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)

A 29-year-old Japanese male was diagnosed as having autoimmune hemolytic anemia (AIHA) in 1985, and systemic lupus erythematosus (SLE) in 1988. In 1989 a large mass was found of the right posterior area in the liver by computed tomography and ultrasonography. The liver biopsy specimen showed non-Hodgkin lymphoma, diffuse large cell type. No adenopathies or other extranodal involvements were detected. He was treated with five courses of CHOP, followed by involved field irradiation 24 Gy. On completion of chemoradiotherapy, the mass was smaller in size with a sign of partial necrosis. Gallium scan was negative suggesting the achievement of a complete remission. Primary hepatic lymphoma is extremely rare, and its occurrence in patients with AIHA and SLE has not been reported previously. We also review the previously reported cases of primary hepatic lymphoma with respect to its clinical management and abscess the therapeutic strategy of this unusual extranodal lymphoma.
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PMID:[Primary hepatic lymphoma in a patient with autoimmune hemolytic anemia and SLE]. 228 76

The influence of age on the prevalence of individual clinical manifestations of systemic lupus erythematosus (SLE) has not been adequately distinguished from racial or gender influences. Therefore, we examined variations in the clinical manifestations of SLE with age in a group of 361 patients. Multivariate regression techniques, including logistic regression and analysis of covariance, were used to identify clinical features associated with age, while controlling for important confounding factors, including race, gender, duration of followup, and treatment effects. Lymphopenia was found more frequently with increasing age, while malar rash, seizures, false-positive VDRL, thrombocytopenia (in whites), proteinuria (0.5-3.5 g/day), elevated antidouble stranded DNA antibodies, and hypocomplementemia were found less frequently. No age relationship was found for the prevalence of 16 of 24 clinical features examined, including the important disease manifestations of arthritis, serositis, psychosis, nephrotic-range proteinuria, renal failure, autoimmune hemolytic anemia, and leukopenia. The use of regression analysis allows the recognition of similarities and differences in cumulative clinical features of SLE due to age in isolation from the effects of other demographic factors.
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PMID:Age associated clinical manifestations of systemic lupus erythematosus: a multivariate regression analysis. 234 26

Autoimmune hemolytic anemia (AIHA), the first autoimmune disease to be recognized, is a manifestation of defective immune regulation. Although often associated with a lymphoid neoplasm or an overt immunologic disorder, AIHA frequently appears without apparent cause. Many patients with the "idiopathic" disease have been found to have various immunologic abnormalities in addition to the antibodies reacting with red cells. Although familial AIHA is uncommon, other autoimmune diseases and serologic abnormalities have been encountered in relatives of numerous patients. Few detailed family studies have been performed, but the available data suggest that predisposition to AIHA and to the associated immunologic disorders often is genetically transmitted. Less information is available about autoimmune thrombocytopenic purpura, in part because of the historic difficulty in recognizing autoantibodies that react with platelets. However, there is good evidence for genetically determined predisposing factors in some cases. Using the BFP reaction as an indicator, we add to the evidence that AIHA and autoimmune thrombocytopenia, like SLE, tend to occur in persons with a long-standing occult immunologic defect that often has a genetic basis. In our studies, 11 patients with AIHA or ITP had BFP reactions. The serologic abnormality in 4 had been known to precede the blood disorder by 6 to 44 years. Five of the patients had an additional disease believed to have an immunologic pathogenesis. Serologic abnormalities apart from the BFP reaction and the red cell or platelet antibodies were demonstrated in 9. Autoimmune diseases or serologic changes are known to have affected relatives of 5 patients, including 4 who had 1 or more relatives with BFP reactions. Serologic tests for syphilis were negative in 2 sibs with autoimmune thrombocytopenic purpura whose father had a chronic BFP reaction and thyroiditis, but all 3 had low levels of IgA and IgM. Lymphoproliferative disorders appeared in 3 of the patients with BFP reactions, and 2 had relatives with lymphoid neoplasms. A lymphoma occurred in a woman with cold agglutinin disease 21 years after the discovery of the erythrocyte antibody. Our observations support the view that an abnormality of cells of the immune system, often genetically determined, may predispose to serologic changes, immune deficiency, autoimmune diseases and neoplasia.
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PMID:Biologic false-positive serologic tests for syphilis and other serologic abnormalities in autoimmune hemolytic anemia and thrombocytopenic purpura. 264 9

