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Key Features

  • An ANCA-associated vasculitis (along with granulomatosis with polyangiitis and microscopic polyangiitis)

  • However, the presence of ANCA occurs in < 50% of patients (usually anti-MPO)

  • Previously called Churg-Strauss syndrome

Clinical Findings

  • Peripheral eosinophilia

  • Sinusitis with polyposis

  • Asthma

  • Lung infiltrates

  • Vasculitic skin involvement

  • Glomerulonephritis

  • Vasculitic neuropathy

  • Myocarditis can lead to arrhythmias and heart failure if untreated

Diagnosis

  • Peripheral eosinophilia and vasculitic features

  • Obtain ANCA and serum tryptase peripheral flow cytometry for PDGF receptor abnormalities

  • Test for helminthic infections

  • Eosinophilic infiltrates on tissue samples strongly suggest the diagnosis of eosinophilic granulomatosis, especially if accompanied by vasculitis (purpura, glomerulonephritis, vasculitic ulcers, mononeuritis multiplex)

Treatment

  • Corticosteroids remain first-line treatment

  • Azathioprine or methotrexate can be added to corticosteroids for treatment of mild to moderate disease

  • Mepolizumab, an IL-5 inhibitor

    • FDA approved for the treatment of eosinophilic granulomatosis with polyangiitis

    • However, it has not been studied for severe life- or organ-threatening vasculitic disease manifestations (which generally require cyclophosphamide)

  • Rituximab may be considered as an option for patients with refractory disease

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