Prognostic factors and long-term outcome with anca-associated kidney vasculitis in childhood

Marta Calatroni, Filippo Consonni, Marco Allinovi, Alessandra Bettiol, Natasha Jawa, Susanna Fiasella, Dritan Curi, Sarah Abu Rumeileh, Leonardo Tomei, Laura Fortunato, Elena Gelain, Davide Gianfreda, Elena Oliva, Guido Jeannin, Chiara Salviani, Giacomo Emmi, Monica Bodria, Renato A. Sinico, Gabriella Moroni, Giuseppe A. RamirezEnrica Bozzolo, Enrico Tombetti, Sara Monti, Claudia Bracaglia, Giulia Marucci, Serena Pastore, Pasquale Esposito, Maria G. Catanoso, Barbara Crapella, Giovanni Montini, Rosa Roperto, Marco Materassi, Giovanni M. Rossi, Salvatore Badalamenti, Rae S.M. Yeung, Paola Romagnani, Gian M. Ghiggeri, Damien Noone, Augusto Vaglio

Research output: Contribution to journalArticlepeer-review


Background and objectives ANCA-associated vasculitis is extremely rare in children. We report the clinicopathologic features, long-term outcomes, and prognostic factors of a large pediatric cohort of patients with ANCA-associated kidney vasculitis. Design, setting, participants, & measurements This retrospective study included 85 consecutive patients with kidney biopsy specimen–proven ANCA-associated vasculitis from tertiary referral centers in Italy and Canada. Kidney biopsy specimens were categorized as focal, crescentic, sclerotic, or mixed, according to the Berden classification. The prognostic significance of baseline clinical, laboratory, and histologic findings was analyzed with respect to kidney failure or CKD stage 3–5/kidney failure. Results A total of 53 patients had microscopic polyangiitis (62%), and 32 had granulomatosis with polyangiitis (38%). Rapidly progressive GN was the most frequent presentation (39%); a third of the patients also had nephrotic-range proteinuria. Kidney biopsy specimens were classified as focal in 21% of the patients, crescentic in 51%, sclerotic in 15%, and mixed in 13%. Remission-induction therapies included cyclophosphamide in 78% of patients. A total of 25 patients (29%) reached kidney failure. The median (interquartile range) time to kidney failure or last follow-up was 35 (6–89) months in the whole cohort, and 73 (24–109) months among the patients who did not reach this outcome. Patients whose biopsy specimens showed sclerotic histology had significantly shorter kidney survival (hazard ratio, 11.80; 95% confidence interval, 2.49 to 55.99) and survival free of CKD stage 3–5 (hazard ratio, 8.88; 95% confidence interval, 2.43 to 32.48), as compared with those with focal/mixed histology. Baseline eGFR, low serum albumin, hypertension, central nervous system complications, and sclerotic histology, which reflected severe kidney involvement, were associated with both kidney failure and CKD stage 3–5/kidney failure at unadjusted analysis; no independent prognostic factors emerged at multivariable analysis. Conclusions Children with ANCA-associated kidney vasculitis often have aggressive presentation; a third of such children progress to kidney failure and this usually occurs early during follow-up. A severe clinical presentation is associated with the development of CKD or kidney failure.

Original languageEnglish
Pages (from-to)1043-1051
Number of pages9
JournalClinical Journal of the American Society of Nephrology
Issue number7
Publication statusPublished - Jul 2021

ASJC Scopus subject areas

  • Epidemiology
  • Critical Care and Intensive Care Medicine
  • Nephrology
  • Transplantation


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