Crescentic glomerulonephritis due to linear IgA anti-glomerular basement membrane disease: report of a rare case
Braz. j. med. biol. res
; 57: e13466, fev.2024. graf
Article
in English
|
LILACS-Express
| LILACS
| ID: biblio-1557304
Responsible library:
BR1.1
ABSTRACT
Anti-glomerular basement membrane (GBM) disease is a rare and severe vasculitis that affects the glomerular and pulmonary capillaries and has an incidence of less than 2 cases per million individuals per year. Anti-GBM disease is mediated by autoantibodies against the α3 chain of type IV collagen. In the majority of cases, the autoantibodies are of the immunoglobulin G (IgG) class, with rare cases being mediated by immunoglobulin M (IgM) or immunoglobulin A (IgA); there are less than 15 IgA-mediated cases reported in the literature worldwide. The classic form of this disease manifests with rapidly progressive glomerulonephritis (RPGN), with or without pulmonary hemorrhage, and the diagnosis consists of identifying high titers of autoantibodies in the serum and/or deposited in the tissues. IgA antibodies are not identified in routine immunoassay tests, and renal biopsy with immunofluorescence is essential for diagnosis. We present a case of RPGN due to anti-GBM disease with linear IgA deposition, whose diagnosis was made exclusively by renal biopsy and with an unfavorable prognosis.
Full text:
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Collection:
International databases
Database:
LILACS
Language:
English
Journal:
Braz. j. med. biol. res
Journal subject:
Biology
/
Medicine
Year:
2024
Document type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Hospital Universitário Evangélico Mackenzie/BR