Long-term outcome of renal transplantation in childhood-onset anti-neutrophil cytoplasmic antibody-associated vasculitis

Background There have been a few reports of RTx for AAV in children; however, post-transplant recurrence rate and long-term prognosis remain unclear. Here, we describe the long-term outcomes of RTx in childhood-onset AAV. Methods We conducted a retrospective study of children who underwent RTx for AAV between 1999 and 2017 and had a follow-up period of >2 years. Results Seven patients consisting of three children with MPA and four with RLV were analyzed. Age at Dx was 5.9 (median; range, 4.1-14.5) years. PD was instituted in all patients, and median time on dialysis was 26 (range, 14-63) months. Age at RTx was 12.8 (median; range, 8.7-16.3) years. There were no recurrences of AAV noted during the median follow-up period of 7.0 (range, 2.7-18.8) years after RTx. Graft loss occurred in one patient due to non-adherence. Estimated glomerular filtration rate of the remaining patients at the last follow-up was 73.0 (median; range, 50.7-93.9) mL/min/1.73 m(2). No malignancies and deaths occurred during the observational period. Conclusions Our study suggests that RTx for AAV with ESRD is a potentially safe and effective treatment choice for children with AAV.