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Eosinophilic granulomatosis with polyangiitis.

Alexandra Villa-Forte1

  • 1Department of Rheumatic and Immunologic Diseases, Cleveland Clinic - Center for Vasculitis Care and Research, Cleveland, Ohio, USA.

Postgraduate Medicine
|October 19, 2022
PubMed
Summary

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare vasculitis characterized by asthma and eosinophilia. Diagnosis can be challenging, and while treatment can control symptoms, relapses are common, necessitating further research for improved therapies.

Keywords:
ANCA-associated vasculitisChurg-strauss syndromeallergic angiitis and granulomatosisvasculitis

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Area of Science:

  • Rheumatology
  • Immunology
  • Pulmonology

Background:

  • Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare small to medium vessel necrotizing vasculitis.
  • It is often classified with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), but ANCA positivity is infrequent in EGPA.
  • Unique features include asthma and eosinophilia, distinguishing it from other vasculitides.

Purpose of the Study:

  • To review the epidemiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of EGPA.
  • To highlight the diagnostic challenges and unique characteristics of EGPA.
  • To discuss current treatment strategies and the need for improved therapies.

Main Methods:

  • Literature review of epidemiology, pathogenesis, clinical features, diagnosis, treatment, and prognosis of EGPA.
  • Analysis of diagnostic criteria and challenges, including the role of ANCA testing and tissue biopsy.
  • Synthesis of information on treatment approaches based on disease severity and prognostic factors.

Main Results:

  • EGPA is rare, with diagnosis often delayed due to evolving symptoms.
  • Asthma and eosinophilia are hallmark features; polyneuropathies are common and may require aggressive immunosuppression.
  • Cardiac involvement is the leading cause of mortality.

Conclusions:

  • Effective treatment can control EGPA symptoms, but relapses are frequent, and prolonged corticosteroid use is often required, especially for asthma.
  • Understanding EGPA's heterogeneity is crucial for developing more targeted and effective therapies.
  • Further research is needed to improve long-term management and outcomes for patients with EGPA.