The present report describes the clinical and laboratory profile of 82 previously healthy individuals who developed cytomegalovirus (CMV)-induced mononucleosis. Many of these patients posed initial diagnostic problems and were hospitalized with diagnoses such as fever of undetermined origin, active viral hepatitis, acute leukemia, probable systemic lupus erythematosus, autoimmune hemolytic anemia, and severe pancytopenia. These patients underwent a variety of diagnostic biopsies, including liver biopsies (6) and bone marrow aspirations (9). Four patients had exploratory laparotomies, 1 for a ruptured spleen, and another had a splenectomy following an erroneous initial diagnosis of agnogenic myeloid metaplasia. There was no apparent clinical response to a short course of steroid therapy in 3 of 5 cases and acyclovir in another. The vast majority of these patients demonstrated infectious mononucleosis-type reactive blood smears, negative heterophil antibody studies, mildly or moderately elevated aspartate aminotransferase activity, and evidence for subclinical hemolysis on serial specimens. The peak serum bilirubin levels were above 2.0 mg/dl in only 2 of 71 cases tested, both of the latter patients having significant hemolysis (hemoglobin values 8.6-9.3 g/dl). The CMV-IgM test had a high sensitivity for detection of CMV macroglobulins (positive in 81 of 82 cases). In contrast, complement-fixing antibodies to CMV showed diagnostic four-fold titer changes in only 39/82 cases (47.6%). Despite its great sensitivity, the CMV-IgM test is limited by a one-way crossreaction of acute Epstein-Barr virus (EBV)-IM sera and spurious positive reactions in some sera due to the presence of rheumatoid factors. Based on EBV-specific serologic studies, the 82 patients with CMV-IM could be divided into 4 groups: 3 patients without antibodies to EBV; 2) 69 patients with uncomplicated serologic data indicative of long-past EBV infections; (3) 6 patients with unusual antibody profiles, e.g., anti-D responses; and (4) 5 patients, including 1 originally susceptible to EBV, with apparent dual CMV/EBV infections. At the conclusion of our study, final diagnoses and initial hematologic data were correlated in 750 cases in which CMV macroglobulins were searched for. The vast majority of patients with active CMV infections initially demonstrated either markedly or moderately reactive peripheral blood smears. These data support our impression that diagnostic tests for CMV, as well as for EBV, are seldom indicated in symptomatic previously healthy patients whose blood smears during the acute phase (first several weeks) of their illnesses are either nonreactive or minimally reactive.
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PMID:Clinical and laboratory evaluation of cytomegalovirus-induced mononucleosis in previously healthy individuals. Report of 82 cases. 300 99

Immunotoxicologic studies have demonstrated that autoimmune responses and/or autoimmune diseases are induced in humans and experimental animals by chronic exposure to various chemicals. The present review is focused on seven groups of chemically induced human disorders, i.e. systemic lupus erythematosus, autoimmune hemolytic anemia, myasthenia gravis, pemphigus, glomerulonephritis, thyroiditis and hepatitis. Results obtained from studies of the available experimental counterparts of these diseases, i.e. those models obtained from the exposure of laboratory animals to various chemicals, are then analyzed. Finally, we present the lessons that can be derived from immunotoxicologic investigations regarding mechanisms of induction, heterogeneity of chemicals involved, humoral vs. cellular immune responses and genetic predisposition to chemically induced autoimmunity.
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PMID:Autoimmunity induced by chemicals. 304 16

