Document Detail


Fibrillary glomerulonephritis: a report of 66 cases from a single institution.
MedLine Citation:
PMID:  21441134     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
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:
Type:  Journal Article     Date:  2011-03-24
Journal Detail:
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:
Created Date:  2011-04-08     Completed Date:  2011-08-18     Revised Date:  2012-04-02    
Medline Journal Info:
Nlm Unique ID:  101271570     Medline TA:  Clin J Am Soc Nephrol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  775-84     Citation Subset:  IM    
Copyright Information:
© 2011 by the American Society of Nephrology
Affiliation:
Mayo Clinic, Division of Anatomic Pathology, Hilton 10-20, 200 First Street SW, Rochester, MN 55905, USA. nasr.samih@mayo.edu
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MeSH Terms
Descriptor/Qualifier:
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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