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 64-year-old female patient with a prosthetic mitral valve suffered from recurrent typical angina and dyspnea. Left heart catheterization excluded a dysfunction of the prosthesis and coronary artery disease (CAD). A stress thallium scan demonstrated an ischemic reaction. An antimyosin scintigram was positive, indicating myocytal membrane disruption. Serological tests were suspicious for systemic lupus erythematosus (SLE). Therefore, an endomyocardial biopsy (EMB) was performed and a severe alteration of an intramyocardial artery, comparable with chronic SLE, was diagnosed. EMB is a useful diagnostic tool in patients with typical chest pain, positive thallium and anti-myosin scintigrams, however exclusion of CAD.
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PMID:[Diagnosis by endomyocardial biopsy: angina pectoris as a manifestation of lupus erythematosus]. 175 Feb 31

Pulmonary hypertension due to recurrent thromboembolism is a rare disease but life-threatening. We evaluated 18 patients (11 female, 7 male) with this pathology between 1973 and 1991. We compared clinical features and evolution of our patients with the ones of the literature. The mean interval between beginning of symptoms and diagnosis was 5 years (range 1-10 years) and the most frequent symptom was increasing dyspnoea. In 2 of our patients there were well definite predisposing causes for thromboembolism (intracardiac catheters), 6 of the others had a previous episode of acute pulmonary embolism. Mean pulmonary arterial pressure was 50 mmHg and low output was present in 8 of these. Lung perfusion scintigraphy was diagnostic in 98% of cases showing segmental defects and pulmonary angiography confirms diagnosis revealing abrupt cut-off of cases showing segmental defects and pulmonary angiography confirms diagnosis revealing abrupt cut-off a major pulmonary artery. Angiographic evaluation of thrombus extent and location was difficult. In a small number of patients was found lupus anticoagulant, deficiency of protein C, of protein S and of antithrombin III. Mortality in medical treatment was 39% at a mean follow-up of 4-5 years. Progression of pulmonary hypertension was due to recurrent pulmonary embolism only in 30-40% of cases. The role of caval filter is not well established. Thromboendarterectomy shows immediate good results at short time but the long-term results are not known.
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PMID:[Thromboembolic pulmonary hypertension]. 184 71

Five patients with pulmonary hemorrhage associated with SLE were studied. Chest radiographs showed extensive alveolar opacity immediately after hemoptysis or dyspnea in four patients. In one patient an interstitial pattern seen initially progressed rapidly to an alveolar pattern. Three patients were successfully treated with corticosteroids and are doing well at present. The diagnosis and treatment of pulmonary hemorrhage is vital to patients with SLE. In the appropriate clinical setting chest radiographic findings of acute alveolar disease should suggest pulmonary hemorrhage, and empiric treatment with corticosteroids should be considered.
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PMID:Pulmonary hemorrhage in patients with systemic lupus erythematosus. 185 2

Two cases of nocardiosis with underlying SLE are presented. Both were female patients aged 19 and 34 years and had been treated with steroid and endoxan for some time. Death from lesions in the respiratory tract and right kidney with growth of Nocardia asteroides at autopsy was noted in the first case. The second patient exhibited fever with dyspnea and subsequent peripheral neuromuscular dysfunction. Disseminated nocardial abscesses in multiple organs including lungs, liver, spleen, lymph nodes and subcutaneous tissue were disclosed postmortem. Moreover, intravascular dissemination of the organisms had resulted in thrombosis of several blood vessels supplying the spinal cord resulting in widespread myelomalacia. Recent infarction of a few spinal nerve roots was also observed.
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PMID:Nocardiosis: report of 2 cases with review of literature in Thailand. 202 81

A 22-year-old man was admitted because of hemosputum and progressive dyspnea with 3 attacks of chest pain and dyspnea over the previous 4 months. Chest roentgenography showed pulmonary infarction of the left lower lobe, and the diagnosis was confirmed by pulmonary perfusion and inhalation scintigraphy and pulmonary arteriography. Thrombolytic therapy was performed, but no significant effect could be obtained and anticoagulant therapy was performed continuously. No deep-vein thrombosis could be seen. He was considered to have autoimmune hemolytic anemia with lupus anticoagulant on the basis of auto-antibody data. Lupus anticoagulant is an antibody to phospholipid, and it is suggested that a decrease in the production of prostanoid in the endothelium causes thrombosis. In this case, as the patient showed a low level of 6-keto-PGF1 alpha in the blood, it is suggested that one of the etiological factors of pulmonary thromboembolism is a disorder of prostacyclin production in the endothelium, causing thrombosis by lupus anticoagulant.
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PMID:[A case of pulmonary thromboembolism due to circulating lupus anticoagulant]. 212 Apr 98

