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Clin J Am Soc Nephrol. 2011 Apr;6(4):775-84. doi: 10.2215/CJN.08300910. Epub 2011 Mar 24.

Fibrillary glomerulonephritis: a report of 66 cases from a single institution.

Author information

1
Mayo Clinic, Division of Anatomic Pathology, Hilton 10-20, 200 First Street SW, Rochester, MN 55905, USA. nasr.samih@mayo.edu

Abstract

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.

PMID:
21441134
PMCID:
PMC3069369
DOI:
10.2215/CJN.08300910
[Indexed for MEDLINE]
Free PMC Article

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