| Fibrillary glomerulonephritis: a report of 66 cases from a single institution. | |
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MedLine Citation:
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PMID: 21441134 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND AND OBJECTIVES: Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease. Most previously reported cases were idiopathic. To better define the clinical-pathologic spectrum and prognosis, we report the largest single-center series with the longest follow-up. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: The characteristics of 66 FGN patients who were seen at Mayo Clinic, Rochester, between 1993 and 2010 are provided. RESULTS: The mean age at diagnosis was 53 years. Ninety-five percent of patients were white, and the female:male ratio was 1.2:1. Underlying malignancy (most commonly carcinoma), dysproteinemia, or autoimmune disease (most commonly Crohn's disease, SLE, Graves' disease, and idiopathic thrombocytopenic purpura), were present in 23, 17, and 15% of patients, respectively. Presentation included proteinuria (100%), nephrotic syndrome (38%), renal insufficiency (66%), hematuria (52%), and hypertension (71%). The most common histologic pattern was mesangial proliferative/sclerosing GN followed by membranoproliferative GN. During an average of 52.3 months of follow-up for 61 patients with available data, 13% had complete or partial remission, 43% had persistent renal dysfunction, and 44% progressed to ESRD. The disease recurred in 36% of 14 patients who received a kidney transplant. Independent predictors of ESRD by multivariate analysis were older age, higher creatinine and proteinuria at biopsy, and higher percentage of global glomerulosclerosis. CONCLUSIONS: Underlying malignancy, dysproteinemia, or autoimmune diseases are not uncommon in patients with FGN. Prognosis is poor, although remission may occur in a minority of patients without immunosuppressive therapy. Age, degree of renal impairment at diagnosis, and degree of glomerular scarring are predictors of renal survival. |
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Authors:
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Samih H Nasr; Anthony M Valeri; Lynn D Cornell; Mary E Fidler; Sanjeev Sethi; Nelson Leung; Fernando C Fervenza |
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Publication Detail:
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Type: Journal Article Date: 2011-03-24 |
Journal Detail:
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Title: Clinical journal of the American Society of Nephrology : CJASN Volume: 6 ISSN: 1555-905X ISO Abbreviation: Clin J Am Soc Nephrol Publication Date: 2011 Apr |
Date Detail:
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Created Date: 2011-04-08 Completed Date: 2011-08-18 Revised Date: 2012-04-02 |
Medline Journal Info:
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Nlm Unique ID: 101271570 Medline TA: Clin J Am Soc Nephrol Country: United States |
Other Details:
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Languages: eng Pagination: 775-84 Citation Subset: IM |
Copyright Information:
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© 2011 by the American Society of Nephrology |
Affiliation:
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Mayo Clinic, Division of Anatomic Pathology, Hilton 10-20, 200 First Street SW, Rochester, MN 55905, USA. nasr.samih@mayo.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Aged Blood Protein Electrophoresis Female Fluorescent Antibody Technique Glomerulonephritis / blood, complications, drug therapy, pathology* Humans Kidney Failure, Chronic / etiology Male Microscopy, Electron Middle Aged Proportional Hazards Models |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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