but with accumulating experience it may provide a true improvement in control of rheumatic diseases.
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PMID:[Therapy of inflammatory-rheumatic diseases with levamisol, an immunity modulating substance]. 108 57

An 11-month-old girl had systemic lupus erythematosus with a widespread skin rash. She manifested five of the criteria necessary for the diagnosis: oral ulcerations, alopecia, positive lupus erythematosus preparations, convulsions, and thrombocytopenia with leukopenia. Direct immunofluorescence showed deposits of IgG and C3 at the dermoepidermal junction of both abnormal and clinically uninvolved skin, and in subepithelial deposits along the glomerular basement membrane.
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PMID:Systemic lupus erythematosus in a 1-year-old child. 113 Mar 30

Direct cutaneous immunofluorescence microscopical examination of uninvolved skin is an important diagnostic test in systemic lupus erythematosus. Its prognostic significance is undetermined. In twenty-four patients there was an increased incidence of leukopenia, hypocomplementaemia, and LE cells in patients with positive skin immunofluorescence. Positive cutaneous immunofluorescence of uninvolved skin was correlated with the most severe forms of lupus renal disease, membranous glomerulonephritis, and diffuse proliferative glomerulonephritis.
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PMID:The significance of a positive cutaneous immunofluorescence test in systemic lupus erythematosus. 120 77

Two cases of irreversible bone marrow failure are described, one with rheumatoid disease and one with systemic lupus erythematosus. Each case was associated with prior chlorambucil administration, effective in controlling the clinical manifestations (total dosage 398 and 1,764 mg respectively). The irreversibility of the bone marrow depression in the two cases presented stands in contrast to published assurances that chlorambucil-associated leukopenia is dose-related and readily reversible. The cases illustrate that chlorambucil therapy should not be continued after initial leukopenia, until peripheral counts or marrow cellularity has returned to normal. Titration of drug dosage and leukocyte count, as frequently employed with cyclophosphamide and other alkylating agents, must be presumed hazardous. Additional studies are needed to determine if irreversible bone marrow depression is dose-related or idiosyncratic.
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PMID:Irreversible bone marrow failure with chlorambucil. 120 73

A 21-year-old woman was admitted to our hospital because of high fever, cough, and headache. Other clinical manifestations included hepatosplenomegaly, anemia, leukopenia, and mild liver dysfunction. As she had been diagnosed to have systemic lupus erythematosus (SLE) previously, we first suspected that this disease had become exacerbated. However, the titers of anti-EB virus (VCA-IgG) and some other viruses were high on admission, and hemophagocytosis by macrophages was observed on bone marrow examination. Therefore, she was diagnosed to have virus-associated hemophagocytic syndrome (VAHS). Prednisolone therapy was then initiated, but the patient responded poorly. We next tried gamma globulin, and the clinical findings and laboratory data were improved. Our patient showed a very favourable clinical course following gamma globulin therapy, suggesting that steroid and gamma globulin should be considered as a treatment even in the early stages of this syndrome in adults.
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PMID:[Steroid and gamma globulin therapy against virus-associated hemophagocytic syndrome]. 127 36

A 14-year-old female developed systemic lupus erythematosus (SLE)-like symptoms, rash, fever, leukopenia and positive anti-nuclear antibody (ANA) two weeks after administration of carbamazepine (CBZ; Tegretol) used against benign Rolandic epilepsy. Clinical symptoms and leukopenia normalized after discontinuation of CBZ and administration of prednisolone at 40 mg. The cases of CBZ-induced SLE reported in the literature were reviewed.
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PMID:Carbamazepine-induced systemic lupus erythematosus-like disease. 129 28

