Severe acute kidney failure due to double-seropositive anti-GBM glomerulonephritis in a child: a case for rituximab therapy.
Lien Dossche, Lieselot Peremans, Ann Raes et al.
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Case in brief
An 11-year-old girl presented with rapidly progressive glomerulonephritis and kidney failure requiring kidney replacement therapy. Serum testing detected both anti-GBM antibodies and myeloperoxidase-ANCA, and kidney biopsy showed crescents in over 90% of glomeruli. She received high-dose corticosteroids, plasmapheresis, and rituximab; kidney function recovered and dialysis was discontinued.
What made this case unusual
The child had double-seropositive anti-GBM disease with an exceptionally severe renal presentation, yet recovered kidney function after treatment that included rituximab without cyclophosphamide.
Diagnostic / clinical pearl
In rapidly progressive glomerulonephritis, testing for both anti-GBM antibodies and ANCA can identify double-seropositive disease, including in children with severe kidney failure.
Why it matters
This case suggests rituximab may be a therapeutic option without cyclophosphamide in children with severe double-seropositive anti-GBM disease; the report does not establish comparative efficacy.