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

Samih Nasr, Anthony M. Valeri, Lynn D. Cornell, Mary E. Fidler, Sanjeev M Sethi, Nelson Leung, Fernando Custodio Fervenza

Research output: Contribution to journalArticle

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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.

Original languageEnglish (US)
Pages (from-to)775-784
Number of pages10
JournalClinical Journal of the American Society of Nephrology
Volume6
Issue number4
DOIs
StatePublished - Apr 1 2011

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Glomerulonephritis
Kidney
Proteinuria
Autoimmune Diseases
Chronic Kidney Failure
Idiopathic Thrombocytopenic Purpura
Graves Disease
Nephrotic Syndrome
Hematuria
Immunosuppressive Agents
Crohn Disease
Cicatrix
Renal Insufficiency
Creatinine
Neoplasms
Multivariate Analysis
Hypertension
Carcinoma
Transplants
Biopsy

ASJC Scopus subject areas

  • Nephrology
  • Transplantation
  • Epidemiology
  • Critical Care and Intensive Care Medicine

Cite this

Fibrillary glomerulonephritis : A report of 66 cases from a single institution. / Nasr, Samih; Valeri, Anthony M.; Cornell, Lynn D.; Fidler, Mary E.; Sethi, Sanjeev M; Leung, Nelson; Fervenza, Fernando Custodio.

In: Clinical Journal of the American Society of Nephrology, Vol. 6, No. 4, 01.04.2011, p. 775-784.

Research output: Contribution to journalArticle

Nasr, Samih ; Valeri, Anthony M. ; Cornell, Lynn D. ; Fidler, Mary E. ; Sethi, Sanjeev M ; Leung, Nelson ; Fervenza, Fernando Custodio. / Fibrillary glomerulonephritis : A report of 66 cases from a single institution. In: Clinical Journal of the American Society of Nephrology. 2011 ; Vol. 6, No. 4. pp. 775-784.
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AB - 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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