Since 1981 when anti-Ro (SS-A) and/or anti-La (SS-B) antibodies were described to be present in infants with neonatal lupus erythematosus (NLE) and their mothers, subsequent studies have demonstrated the almost universal association of NLE with either or both of these autoantibodies. To our best knowledge, three cases of NLE were reported to be negative in anti-Ro (SS-A) and anti-La (SS-B) antibodies. We report one infant born to a mother with systemic lupus erythematosus (SLE). He had neonatal pancytopenia (thrombocytopenia, anemia, and leukopenia) which got resolved after intravenous immunoglobulin (IVIG) administration. Both anti-Ro (SS-A) and anti-La (SS-B) antibodies were not detectable in his serum by immunodiffusion method while other such as RNP (nonspecific, including U1,U2,U3,...,U6), Sm and Scl-70 antibodies were all positive. This mother had all the above antibodies detectable in her serum. After excluding other possibilities, his pancytopenia was most likely to be attributed to neonatal lupus. We suggest that autoantibodies such as RNP and Sm antibodies may play an important role in the pathogenesis of thrombocytopenia of NLE.
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PMID:Neonatal lupus erythematosus with negative anti-Ro and anti-La antibodies: report of one case. 129 48

The influence of age on autoimmunity was studied in a model in which experimental systemic lupus erythematosus (SLE) is induced in normal mice by the injection of a human monoclonal anti-DNA antibody expressing a common idiotype designated 16/6 Id. The resulting disease is expressed by the production of a variety of autoantibodies and clinical manifestations characteristic to human SLE. Female BALB/c mice, at ages of 2 and 12 months, were immunized with the 16/6 Id. Mice were tested periodically for the presence of autoantibodies. The production of all autoantibodies tested was significantly lower in the older mice as compared to the group of young mice. Clinical manifestations which included leukopenia, increased erythrocyte sedimentation rate and proteinuria were similar in both age groups. Kidney evaluations revealed differences among the two groups of mice. While in all kidney sections of young mice multiple immune complex deposits were detected, in the group of older mice half had similar pathology while the rest either were negative or had only segmental and partial glomerular immune complex depositions. Thus, aging is associated with a decrease in the capacity to respond to the pathogenic anti-DNA, 16/6 Id, by the production of antibodies and autoantibodies and in the expression of a milder disease.
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PMID:The influence of aging on the induction and manifestations of experimental systemic lupus erythematosus. 143 Jan 4

Circulating lupus anticoagulant (LA) is associated with thrombosis in large and small vessels. To determine how often the presence of LA is associated with thrombosis within the renal microcirculation, 33 patients with systemic lupus erythematosus (SLE), renal dysfunction, and LA were identified over a 25-year period (LA group) and 32 patients with renal SLE but with normal gross coagulation screen were matched for age, sex, and biopsy timing (C group). Prevalences of serositis, neuropsychiatric illness, leukopenia, thrombocytopenia, hemolysis, anti-DS-DNA elevation, and complement reduction were similar. Arthritis was less and biologic false-positive (BFP) syphilis serology more common in LA. More LA patients had thrombotic events (LA 39% v C 13%; P = 0.014); bleeding episodes, including postbiopsy, were similar. At biopsy, hypertension (LA 55%, C 41%), serum creatinine (mean +/- SD: LA 186 +/- 168 mumol/L [2.1 +/- 1.9 mg/dL] v C 150 +/- 168 mumol/L [1.7 +/- 1.9 mg/dL]) and proteinuria (LA 2.6 +/- 3.1 g/24 h v C 3.1 +/- 2.7) were similar. Lesions by World Health Organization (WHO) class, activity, and chronicity indices, as well as immunofluorescence (IF) and electron microscopy (EM) findings, were not significantly different. Occlusive glomerular, arteriolar, and arterial fibrin thrombi, along with varying degrees of renal thrombotic microangiopathy, were seen in five of 33 patients with LA, but zero of 32 C patients (P = 0.053); three of these five patients died soon after biopsy. Overall, mortality was not different between LA and C. We conclude that the majority of patients with SLE, renal dysfunction, and LA exhibit renal morphologic findings indistinguishable from patients without LA. However, a significant minority of LA patients have thrombotic microangiopathy in their biopsy, which is accompanied by a worse prognosis.
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PMID:Lupus anticoagulant in systemic lupus erythematosus: a clinical and renal pathological study. 144 58


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