Thanks to Dr. Sandy Park for an excellent case of EGPA!

Learning Points:

• Eosinophilic Granulomatosis with Polyangiitis (EGPA, formerly Churg-Strauss Syndrome) is a multisystem, small vessel vasculitis characterized by asthma, rhinosinusitis, and peripheral eosinophilia.
• EGPA may have involvement of multiple systems including pulmonary (asthma, alveolar hemorrhage), upper airway (allergic rhinitis, sinusitis), skin (nodules, maculopapular rash, leukocytoclastic vasculitis), neurologic (mononeuritis multiplex), MSK (arthralgias, myalgias), cardiac (pericarditis, heart failure), renal and GI.
• Diagnosis requires a high level of suspicion, and may be made based on the Lanham criteria or the diagnostic criteria set out by the ACR. Click here for the original 1990 article outlining EGPA diagnostic criteria.
• Severity of disease may be assessed by the Five factors score, and is influenced by age, cardiac involvement, renal insufficiency, GI involvement, and absence of ENT involvement.
• Mainstay of treatment is corticosteroids and cyclophosphamide, however new evidence indicates rituximab may be an effective steroid sparing agent in the treatment of EGPA. Click this article to read all about it!