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.
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: Brian G M Durie; Robert A Kyle; Andrew Belch; William Bensinger; Joan Blade; Mario Boccadoro; J Anthony Child; Raymond Comenzo; Ben Djulbegovic; Dorotea Fantl; Gosta Gahrton; Jean Luc Harousseau; Vania Hungria; Douglas Joshua; Heinz Ludwig; Jayesh Mehta; Angelina Rodrique Morales; Gareth Morgan; Amara Nouel; Martin Oken; Raymond Powles; David Roodman; Jesus San Miguel; Kazuyuki Shimizu; Seema Singhal; Bhawna Sirohi; Pieter Sonneveld; Guido Tricot; Brian Van Ness Journal: Hematol J Date: 2003
Authors: Jordan L Rosenstock; Glen S Markowitz; Anthony M Valeri; Giuseppe Sacchi; Gerald B Appel; Vivette D D'Agati Journal: Kidney Int Date: 2003-04 Impact factor: 10.612
Authors: Claudia Yuste; Eduardo Gutierrez; Angel Manuel Sevillano; Alfonso Rubio-Navarro; Juan Manuel Amaro-Villalobos; Alberto Ortiz; Jesus Egido; Manuel Praga; Juan Antonio Moreno Journal: World J Nephrol Date: 2015-05-06