Eosinophilic Granulomatosis with Polyangiitis

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