TPE is selectively used as an adjunctive therapy in children with severe or refractory AAV, particularly in those with advanced kidney involvement, and was associated with significant reduction in disease activity scores, though persistent renal abnormalities remained frequent.
Paediatric AAV patients who received TPE had significantly higher disease activity at diagnosis compared to those who did not receive TPE.
TPE was associated with a statistically significant reduction in Paediatric Vasculitis Activity Score.
The primary indication for TPE in paediatric AAV was severe kidney involvement.
Severe extra-renal organ manifestations occurred predominantly in the TPE-treated group.
One patient died from a catheter-related infectious complication during TPE treatment.
Persistent renal abnormalities remained frequent following TPE despite improvement in acute disease activity.
This research examined how therapeutic plasma exchange (TPE) — a procedure that filters harmful antibodies and proteins from the blood — is used in children with a rare, life-threatening condition called ANCA-associated vasculitis (AAV), which causes inflammation of blood vessels and can damage multiple organs. Researchers looked at 26 children with AAV across multiple centers, comparing 11 who received TPE alongside standard immunosuppressive medications to 15 who received standard medications alone. They found that the children who received TPE were much sicker at the start, with significantly higher disease activity scores, worse kidney function, and more widespread organ involvement including the lungs, gut, heart, and skin. Among children who received TPE, disease activity scores dropped substantially — from a median score of 12 down to 4.5 — suggesting the treatment helped control the most dangerous features of the disease in the short term. However, kidney problems often persisted even after treatment, reflecting how severely the kidneys were damaged before TPE was started. One child died from a blood infection related to the catheter used for the procedure, underscoring that TPE carries serious risks. This research suggests that TPE may play a role as an add-on therapy for the most severely affected children with AAV, particularly those with advanced kidney disease or other life-threatening organ involvement, but it does not cure the underlying kidney damage. The findings highlight the need for early recognition of severe disease and careful monitoring for complications during treatment. Given the small number of patients studied, larger studies are needed to better define which children are most likely to benefit from TPE.
Menentoğlu B, Küçükali B, Demirkan F, Kılbaş G, Türkmen &, Küçük E, et al.. (2026). Therapeutic plasma exchange in paediatric ANCA-associated vasculitis: a multicentre real-world experience.. Rheumatology (Oxford, England). https://doi.org/10.1093/rheumatology/keag497