Skip to Main Content

KEY FEATURES

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

  • However, the presence of antineutrophil cytoplasmic antibody (ANCA) occurs in < 50% of patients (usually anti-myeloperoxidase [MPO] antibody)

  • 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 vasculitis

  • Laboratory work-up for unexplained eosinophilia should include

    • ANCA testing

    • Serum tryptase levels

    • Peripheral flow cytometry for platelet-derived growth factor (PDGF) receptor abnormalities (seen in eosinophilic leukemia)

    • Testing for helminthic infections

  • Eosinophilic infiltrates on tissue samples

    • Strongly suggest the diagnosis of eosinophilic granulomatosis with polyangiitis

    • Diagnosis even more likely if accompanied by vasculitis (purpura, glomerulonephritis, vasculitic ulcers, mononeuritis multiplex)

TREATMENT

  • Corticosteroids remain first-line treatment

  • Azathioprine or methotrexate for mild to moderate disease

  • Mepolizumab, an interleukin (IL)-5 inhibitor

    • Food and Drug Administration-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

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.

  • Create a Free Profile