Details
| Translated title of the contribution | Fibrillary glomerulonephritis as rare cause of a nephrotic syndrome - Observations in five patients |
|---|---|
| Original language | German |
| Pages (from-to) | 214-224 |
| Number of pages | 11 |
| Journal | Nieren- und Hochdruckkrankheiten |
| Volume | 32 |
| Issue number | 5 |
| Publication status | Published - 1 May 2003 |
| Externally published | Yes |
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
- Medicine(all)
- Internal Medicine
- Medicine(all)
- Nephrology
Sustainable Development Goals
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In: Nieren- und Hochdruckkrankheiten, Vol. 32, No. 5, 01.05.2003, p. 214-224.
Research output: Contribution to journal › Article › Research › peer review
}
TY - JOUR
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.
PY - 2003/5/1
Y1 - 2003/5/1
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.
AB - 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.
KW - Cyclophosphamide
KW - Fibrillary glomerulonephritis
KW - Immunosuppressants
KW - Intra- and extracapillary necrosis
KW - Nephrotic syndrome
KW - NSAIDs
KW - Steroids
UR - http://www.scopus.com/inward/record.url?scp=0037982807&partnerID=8YFLogxK
U2 - 10.5414/nhp32214
DO - 10.5414/nhp32214
M3 - Artikel
AN - SCOPUS:0037982807
VL - 32
SP - 214
EP - 224
JO - Nieren- und Hochdruckkrankheiten
JF - Nieren- und Hochdruckkrankheiten
SN - 0300-5224
IS - 5
ER -