PReS-FINAL-2361: Maintenance treatment in childhood granulomatosis with polyangiitis

Results 32 children were diagnosed with GPA since January 2000. Twenty-one girls and 11 boys, with a median age of 13.7 years at diagnosis. ANCA was positive in 30 children (26 c-ANCA with 25 anti-PR-3, 4 p-ANCA with 4 anti MPO) and 2 were ANCA negative (1 antiPR3 positive). Eight children had limited disease and 24 systemic disease. All systemic patients were treated with pulses cyclophospamide iv (mean 7 pulses) and methylprednisone (mean 5 pulses) iv, and 6 children received plasmapheresis. Maintenance treatment in this group consisted of MTX in 7, AZA in 14, MMF in 3 children. In the limited disease group, treatment consited of oral prednisone in all, MTX in 7 children and AZA in 1. Relapses were seen in 14 children. One child did not receive any treatment at time of relapse. Two children with limited disease relapsed, both while still on MTX. 11 children with systemic disease were still on treatment, MTX in 4, AZA in 5, MMF in 2.