ANCA Status or Clinical Phenotype — What Counts More?

Martin Windpessl, Erica L. Bettac, Philipp Gauckler, Jae Il Shin, Duvuru Geetha, Andreas Kronbichler

Research output: Contribution to journalReview articlepeer-review


Purpose of Review: There is ongoing debate concerning the classification of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. That is, whether classification should be based on the serotype (proteinase 3 (PR3)- or myeloperoxidase (MPO)-ANCA) or on the clinical phenotype (granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA)). To add clarity, this review focused on integration of the most recent literature. Recent Findings: Large clinical trials have provided evidence that a serology-based risk assessment for relapses is more predictive than distinction based on the phenotype. Research conducted in the past decade indicated that a serology-based approach more closely resembles the genetic associations, the clinical presentation (i.e., lung involvement), biomarker biology, treatment response, and is also predicting comorbidities (such as cardiovascular death). Summary: Our review highlights that a serology-based approach could replace a phenotype-based approach to classify ANCA-associated vasculitides. In future, clinical trials and observational studies will presumably focus on this distinction and, as such, translate into a “personalized medicine.”

Original languageEnglish (US)
Article number37
JournalCurrent rheumatology reports
Issue number6
StatePublished - Jun 2021


  • AAV
  • ANCA
  • Granulomatosis with polyangiitis
  • Microscopic polyangiitis
  • Vasculitis

ASJC Scopus subject areas

  • Rheumatology


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