Understanding Long-term Remission Off Therapy in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

Susan L. Hogan, Patrick H. Nachman, Caroline J. Poulton, Yichun Hu, Lauren N. Blazek, Meghan E. Free, J. Charles Jennette, Ronald J. Falk

Research output: Contribution to journalArticlepeer-review

7 Scopus citations


Introduction: In antineutrophil cytoplasmic antibody-associated (ANCA) vasculitis, relapse risk and long-term immunosuppressive therapy are problematic. Stopping immunotherapy has not been well described. Methods: The Glomerular Disease Collaborative Network ANCA vasculitis inception cohort was evaluated. Patients who stopped all immunotherapy and those continuously on immunotherapy (≥2 years) were included. Time to first period off therapy was modeled with end-stage kidney disease and death as competing risks to understand influences of stopping therapy. Cause-specific hazard ratios (HRs) with 95% confidence intervals (CI) and P values are reported. Models controlled for age, sex, ANCA specificity, organ involvement, diagnosis era, and treatments (yes/no). Repeated events analysis was used to assess the time-dependent variable of time off treatment on recurrent relapse with HRs, 95% CIs, and P values are reported (same control variables without treatments). Results: In 427 patients, 277 (65%) stopped therapy (median 20 months from initial induction); 14% for ≥2 different periods of time and 23% for periods ≥5 years. In multivariable models of time to discontinuation of treatment, women (HR 1.33; 95% CI 1.04–1.70; P = 0.024) and those treated with pulse methylprednisolone (HR 1.39; 95% CI 1.05–1.84; P = 0.020) were more likely to stop. The time-dependent variable of time off treatment was associated with fewer recurrent relapses (HR 0.51; 95% CI 0.41–0.63; P < 0.001). Conclusions: Stopping immunotherapy was common. Women and those treated with methylprednisolone stop treatment more often, but underlying mechanisms are unknown. Stopping treatment was associated with fewer relapses, suggesting that even without guidelines there may be benefits without an untoward detriment of relapse.

Original languageEnglish (US)
Pages (from-to)551-560
Number of pages10
JournalKidney International Reports
Issue number4
StatePublished - Apr 2019

Bibliographical note

Funding Information:
This work was supported in part by federal grant P01 DK058335, ANCA Glomerulonephritis: from Molecules to Man (Principal Investigator: R.J. Falk) from the National Institutes of Health/National Institute of Diabetes and Digestive and Kidney Diseases.

Publisher Copyright:
© 2019 International Society of Nephrology


  • ANCA
  • relapse
  • stopping therapy
  • time off therapy
  • vasculitis

PubMed: MeSH publication types

  • Journal Article


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