Case Report

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

Table 1

Pediatric cases of atypical antiglomerular basement membrane disease in the literature.

PublicationAge/genderPresentationBiopsy findingsα3NC1 antibodiesTreatmentOutcome

Jen and Auron (present case)4/MNephrotic-range proteinuria, hematuria, normal renal functionOne glomerulus with segmental endocapillary hypercellularity, karyorrhexis, and small cellular crescentPositiveRituximab, steroids, MMFAsymptomatic with normal renal function at 15 months

Wilson and Dixon [13]14/MNo onset of renal failure within a large cohort of typical anti-GBM diseaseN/APositiveSteroidsLiving 1 yr with normal function
13/MNegativeSteroids, azathioprineLiving 18 mo with normal function
5/MNegativeSteroids, cyclophosphamideLiving 7 yr with normal function

Nagano et al. [14]8/FIncidental hematuria and proteinuria on screeningMild mesangial proliferation; no crescentsPositivePlasmapheresis, steroids, cyclophosphamideDecreased proteinuria and antibody titers; no long-term follow-up