What are the guidelines on the workup of EGPA (Churg-Strauss syndrome)?

Updated: Dec 24, 2018
  • Author: Spencer T Lowe, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

European guidelines from the EGPA Consensus Task Force recommend the following for the workup of eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome) [2] :

  • Serologic testing for toxocariasis and HIV
  • Specific IgE and IgG for Aspergillus species
  • Testing for Aspergillus spp. in sputum, bronchoalveolar lavage fluid, or both
  • Tryptase and vitamin B12 levels
  • Peripheral blood smear (looking for dysplastic eosinophils or blasts)
  • Antineutrophil cytoplasmic antibody (ANCA) with indirect immunofluorescence and enzyme-linked immunosorbent assay (ELISA)
  • Chest computed tomography (CT) scan

Additional investigations should be guided by patient-specific clinical findings and an extensive search for causes of hypereosinophilia. [2]

The EGPA Consensus Task Force encourages obtaining biopsies from patients with suspected EGPA. In the correct clinical context (asthma with eosinophilia or systemic manifestations, or even eosinophilia with extrapulmonary disease), a biopsy showing small- or medium-vessel vasculitis, a strong clinical surrogate of vasculitis, or both strongly supports a diagnosis of EGPA. [2]


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