Die fibrilläre Glomerulonephritis als seltene Ursache eines nephrotischen Syndroms: Verlaufsbeobachtungen von fünf Patienten

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External Research Organisations

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

Translated title of the contributionFibrillary glomerulonephritis as rare cause of a nephrotic syndrome - Observations in five patients
Original languageGerman
Pages (from-to)214-224
Number of pages11
JournalNieren- und Hochdruckkrankheiten
Volume32
Issue number5
Publication statusPublished - 1 May 2003
Externally publishedYes

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.

ASJC Scopus subject areas

Sustainable Development Goals

Cite this

Die fibrilläre Glomerulonephritis als seltene Ursache eines nephrotischen Syndroms: Verlaufsbeobachtungen von fünf Patienten. / Blume, Cornelia; Ivens, K.; Jehle, P. et al.
In: Nieren- und Hochdruckkrankheiten, Vol. 32, No. 5, 01.05.2003, p. 214-224.

Research output: Contribution to journalArticleResearchpeer review

Blume, C, Ivens, K, Jehle, P, Keller, F, Helmchen, U & Grabensee, B 2003, 'Die fibrilläre Glomerulonephritis als seltene Ursache eines nephrotischen Syndroms: Verlaufsbeobachtungen von fünf Patienten', Nieren- und Hochdruckkrankheiten, vol. 32, no. 5, pp. 214-224. https://doi.org/10.5414/nhp32214
Blume, C., Ivens, K., Jehle, P., Keller, F., Helmchen, U., & Grabensee, B. (2003). Die fibrilläre Glomerulonephritis als seltene Ursache eines nephrotischen Syndroms: Verlaufsbeobachtungen von fünf Patienten. Nieren- und Hochdruckkrankheiten, 32(5), 214-224. https://doi.org/10.5414/nhp32214
Blume C, Ivens K, Jehle P, Keller F, Helmchen U, Grabensee B. Die fibrilläre Glomerulonephritis als seltene Ursache eines nephrotischen Syndroms: Verlaufsbeobachtungen von fünf Patienten. Nieren- und Hochdruckkrankheiten. 2003 May 1;32(5):214-224. doi: 10.5414/nhp32214
Blume, Cornelia ; Ivens, K. ; Jehle, P. et al. / Die fibrilläre Glomerulonephritis als seltene Ursache eines nephrotischen Syndroms : Verlaufsbeobachtungen von fünf Patienten. In: Nieren- und Hochdruckkrankheiten. 2003 ; Vol. 32, No. 5. pp. 214-224.
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T1 - Die fibrilläre Glomerulonephritis als seltene Ursache eines nephrotischen Syndroms

T2 - Verlaufsbeobachtungen von fünf Patienten

AU - Blume, Cornelia

AU - Ivens, K.

AU - Jehle, P.

AU - Keller, F.

AU - Helmchen, Udo

AU - Grabensee, B.

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N2 - 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.

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JO - Nieren- und Hochdruckkrankheiten

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SN - 0300-5224

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ER -

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