Fibrillary glomerulonephritis associated with crescents as a therapeutic challenge

authored by

Cornelia Blume, Katrin Ivens, Philip May, Udo Helmchen, Peter M. Jehle, Frieder Keller, Bernd Grabensee, Martin K. Riedel

Abstract

Most cases of fibrillary glomerulonephritis (FG) terminate in end-stage renal disease within a few years. We report on two female patients (41 and 50 years old) with the diagnosis of FG associated with crescentic glomerulonephritis, a combination found in 20% to 25% of cases of FG. A broad spectrum of infectious disease and systemic immunologic disorders could be ruled out by specific assays. Both patients had severely impaired renal function, nephrotic syndrome, and hypertension. Based on the biopsy finding with necrotizing FG, treatment was started with corticosteroids and cyclophosphamide. In both patients, renal function recovered markedly within 6 months of treatment, in one case remaining stable for 3.5 years. Whether or not cyclophosphamide treatment changed the course of the FG itself or counteracted the acute crescentic process cannot be determined from these two patients. Based on these promising preliminary findings and the poor prognosis of FG, however, we recommend cyclophosphamide treatment of patients with FG and additional crescentic glomerulonephritis. For a systematic evaluation of the therapeutic options in FG, a multicenter clinical trial should be conducted.

External Organisation(s)

University Hospital Ulm (UKU)
University Medical Center Hamburg-Eppendorf
University Hospital Düsseldorf

Type

Article

Journal

American Journal of Kidney Diseases

Volume

40

Pages

420-425

No. of pages

6

ISSN

0272-6386

Publication date

08.2002

Publication status

Published

Peer reviewed

Yes

ASJC Scopus subject areas

Nephrology

Sustainable Development Goals

SDG 3 - Good Health and Well-being

Electronic version(s)

https://doi.org/10.1053/ajkd.2002.34548 (Access: Closed)