Atypical Antiglomerular Basement Membrane Disease in a Pediatric Patient Successfully Treated with Rituximab.

ABSTRACT: Classic antiglomerular basement membrane (anti-GBM) disease is an exceedingly rare but extremely aggressive form of glomerulonephritis, typically caused by autoantibodies directed against cryptic, conformational epitopes within the noncollagenous domain of the type IV collagen alpha-3 subunit. Pathologic diagnosis is established by the presence of strong, diffuse, linear staining for immunoglobulin on immunofluorescence microscopy. Recently, patients with atypical clinical and pathologic findings of anti-GBM disease have been described. These patients tend to have an indolent clinical course, without pulmonary involvement, and laboratory testing rarely reveals the presence of anti-GBM antibodies. Specific guidelines for the treatment and management of these patients are unclear. Here, we d

SUBMITTER: Jen KY 

PROVIDER: S-EPMC8313325 | biostudies-literature | 2021

REPOSITORIES: biostudies-literature