What are the diagnostic criteria for 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

In 1951, Churg and Strauss first described the syndrome in 13 patients who had asthma, eosinophilia, granulomatous inflammation, necrotizing systemic vasculitis, and necrotizing glomerulonephritis. [4] In 1990, the American College of Rheumatology (ACR) proposed the following six criteria for the diagnosis of Churg-Strauss syndrome [7] :

  • Asthma (wheezing, expiratory rhonchi)
  • Eosinophilia of more than 10% in peripheral blood
  • Paranasal sinusitis
  • Pulmonary infiltrates (may be transient)
  • Histological proof of vasculitis with extravascular eosinophils
  • Mononeuritis multiplex or polyneuropathy

The presence of four or more criteria yields a sensitivity of 85% and a specificity of 99.7%. The 1994 Chapel Hill consensus conference on the classification of vasculitides did not modify the ACR criteria. [8]


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