At the Heart of Eosinophilic Granulomatosis with Polyangiitis: into Cardiac and Vascular Involvement

Curr Rheumatol Rep. 2022 Nov;24(11):337-351. doi: 10.1007/s11926-022-01087-1. Epub 2022 Oct 4.


Purpose of review: To provide an overview of existing literature on pathogenetic and clinical aspects of cardiac and vascular involvement in eosinophilic granulomatosis with polyangiitis (EGPA).

Recent findings: In EGPA, cardiac and vascular involvement are more common than previously thought. However, no international recommendations on the topic are available yet. Herein, we summarize the existing evidence on the topic and propose a diagnostic approach for cardiac involvement in EGPA. The prevalence of cardiovascular involvement in patients with EGPA varies greatly among published studies, ranging between 3.1-18.7% for occlusive arterial disease, 5.8-30% for venous thrombosis and 17-92% for heart involvement. Cardiac involvement in EGPA is associated with high mortality even though manifestations are heterogeneous. In principle, every anatomical structure of the heart can be involved, and EGPA-related heart disease may be completely asymptomatic at first. A careful diagnostic work-up for early detection and prompt treatment initiation is therefore required. While cardiac manifestations are more common in anti-neutrophil cytoplasmic antibodies (ANCA)-negative patients, arterial and venous thrombotic events are not linked to ANCA status but correlate closely with disease activity and accumulate at disease onset. Thrombotic events (mainly venous) are considerably more frequent in EGPA than in the general population contributing substantially to morbidity and highlighting the importance of developing specific prevention strategies for patients who are diagnosed with EGPA.

Keywords: Cardiovascular; Eosinophilic granulomatosis with polyangiitis; Heart; Vascular.

Publication types

  • Review

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic
  • Churg-Strauss Syndrome* / complications
  • Churg-Strauss Syndrome* / diagnosis
  • Churg-Strauss Syndrome* / drug therapy
  • Granulomatosis with Polyangiitis* / complications
  • Granulomatosis with Polyangiitis* / diagnosis
  • Granulomatosis with Polyangiitis* / drug therapy
  • Heart
  • Heart Diseases*
  • Humans


  • Antibodies, Antineutrophil Cytoplasmic