Die fibrilläre Glomerulonephritis als seltene Ursache eines nephrotischen Syndroms

Verlaufsbeobachtungen von fünf Patienten

authored by

Cornelia Blume, K. Ivens, P. Jehle, F. Keller, Udo Helmchen, B. Grabensee

Abstract

Fibrillary glomerulonephritis (FG) is a severe renal failure that normally leads to end-stage renal insufficiency within a few years. Patients with FG present a severe nephrotic syndrome that is associated with hypertonia, hematuria and usually a rapidly worsening renal function. Light microscopic analysis of renal histology in the past often led to miscellaneous wrong diagnoses, as membranous or membranoproliferative glomerulonephritis. The immunohistological findings mostly were divergent and also did not lead to a distinct diagnosis. Therefore, the electronmicroscopic analysis of renal biopsies is essential for the diagnosis of FG. FG has an incidence of 1 - 2% within all forms of glomerulonephritis. The actually presented literature concerning this rare kidney disease does not offer any proven therapeutical options. Generally, up to now, treatment of FG with immunosuppressants is not recommended since analyzing gains and risks, immunosuppressive therapies do not offer an obvious benefit for the patients. We observed five patients with FG, that, in one male patient, was associated with a successfully treated cancer and in another male patient with a spondylitis ancylans (M. Bechterew) without accompanying amyloidosis. In three of the five patients, severe limitation of renal function already occurred at the time of diagnosis. Whereas two of the five patients rapidly developed end-stage renal disease, another two patients, in which FG was associated with an acute extra- and intracapillary proliferation with crescents, were successfully treated with immunosuppressive therapy. Regarding the cases presented here, perspectives and therapeutical options of the FG are discussed.

External Organisation(s)

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

Type

Article

Journal

Nieren- und Hochdruckkrankheiten

Volume

32

Pages

214-224

No. of pages

11

ISSN

0300-5224

Publication date

01.05.2003

Publication status

Published

Peer reviewed

Yes

ASJC Scopus subject areas

Internal Medicine, Nephrology

Sustainable Development Goals

SDG 3 - Good Health and Well-being

Electronic version(s)

https://doi.org/10.5414/nhp32214 (Access: Closed)