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

Six immunologic tests were conducted on a large population of dogs with a variety of diseases. The test results were analyzed retrospectively and correlated to the clinical and final pathologic diagnoses. The results indicated that rheumatoid factor and the direct antiglobulin (Coombs) test were both sensitive and specific for the diagnoses of canine rheumatoid arthritis and autoimmune hemolytic anemia. The antinuclear antibody test was useful in supporting the diagnosis of systemic lupus erythematosus (SLE) only when both antibody titer and the staining pattern were taken into consideration. The lupus erythematosus cell test was specific but not sensitive when used to confirm a diagnosis of canine SLE. Cellulose acetate serum electrophoresis and immunoelectrophoretic techniques were each useful in supporting the diagnosis of multiple myeloma. Epizootiologic data collected in this study indicated that dogs with primary immunologic disease had a poor prognosis. A female predisposition was observed in cases of canine autoimmune hemolytic anemia and SLE. Ovariectomy seemed to prevent the development of canine SLE.
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PMID:Efficacy of immunoserodiagnostic procedures in the recognition of canine immunologic diseases. 722 94

Chronic lymphocytic leukemia (CLL) is a malignant clonal expansion of CD5+B lymphocytes. The CD5+B lymphocytes have been postulated to produce autoantibodies. CLL patients may demonstrate features of autoimmunity including autoimmune hemolytic anemia. However, the origin of the autoantibodies causing the hemolysis is not clear. The present studies were performed to determine whether these autoantibodies are the products of the neoplastic B-CLL clones. Immunoglobulins (Ig) were eluted from washed red blood cells (RBC) obtained from two CLL patients at the time they had autoimmune (DAT-direct antiglobulin test-positive) hemolytic anemia. The light chain phenotypes of these eluted autoantibodies were determined and found to be monotypic with exact correlation to the light chain expressed on the surface of the B-CLL clones. Elutions from RBC of DAT negative patients or normal volunteers failed to demonstrate measurable amounts of Ig. In contrast, Ig eluted from RBC obtained from SLE patients with DAT positive hemolytic anemia found to be polyclonal autoantibodies exhibiting both light chain types. Furthermore, CD5+B lymphocytes obtained from the same two CLL patients (DAT+) produce, in vitro understimulation with phorbal myristate acetate (PMA), monoclonal antibodies which react and bind to RBC. Thus these studies provide direct evidence demonstrating that the antibodies causing the autoimmune hemolytic anemia in our two CLL patients are the products of the B-CLL neoplastic clones.
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PMID:Mechanism of autoimmune hemolytic anemia in chronic lymphocytic leukemia. 769 May 17

The administration of intravenous immunoglobulin (IVIG) in immune and autoimmune diseases led us to use this agent to ameliorate or prevent the consequences of non-ABO incompatible transfusions in patients who need this form of therapy. IVIG (400 mg/kg/day) was infused within 24 h of transfusion in 5 patients with: (1) intestinal angiodysplasia, gastrointestinal bleeding, and anti-Kpb; (2) paroxysmal nocturnal hemoglobinuria, anti-c, anti E, anti Fyb, anti-K and autoantibodies; (3) lymphoma and autoimmune hemolytic anemia (AIHA); (4) systemic lupus erythematosus (SLE), AIHA, and anti-D, and (5) SLE and AHIA. A sustained increase in hematocrit was noted and no transfusion reaction developed in any of the cases. A single dose of pretransfusion IVIG may therefore be a useful therapeutic alternative in patients for whom no compatible blood is available. Patients with severe anemia, allo- and autoantibodies, either showing hemolysis in their pathophysiology or not, cause a serious problem in any transfusion center, especially when dealing with emergencies. In order to reduce the risks of incompatible transfusions, different modalities have previously been attempted, all with poor results. In 1989 we reported the successful use of pretransfusional high-dose intravenous immunoglobulin (IVIG) in a patient with gastrointestinal bleeding and anti-Kpb. The transfusion of incompatible red blood cells improved the anemia and allowed the exploratory laparotomy to take place. A protocol was then developed based on this case administering pretransfusion IVIG in high doses for patients for whom no compatible blood (non-ABO) is available.
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PMID:High-dose intravenous immunoglobulin in non-ABO transfusion incompatibility. 780 11

