Fibrillary glomerulonephritis associated with crescents as a therapeutic challenge

verfasst von

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.

Externe Organisation(en)

Universitätsklinikum Ulm (UKU)
Universitätsklinikum Hamburg-Eppendorf
Universitätsklinikum Düsseldorf

Typ

Artikel

Journal

American Journal of Kidney Diseases

Band

40

Seiten

420-425

Anzahl der Seiten

6

ISSN

0272-6386

Publikationsdatum

08.2002

Publikationsstatus

Veröffentlicht

Peer-reviewed

Ja

ASJC Scopus Sachgebiete

Nephrologie

Ziele für nachhaltige Entwicklung

SDG 3 – Gute Gesundheit und Wohlergehen

Elektronische Version(en)

https://doi.org/10.1053/ajkd.2002.34548 (Zugang: Geschlossen)