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.
| Publication | Age/gender | Presentation | Biopsy findings | α3NC1 antibodies | Treatment | Outcome |
| Jen and Auron (present case) | 4/M | Nephrotic-range proteinuria, hematuria, normal renal function | One glomerulus with segmental endocapillary hypercellularity, karyorrhexis, and small cellular crescent | Positive | Rituximab, steroids, MMF | Asymptomatic with normal renal function at 15 months |
| Wilson and Dixon [13] | 14/M | No onset of renal failure within a large cohort of typical anti-GBM disease | N/A | Positive | Steroids | Living 1 yr with normal function | 13/M | Negative | Steroids, azathioprine | Living 18 mo with normal function | 5/M | Negative | Steroids, cyclophosphamide | Living 7 yr with normal function |
| Nagano et al. [14] | 8/F | Incidental hematuria and proteinuria on screening | Mild mesangial proliferation; no crescents | Positive | Plasmapheresis, steroids, cyclophosphamide | Decreased proteinuria and antibody titers; no long-term follow-up |
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