Eosinophilic Granulomatosis with Poly-Angiitis (EGPA) : A Respiratory perspective

The lung is commonly involved in systemic disorders: Vasculitis eg ANCA associated vasculitis, but also by; Eosinophilic disorders, eg eosinophilic asthma, eosinophilic pneumonitis, allergic bronchopulmonary aspergillosis and EGPA. An awareness of respiratory involvement by EGPA with multi-organ assessment ensures an Early EGPA diagnosis. Prompt immunosuppressive therapy is essential to optimise long term outcomes and prevent end organ damage.

The immunology of EGPA is explored highlighing the importance of asthma, sino-nasal disease, eosinophilia, extra-pulmonary organ damage, vasculitis and eosinophilic inflammation in diagnosis,.

Recent published management guidelines include American College of Rheumatology 2021, EULAR 2022, and British Society of Rheumatology 2025. The Overlap of ANCA associated vasculitis, EGPA, and hyper-eosinphilic syndromes discussed. The role of the respiratory physician is highlighted in EGPA and vasculitis MDT, for diagnosis and management.

Optimum treatment includes early Corticosteroid treatment , in combination with Rituximab or Cyclophosphamide, as remission induction therapy for EGPA, particularly for life or organ threatening disease. Novel clinical studies of Mepolizumab or Benralizumab as early anti-Interleukin-5/IL-5 Receptor antagonist eosinophil depleting therapy in EGPA are discussed, complementing conventional immunosuppression for remission induction in non-life threatening EGPA, and as early remission maintenance regimens, to facilitate corticosteroid weaning, to reduce steroid related co-morbidity and immunosuppression burden, and ensure long term maintenance remission. Rituximab, Methotrexate, mycophenolate or Azathioprine may be considered as alternative options when anti-IL-5/ IL-5R not available, or as adjunctive maintenance therapies depending on disease phenotype.