Sixteen pediatric patients diagnosed with a variety of autoimmune-mediated hematocytopenias were treated with one to 50 courses of intravenous gamma globulin (IVIG), pH 4.25, over the course of one to 30 months. Thirteen patients had immune thrombocytopenic purpura (ITP), two had autoimmune neutropenia, and one had autoimmune hemolytic anemia. In one patient, chronic ITP was associated with systemic lupus erythematosis, and in a second patient, acute ITP was the presenting manifestation of infection with human immunodeficiency virus. Initial therapy consisted of 400 mg/kg/dose daily for five days for the first seven patients treated, and 1,000 mg/kg/dose daily for two days for the remaining nine patients. In 15 of 16 patients, there was a response to IVIG therapy. In nine of 16 patients, maintenance IVIG therapy for two to more than 30 months was required. Minimal toxicity was experienced in four of 210 separate infusions. Data are presented to support the use of IVIG in the management of childhood autoimmune disorders.
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PMID:Efficacy of intravenous gamma globulin in autoimmune-mediated pediatric blood dyscrasias. 311 7

Alfalfa sprouts can induce systemic lupus erythematosus (SLE) in monkeys. This property of alfalfa sprouts has been attributed to their non-protein amino acid constituent, L-canavanine. Occurrence of autoimmune hemolytic anemia and exacerbation of SLE have been linked to ingestion of alfalfa tablets containing L-canavanine. In this report we show that L-canavanine has dose-related effects in vitro on human immunoregulatory cells, which could explain its lupus-inducing potential. These effects include: 1) diminution of the mitogenic response to both phytohemagglutinin and concanavalin A but not to pokeweed mitogen, as determined in both thymidine incorporation and cell cycle studies, and 2) abrogation of concanavalin A-induced suppressor cell function, which results in increased release of both IgG and DNA binding activity into supernatants by cells from normal subjects and SLE patients. These immunoregulatory effects of L-canavanine may explain the induction or exacerbation of SLE by alfalfa.
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PMID:Effects of L-canavanine on T cells may explain the induction of systemic lupus erythematosus by alfalfa. 315 17

The authors report a patient with fulminant autoimmune hemolytic anemia due to a rare warm IgM autoagglutinin more reactive at 37 degrees C than at lower temperatures and secondary to systemic lupus erythematosis. The patient's clinical course and the serologic and immunochemical characteristics of the antibody are described, including the possibility that transfusions of small amounts of incompatible red cells may have contributed to the hemolysis. The consequences of using the initial serologic test results as the basis for therapy are discussed.
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PMID:Hemolytic warm IgM autoagglutinins in autoimmune hemolytic anemia. 368 54

Of 860 patients with systemic lupus erythematosus (SLE) who were evaluated during a 25-year-period, 16 (1.9%) underwent splenectomy. Twelve of these patients had steroid resistant thrombocytopenia. An excellent long-term outcome occurred in eight (67%), significant improvement occurred in three (25%), and one patient died who also had chronic active hepatitis and portal hypertension. In two of three patients (67%) with autoimmune hemolytic anemia, the condition was corrected by splenectomy; in the third patient there was some improvement, but reduced doses of corticosteroids were required. One patient with severe neutropenia and recurrent bacterial infection obtained lasting benefit following splenectomy. Histologic examination of the removed spleen was not helpful in corroborating the diagnosis of SLE in these well established cases. Splenectomy had no adverse affect upon other aspects of SLE, in particular upon renal function. The authors conclude that the indications for splenectomy have proven to be of value in selected SLE patients with autoimmune or hypersplenic cytopenia.
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PMID:Splenectomy in systemic lupus erythematosis. 372 70

Twenty four cases of pure red cell aplasia were reported. No underlying diseases were found in two cases. Of the 22 cases with secondary form, 10 were from infections, mostly gram negative organisms. Three cases had systemic lupus erythematosus, two had autoimmune hemolytic anemia. The following conditions were found in one each: thymoma, thyroid carcinoma, protein calorie malnutrition, rheumatoid arthritis, non-Hodgkin lymphoma and Sheehan's syndrome. Three patients died, two from uncontrolled infection, the other from uncontrolled SLE and subsequently systemic fungal infection. Only one of the 2 primary cases responded to immunosuppressive drugs. The majority of patients with underlying infections, PRCA resolved after the infections were treated. This is the first reported series of PRCA in Thailand.
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PMID:Pure red cell aplasia in Thailand: report of twenty four cases. 393 65


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