Complement activation associated with ADAMTS13 deficiency may contribute to the characteristic glomerular manifestations in Upshaw-Schulman syndrome.

•Upshaw-Schulman syndrome (USS) is a congenital thrombotic thrombocytopenic purpura with mutations in the ADAMTS13 gene.•In USS cases, glomerular segmental sclerosis was predominantly localized in the perihilar region.•The severity of glomerular injury in USS may be associated with deficient ADAMTS13 expression and complement activation.