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Abstract: SA-PO673

Predictors of Treatment Resistance and Relapse in Childhood-Onset ANCA-Associated Vasculitis: A Nationwide Japanese Survey

Session Information

  • Pediatric Glomerular Disease
    November 09, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Pediatric Nephrology

  • 1700 Pediatric Nephrology


  • Hirano, Daishi, The Jikei university school of medicine, Tokyo, Japan
  • Ishikawa, Tomoaki, Nara Medical Universtty, Nara, Japan
  • Sato, Mai, National Center for Child Health and Developement, Tokyo, Japan
  • Inaba, Aya, Yokohama City University Medical Center, Yokohama, Japan
  • Ito, Shuichi, Department of Pediatrics, Graduate School of Medicine, Yokohama City University, Yokohama, Japan

Treatment resistance and relapse in childhood-onset antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) are major challenges for pediatricians. The aim of this study was to assess the predictive factors for treatment resistance and relapse in a nationwide cohort of Japanese patients with childhood-onset AAV.


Forty-five consecutive patients with childhood-onset AAV were recruited for inclusion in this study. The value of various demographic and clinical parameters for the prediction of treatment resistance and relapse were analyzed.


The cohort consisted of 45 children; 34 (76%) were female, 36 (80%) had MPA, 9 (20%) had GPA, 78% were MPO-ANCA-positive, 20% were PR3-ANCA-positive. After the induction phase treatment, treatment resistance occurred in 20 (44%). Multivariable logistic regression models revealed that decreased estimated glomerular filtration rate (eGFR) at presentation predicted treatment resistance (odds ratio [OR] 30.71, 95% confidence interval [95% CI] 2.30-410.43, P = 0.010). Relapse occurred in 14 (31%) of 45 patients in whom remission was achieved and was independently associated with PR3-ANCA (OR 6.95, 95% CI 1.05-46.04, P = 0.044). Although not significant, male tended to be associated with relapse (p = 0.05).


Our findings highlight the important effect of severity of renal disease at presentation as predictors of treatment resistance. Increased risk for relapse appears to be related to the presence of anti-PR3 antibody seropositivity.