Early Diagnosis and Treatment in Patients With Eosinophilic Granulomatosis With Polyangiitis.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), originally termed Churg-Strauss syndrome, represents the rarest form of antineutrophil cytoplasmic antibody-associated vasculitis. It is characterized by the presence of asthma, rhinosinusitis with or without nasal polyps, eosinophilic inflammation of the blood, and tissues and necrotizing vasculitis of small to medium-sized blood vessels. Owing to its rare, multisystemic nature with diverse symptom presentation, diagnosis is complex, requiring a multidisciplinary approach and a careful array of diagnostic and clinical assessments. Conventional therapy is composed of the use of oral glucocorticoids, which are associated with long-term adverse effects, and other immunomodulatory drugs. However, earlier diagnosis and prompt tailored treatment can improve clinical outcomes and reduce drug-related toxicity. The initiation of biologic therapies, such as those blocking IL-5 or its receptor, which have recently been approved for the treatment of non-severe relapsing EGPA, has emerged as a paradigm shift in management. An illustrative case is used to present a comprehensive representation of the diagnosis, pathophysiology, and management of EGPA.