Eosinophilic granulomatosis with polyangiitis: case report and literature review

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome, is a multisystem disorder characterised by asthma, blood and tissue eosinophilia and small-vessel vasculitis. Eosinophilic tissue infiltration and extravascular granuloma formation can lead to damage in any organ, but it is classically seen to cause pulmonary infiltrates, sino-nasal disease, peripheral neuropathy, renal and cardiac involvement, and rashes. EGPA is part of the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis syndromes, with the antibody being detected in ∼30-40% of cases and mostly against myeloperoxidase. Two genetically and clinically distinct phenotypes, defined by the presence or absence of ANCA have been identified. Treatment for EGPA focuses on inducing and maintaining disease remission. To date, oral corticosteroids remain first-line agents whilst second-line treatments include immunosuppressants such as cyclophosphamide, azathioprine, methotrexate, rituximab and mycophenolate mofetil. However, long-term steroid usage results in multiple and well-known adverse health effects and new insights into the pathophysiology of EGPA have allowed for the development of targeted biologic therapies, like the anti-eosinophilic, anti-interleukin-5 monoclonal antibodies.

FIGURE 1

Three clinical phases of eosinophilic granulomatosis with polyangiitis.

FIGURE 2

a) Eosinophilic tissue infiltration. b) Vasculitic rash in a patient with eosinophilic granulomatosis with polyangiitis (EGPA). c) Cardiac magnetic resonance imaging (MRI), T2W MRI showing an area of high signal in an acutely injured, inflamed inferior wall in a patient with EGPA (image courtesy of Tevfik Ismail, Consultant Cardiologist, Guy's and St Thomas' NHS Trust). d, e) Computed tomography (CT) of the chest showing peribronchovascular and patchy ground-glass opacification in a 45-year-old male patient (d) and 48-year-old female (e) patient with EGPA. Images in panels a, b, d and e are the authors’ own, all images are published with patient consent.