Cases reported "Immune Complex Diseases"

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1/6. Immune-complex glomerulonephritis in a patient with mixed connective tissue disease.

    Renal involvement and hypocomplementemia in mixed connective tissue disease are reported to be rare. A patient is described here with mixed connective tissue disease and persistently low serum C'3 levels in whom renal insufficiency and nephrotic syndrome developed secondary to immune-complex glomerulonephritis. light microscopy of the renal biopsy specimen showed predominantly a membranous lesion. Immunofluorescent staining showed granular deposition along the basement membrane of immunoglobulin g, immunoglobulin m, fibrinogen and C3. Electron microscopy showed numerous electron-dense deposits along the glomerular capillary membrane and in the mesangium.
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keywords = mixed connective, connective
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2/6. Immune complex nephropathy in mixed connective tissue disease.

    Initial reports on the clinical spectrum of mixed connective tissue disease (MCTD) indicated that renal involvement was uncommon. Four of 20 patients with MCTD underwent renal biopsy and all showed evidence of immune complex trapping with a membranous glomerulonephritis in three patients. Clinically evident renal disease was absent in two patients. These findings suggest that renal involvement in MCTD may be more prevalent than previously indicated.
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ranking = 0.83333333333333
keywords = mixed connective, connective
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3/6. Idiopathic retroperitoneal fibrosis: a systemic connective tissue disease?

    Renal histology of a patient with idiopathic retroperitoneal fibrosis demonstrated a proliferative crescentic glomerulonephritis with intramembranous electron-dense deposits. These findings were interpreted as being consistent with an immune complex glomerulonephritis. Serologic studies revealed a positive antismooth muscle antibody titer of 1:80 and a weakly positive antinuclear antibody titer of 1:40. No distinct systemic disease was identified. While fibrosis of the retroperitoneum can occur in association with a number of distinct pathologic conditions and pharmacologic agents, the mechanism responsible for the development of the idiopathic variety of retroperitoneal fibrosis remains unclear. Our observation of an immune complex glomerulonephritis and the cumulative data on idiopathic retroperitoneal fibrosis are consistent with the concept that the fibrosis may be a local expression of an immunologically mediated systemic disease.
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ranking = 0.019975923904832
keywords = connective
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4/6. Renal involvement in mixed connective tissue disease. Report of 5 cases.

    5 cases with the compatible serological criteria of mixed connective tissue disease described earlier are presented. In 1 of them with a moderate degree of proteinuria, the renal biopsy disclosed membranous nephritis. However, despite the absence of overt clinical renal disease in the other 4 cases, biopsies disclosed membranous nephritis in 1 and mild mesangial proliferative glomerulonephritis in the remaining 3 cases. In the follow-up of these 4 cases, 2 subsequently developed abnormal urinalysis. Electron microscopic examinations demonstrated electron-dense deposits in glomeruli, and 4 of these patients also had microtubular structures in the endothelial cytoplasm. Contrarily to the original concept, our findings suggest that mixed connective tissue disease also induces immune complex disease.
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keywords = mixed connective, connective
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5/6. Musculoskeletal syndromes associated with malignancy.

    Evidence has been presented supporting a causal relationship between malignancies and musculoskeletal syndromes. This discussion has dealt primarily with lesser known relationships, more common associations such as hypertrophic osteoarthropathy and dermatomyositis being reviewed elsewhere. The ones discussed herein closely mimic primary connective tissue diseases and offer an insight into the study of the pathogenesis of these primary diseases. In view of the natural history of malignant disease, the hope for such patients arises from the physicians early diagnosis and treatment of the underlying malignancy. early diagnosis and treatment may in turn be entirely dependent on the physician's awareness of a musculoskeletal syndrome being the presenting feature of an otherwise occult neoplasm. Several connective tissue syndromes appear to predispose to the development of malignancy, and increasing evidence suggests that this development of malignancy may be further enhanced by immunosuppressive therapy.
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ranking = 0.0099879619524162
keywords = connective
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6/6. Multiple antinuclear antibodies in mixed connective tissue disease: report of a patient with an unusual antibody profile.

    A patient with antibodies to RNP, n-dna, Sm and PM-I is described. The first clinical manifestations of connective tissue disease appeared at age 10. A cystic pulmonary lesion, pericardial effusion and nephropathy appeared after 7 years of disease limited to skin and joints. Pulmonary, pericardial and renal disease all appeared to respond to corticosteroid therapy.
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ranking = 0.67166064764287
keywords = mixed connective, connective
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