In autoimmune diseases striking abnormalities of T and B cell activation and of cytokine production are present. In 14 patients with autoimmune hemolytic anemia (AIHA), idiopathic or in the course of: lymphoma, B hepatitis, carcinoma, drug therapy (alpha-methyldopa), systemic lupus erythematosus (SLE), and not yet submitted to immunosuppressive therapy, the PBL proliferative response to PHA and the IL1 alpha, IL2, IL4 and IL2R serum levels have been valued. While the stimulation index of PBL was strongly reduced in 10 cases (64 +/- 56 vs 138 +/- 45 in the control group), IL1 alpha, IL2 and IL2R were greatly increased in all the patients, and IL4 in 5 (IL1 alpha :199 +/- 268 pg/ml in patients vs 0.30 +/- 0.2 in controls; IL2:716 +/- 311 pg/ml vs 16 +/- 4; IL4:29 +/- 13 pg/ml vs 13 +/- 7; IL2R:1233 +/- 471 U/ml vs 256 +/- 114). Cytokine serum levels were not related with the associated disease, with the CD4+ and CD8+ cells absolute number or with PBL blastogenic in vitro response. The high serum levels of cytokines and IL2R suggest that in AIHA there exist a CD4+ lymphocyte hyperactivation (the low proliferative response of PBL might imply a temporary functional exhaustion of T lymphocytes) as in the other autoimmune diseases.
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PMID:High cytokine serum levels in patients with autoimmune hemolytic anemia (AIHA). 785 62

In the past decade, interest in the potential clinical significance of lupus anticoagulant (LA) has grown tremendously. Recent reviews from the Western countries have found an average frequency of 34% for LA in patients with systemic lupus erythematosus (SLE). By using various laboratory procedures, namely, standard and diluted activated partial thromboplastin time, kaolin clotting time, tissue thromboplastin inhibition test and platelet neutralization test, we found the frequency of LA in 91 consecutive Thai SLE patients to be 17.5%, compared with 0.8% in the age-matched normal control population. The presence of LA was significantly associated with disease activity (p = 0.01). A statistically significant association was also observed between the presence of LA and convulsive disorders (p = 0.04), thrombocytopenia (p = 0.001) and autoimmune hemolytic anemia (p = 0.02).
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PMID:Lupus anticoagulant in Thai systemic lupus erythematosus patients. 788 86

Antiphospholipid antibodies (APA) comprise a family of immunoglobulins characterized by their pattern of reactivity in a number of laboratory tests. Included in this family are lupus anticoagulant (LA) anticardiolipin antibodies (ACA) and antibodies causing biologic false positive serologic tests for syphilis (BFP-STS). LA and ACA occur in a variety of conditions, including other autoimmunes disorders, infectious diseases, neoplasic disorders, in association with certain drugs and in otherwise healthy individuals. Clinical interest in LA and ACA is increasing. Antiphospholipid antibody syndrome is characterized by a triad of clinical features which include fetal loss, thromboembolic disease and thrombocytopenia. Other clinical manifestations related with APA are livedo reticularis, cutaneous necrosis, hemolytic anemia, heart valve disease, chorea, migraine and obstetric problems as fetal growth retardation, pre-eclampsia, post-partum serositis or neonatal thrombosis or catastrophic antiphospholipid syndrome. Therapy is mainly directed against the widespread and diverse manifestations associated with the obstruction of small and large vessels. Long-term treatment with oral anticoagulation therapy is advised, even if the venous or arterial occlusion occurred many years previously. In patients with primary antiphospholipid syndrome there is no evidence that the prophylactic administration of steroids or immunosuppression will prevent thromboembolic events. Although the administration of more energetic immunosuppression with cyclophosphamide in pulse form is effective in reducing elevated antibody levels, there is usually a rapid rebound to pretreatment levels shortly after discontinuation of the therapy. A history of recurrent fetal loss requires mandatory treatment during pregnancy. Although the actual prospective risk of pregnancy loss in women with antiphospholipid syndrome and prior pregnancy loss is unknown, it may exceed 60%. Because of this many investigators have treated women with antiphospholipid syndrome with either antiplatelet agents, immunosuppressive agents, or anticoagulants in an attempt to improve pregnancy outcome. Unfortunately, there is no unequivocal proof that any of these therapies are fully efficacious. Despite varying treatment protocols, the live birth rate with treatment was 70%, similar to that reported in the recent randomized clinical trial. Thrombocytopenia and autoimmune hemolytic anemia in patients with APA are treated similarly as patients without APA. Treatment of asymptomatic patients isn't indicated, because only approximately 10-15% of patients with APA developed complications.
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PMID:Clinical and therapeutic aspects associated to phospholipid binding antibodies (lupus anticoagulant and anticardiolipin antibodies). 798 46

