Abstract / Summary
Abstract Background ANCA-associated vasculitis (AAV) is rare in children, and evidence guiding therapeutic apheresis (TA) is almost exclusively derived from adult studies. We evaluated TA use and renal outcomes in a multicenter pediatric cohort and assessed current clinical practice in a nationwide survey of pediatric nephrologists. We hypothesized that TA would not provide substantial long-term renal benefit beyond contemporary induction therapy. Methods Clinical outcomes of pediatric patients with renal involvement in AAV treated at four major German centers over 12 years were retrospectively analyzed, comparing patients who received TA with those who did not. Current clinical practice was assessed through an online case-based survey within the Society of German Pediatric Nephrology. Results Nineteen patients were included (57.9% microscopic polyangiitis, 42.1% granulomatosis with polyangiitis), with a median age at diagnosis of 14.2 years. Six (31.6%) underwent TA. Median eGFR at diagnosis tended to be lower with TA (45 vs. 63 ml/min/1.73 m²), without reaching statistical significance. Renal function improved in both groups, reaching median eGFRs of 80 and 96 ml/min/1.73 m² at 36 months, respectively. Among surveyed German pediatric nephrology experts, 80–93% reported that they would use TA during treatment, particularly for severe or organ-threatening disease. Conclusions Clinical outcome data did not demonstrate a clear renal benefit of TA in pediatric AAV, whereas the expert survey indicates that TA remains widely used for organ-threatening disease.