Factors Associated With Pathogenicity of Anti-Glomerular Basal Membrane Antibodies: A Case Report.

ABSTRACT: Antiglomerular basement membrane (GBM) disease is known as a super-acute proliferative glomerulonephritis caused by auto-antibodies targeting the NC1 domain of the ?3 chain of type IV collagen.Here, we describe a case of atypical anti-GBM disease presenting as a dialysis-dependent acute renal failure with unusual mild glomerular involvement. We found that immunoglobulin G (IgG) deposits were restricted to the uncommon IgG2 and IgG4 subclasses, and that blood was positive for anti-GBM antibodies by immunofluorescence, but not by Enzyme Linked Immunosorbent Assay (ELISA). The patient was treated with plasma exchanges, corticosteroids, and cyclosphosphamide. He eventually regained a normal renal function.This case demonstrates that biopsy-proven anti-GBM disease can have reduced pathogenicity. Referring to previous studies of anti-GBM detection in the blood from healthy or minimally ill individuals, we discuss the antigenic specificities, the IgG subclasses, and the involvement of complement in this observation.We suggest that anti-GBM disease is a heterogeneous entity and that the study of IgG subclasses by immunofluorescence may help to distinguish categories with different severities.

SUBMITTER: Ossman R 

PROVIDER: S-EPMC4902534 | biostudies-literature | 2016 May

REPOSITORIES: biostudies-literature