ANCA-negative EGPA: only eosinophils without vasculitis? Insights from anti-T2 biologics

Front Immunol. 2023 Nov 27:14:1325299. doi: 10.3389/fimmu.2023.1325299. eCollection 2023.

Abstract

The pathogenic role of p-ANCA in eosinophilic granulomatosis with polyangiitis (EGPA) is a long-standing matter of debate. In this work, we report our real-life experience with EGPA patients, treated with biologics targeting type 2 (T2)-eosinophilic inflammation (Mepolizumab, Benralizumab, Dupilumab). Interestingly, we observed EGPA extrarespiratory relapses only in p-ANCA-positive patients (2/5 cutaneous vasculitis, 3/5 constitutional symptoms), with new rise of p-ANCA and normal eosinophil blood count. Notably, revising our cohort with the new ACR 2022 criteria, these five patients were the only ones to satisfy the entry criterion of vasculitis' defined diagnosis at disease onset. These observations may suggest that biologics, selectively turning off T2 inflammation, may have unmasked p-ANCA exclusive role in the pathogenesis of vasculitis in EGPA. Therefore, we raise the question whether EGPA vasculitis exists only in p-ANCA-positive patients, and whether p-ANCA-negative disease is "only eosinophils without vasculitis".

Keywords: ANCA; EGPA; biologics; eosinophils; vasculitis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic
  • Biological Products*
  • Churg-Strauss Syndrome*
  • Eosinophils / pathology
  • Granulomatosis with Polyangiitis* / diagnosis
  • Humans
  • Inflammation / pathology

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Biological Products

Grants and funding

The author(s) declare financial support was received for the research, authorship, and/or publication of this article. The research leading to these results has received funding from the European Union – NextGenerationEU through the Italian Ministry of University and Research under PNRR – M4C2-I1.3 Project PE_00000019 “HEAL ITALIA” to Prof. Gianluca Moroncini.