Hematologic abnormalities are common in association with collagen diseases, specially Systemic Lupus Erythematosus and include anemia, neutropenia, thrombocytopenia with alterations in lymphocyte subpopulations. On the other hand, patients with unexplained fibrosis of the bone marrow (the syndrome of idiopathic myelofibrosis or primary myelofibrosis) have clinical and laboratory evidence of immunologic dysfunction. Clinical findings include the presence of arthritis, vasculitis and erythema nodosum. Laboratory abnormalities include the presence of circulating immune complexes, antinuclear antibodies, positive direct Coombs test, elevated latex fixation and a circulating lupus type anticoagulant. Total hemolytic complement markedly depressed has also been reported. These data suggest that immunologic mechanisms associated with activation of the complement system play an important role in the disease process of some patients with agnogenic myeloid metaplasia with myelofibrosis. A review of the literature revealed that myelofibrosis occurring in the setting of collagen diseases is rare. However, a role for immunologic factors in the pathogenesis of myelofibrosis is also supported by the patients with coincident well defined collagen disease and myelofibrosis. In this report, we present two patients with such an association. Case 1 was a 58-year-old male with a two year duration history of rheumatic arthritis. He had bone erosions on hands, splenomegaly and myelofibrosis. Rheumatoid factor (latex) was positive: 1:2560. He had positive LE cells and hypocomplementemia: 37 CH50/ml (NV 70-150). The patient did not meet criteria for SLE. Case 2 was a 36-year-old female admitted because of dyspnea and fever. Diagnosis of myeloid metaplasia with myelofibrosis and progressive systemic sclerosis had been made four years before hand.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Coexistence of myelofibrosis and collagen diseases]. 213 Feb 12

A 43-year-old woman was admitted to our hospital because of fever and dyspnea. She had facial rash, hair-loss, arthralgia, urinary casts, thrombocytopenia, lymphocytopenia, and deposits of IgG, IgM, C3 in the epidermis. SLE was diagnosed. She also had severe dyspnea, which gradually progressed. In spite of steroid pulse therapy and cyclophosphamide, she eventually died about two months after admission. Chest X-ray film showed bilateral ground-glass shadow and volume-loss. High-resolution CT of the lung showed marked high density area with airbronchogram in the posterior part and a gradually enlarging slight high density area in the anterior area. Pathological findings of the posterior area were atelectasis and dense fibrosis. The findings of the anterior area were collapsed alveoli and dilated alveolar ducts. These findings might suggest that alveolar collapse play a major role in the course of pulmonary fibrosis of this patient.
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PMID:[A case of acute lupus pneumonitis followed by high-resolution CT]. 221 22

A 17-year-old female with a 5-year history of disseminated lupus erythematosus has remained without immunosuppressive therapy for the last 3 years. She was admitted to the hospital for acute abdominal pain, generalized edema, and rapidly developing dyspnea and somnolence. Although all symptoms were consistent with active SLE, septicemia was suspected because of leukocytosis (20,000/microliters), greatly elevated C-reactive protein (45 mg/dl), and normal complement values (C3 0.74 g/l, C4 0.21 g/l). Directly after bacterial blood cultures were prepared, a combined treatment was instituted consisting of plasmapheresis (3 x 2.1 l against fresh frozen plasma), antibiotics, prednisolone, and cyclophosphamide following the last plasmapheresis. Within three days cerebral function returned to normal, edema improved, and CRP fell to 0.5 mg/dl. The blood cultures and pericardial effusion displayed meningococcal colonies.
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PMID:Successful therapy of meningococcal sepsis in acute disseminated lupus erythematosus with plasmapheresis, immunosuppression, and antibiotics. 223 29

Heterogeneity of the CD4 antigen epitopes has been occasionally reported in healthy subjects, in patients affected by autoimmune diseases, such as Graves' disease and systemic lupus erythematosus (SLE), and recently also in HIV-infected subjects. A 63-year-old woman was admitted to the hospital because of dyspnea, autoimmune thrombocytopenia and serum antinuclear autoantibodies. The clinical course and X-ray films of the chest were consistent with idiopathic pulmonary fibrosis. The evaluation of peripheral blood lymphocyte subsets showed low CD4+ cells by use of OKT4 (Ortho Mune) monoclonal antibody (30%, normal range 35-45) and normal values of the same CD4+ subset by use of OKT4A (Ortho Mune) and Leu3a (Becton Dickinson) monoclonal antibodies (48%, normal range 45-55), which are specific for a different epitope of CD4 molecule. These differences indicate that the patient is heterozygous for the OKT4 epitope deficiency on CD4+ lymphocytes surface. The routine use of a panel of monoclonal antibodies, such as OKT4, OKT4A, Leu3a, which recognize different CD4 epitopes, is suggested in order to perform an accurate evaluation of CD4+ lymphocyte subset in patients affected by immune-mediated disorders other than Graves' disease and SLE.
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PMID:[Heterogeneity of epitopes of the CD4 molecule in a female patient with idiopathic pulmonary fibrosis]. 248 2

A case of pseudohypoparathyroidism (PHP) type 1 with systemic lupus erythematosus (SLE) is reported. A 36-year-old women was admitted to our hospital with the complaints of dyspnea, arthralgia, tetany and tremor. Laboratory findings on admission showed; leukopenia, thrombocytopenia, hypocalcemia, positive antinuclear, anti-RNP, anti-Sm antibodies. A diagnosis of PHP type 1 was made from the findings of Albright's osteodystrophy and Ellsworth-Howard test. On the basis of various auto antibodies and clinical findings, the patient was diagnosed as SLE, too. She was started on a therapy of prednisolone at a dose of 40 mg per day. Her clinical manifestations immediately became better, and her laboratory findings subsequently improved. Up to the present, the case report of overlapping syndrome of PHP type 1 and SLE is very rare. Both PHP and SLE were considered to be in a category of autoimmune disease and the relationship between PHP and SLE was discussed.
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PMID:[A case of pseudohypoparathyroidism type 1 with systemic lupus erythematosus]. 281 8


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