We report on a patient with splenic lymphoma of B-cell origin who developed autoimmune hemolytic anemia (AIHA). IgM lambda M-protein, IgM anticardiolipin antibody (ACA), and lupus anticoagulant (LA) were detected in the serum, and direct Coombs' test showed autoantibodies of the IgG1 and IgG2 subclasses on red blood cells (RBC). In in vitro culture, tumor cells isolated from the spleen produced only IgM ACA, which was enhanced by IL-6 but not by IL-4 or IL-5. The levels of ACA and LA decreased after splenectomy and chemotherapy; the strength of the direct Coombs' test, however, did not change. These findings indicated that in this patient the lymphoma cells produced IgM lambda ACA, but not autoantibodies of the IgG1 and IgG2 subclasses against RBC. It was also suggested that IL-6 might at least partially stimulate the production of ACA.
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PMID:Multiple autoantibody production in a patient with splenic lymphoma. 801 67

The purpose of our pilot study was to evaluate the short-and long-term efficacy of T-lymphocyte depletion in the management of patients with refractory, systemic autoimmune diseases. Nine patients with severe, therapy-resistant autoimmune diseases were subjected to T-cell depletion procedure using polyclonal anti-T-cell antibodies combined with peroral administration of azathioprine and/or cyclosporine. The proband group consisted of 4 patients with systemic lupus erythematosus, 3 with progressive systemic sclerosis, and 2 with rheumatoid arthritis. Administration of polyclonal anti-T-cell antibodies was performed at a single occasion via a central venous catheter during 9-10 days. Immunological analyses of T-cell phenotypes and function and assessment of organ function (kidneys, lungs, bone-marrow) has been performed prospectively in all the patients studied. This treatment resulted in prompt and long-lasting (mean follow-up time: 25.6 months) improvement of autoimmune hemolytic anemia, glomerulonephritis, lung fibrosis, skin and joint involvements in the majority of cases. Adverse effects of this treatment included two episodes of infection (E. coli and Cytomegalovirus) and three cases of serum sickness, and were all easily managed. We suggest that this treatment modality adopted from transplant rejection therapy could be employed in cases of severe autoimmune diseases unresponsive to regular immunosuppressive treatment.
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PMID:Use of anti-thymocyte globulin in the management of refractory systemic autoimmune diseases. 826 27

A 34-year-old woman had suffered from systemic lupus erythematosus (SLE) and autoimmune hemolytic anemia (AIHA) in the teen age. She developed progressive ptosis of the eyelids, and difficulty in swallowing and speaking for several years. Endocrinological studies showed primary hypothyroidism. A serum IgG level was elevated (1,973 mg/dl), and antinuclear antibody, thyroid test and microsome test were positive. A muscle biopsy showed massive inflammatory cell infiltrates in the perivascular area in addition to some myopathic change; some variation in fiber size. Immunological staining demonstrated most of these inflammatory cell infiltrates were CD3+ cells and CD4+ cells were counted more than CD8+ cells (CD4/CD8 = 2.3). The diagnoses were confirmed as oculopharyngeal myopathy and Hashimoto's disease. In addition, she had suffered from SLE and AIHA. Therefore we conclude that manifestation of this myopathy may be associated with some autoimmune process.
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PMID:[Oculopharyngeal myopathy with autoimmune disease]. 833 98

This paper reviews the different series of thymectomies performed in patients with autoimmune diseases other than myasthenia gravis. It is possible that thymectomy can decrease the activity of T-helper lymphocytes or, alternatively, it may enhance the activity of T-suppressor lymphocytes, whose function is depressed in autoimmune diseases. Thymectomy was performed empirically for systemic lupus erythematosus and rheumatoid arthritis. The therapeutic benefits were questionable. Conflicting results were reported for thymectomy against autoimmune hemolytic anemia. Several trials were conducted to assess the therapeutic value of thymectomy in multiple sclerosis. Benefits were achieved only in relapsing-remitting but not in chronic-progressive multiple sclerosis. The effect of thymectomy in autoimmune diseases associated with myasthenia gravis were also reported. The authors conclude that thymectomy as treatment for autoimmune diseases other than myasthenia gravis is not elective therapeutic choice and it is acceptable only in selected cases.
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PMID:Thymectomy as treatment of autoimmune diseases other than myasthenia gravis. 848 Mar 42


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