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Dividing the Janus vasculitis? Pathophysiology of eosinophilic granulomatosis with polyangitis

Benjamin Chaigne1, Benjamin Terrier1, Nathalie Thieblemont2

  • 1INSERM, U1016, Institut Cochin, Paris, France; CNRS, UMR8104, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France; Université Paris Descartes, Faculté de Médecine, Pôle de Médecine Interne, Centre de référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France.

Autoimmunity Reviews
|October 28, 2015
PubMed

Insights

Eosinophilic granulomatosis with polyangitis (EGPA) has two main types based on MPO-ANCA presence. This review explores EGPA pathogenesis and the factors causing these distinct disease phenotypes.

Area of Science:

  • Rheumatology
  • Immunology
  • Pathology

Background:

  • Eosinophilic granulomatosis with polyangitis (EGPA) is a rare vasculitis.
  • EGPA is classified under anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV).
  • EGPA presents with two phenotypes based on myeloperoxidase (MPO)-ANCAs.

Purpose of the Study:

  • To review the pathogenesis of EGPA.
  • To differentiate EGPA from similar conditions like asthma and other AAVs.
  • To explore mechanisms behind EGPA's two distinct phenotypes.

Main Methods:

  • Literature review of EGPA pathogenesis.
  • Comparative analysis of EGPA with other vasculitides and allergic conditions.
  • Synthesis of current knowledge on EGPA disease mechanisms.

Main Results:

  • MPO-ANCAs are found in 31-38% of EGPA patients, linked to vasculitic phenotypes.
  • Absence of MPO-ANCAs is associated with increased risk of cardiac involvement.
  • The underlying reasons for this phenotypic dichotomy remain unclear.

Conclusions:

  • Understanding EGPA pathogenesis is crucial for distinguishing it from other diseases.
  • Further research is needed to elucidate the mechanisms driving the two EGPA phenotypes.
  • Identifying these mechanisms may lead to targeted therapies for EGPA patients.
Keywords:
ANCA-MPOEosinophiliaEosinophilic granulomatosis with polyangitisTh2 responseVasculitis

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