Eosinophilic Granulomatosis with Polyangiitis: Clinical Pathology Conference and Review

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss syndrome) is a small vessel vasculitis associated with asthma and eosinophilia. Despite its rarity, continuous gains are being made in understanding the disease with knowledge advancements regarding its epidemiology, heterogeneous clinical manifestations, management, and outcomes. Large knowledge gaps remain, however, particularly surrounding pathophysiologic and diagnostic uncertainties. There is still an incomplete understanding of the interplay between the eosinophilic and vasculitic processes that are features of disease pathogenesis. EGPA is also a conceptually difficult disorder given its dual categorization with hypereosinophilic syndromes and systemic vasculitides and the absence of a biomarker that can reliably distinguish between the two. In addition, recent evidence points to distinct, but partly overlapping, disease phenotypes, yet there is insufficient understanding to inform phenotype-tailored therapies. EGPA also remains a diagnostic challenge in part because asthma may be the primary or predominant manifestation for years, and the chronic corticosteroid requirement may mask other disease features. Efforts are ongoing to better elucidate pathophysiologic mechanisms, resolve classification issues, better characterize disease manifestations, and further clarify disease subcategorization, all of which will translate into better diagnosis and treatment with the possibility of specifically adapted therapies.

Keywords: ANCA vasculitis; Asthma; Churg-Strauss; Eosinophilia; Eosinophilic granulomatosis with polyangiitis.