Updated: 12/28/2021

Eosinophilic Granulomatosis with Polyangiitis (Churg- Strauss Syndrome)

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  • Snapshot
    • A 35-year-old man presents to his primary care physician for numbness and weakness of the left lower extremity and "cold-like" symptoms. He has also noticed skin changes in the affected leg. His medical history is significant for asthma treated with albuterol. Physical exam is significant for 3/5 strength throughout the lower extremity and decreased touch sensation. There are cutaneous and subcutaneous nodules. Laboratory findings are significant for positive MPO-ANCA/p-ANCA antibodies and a serum eosinophil level of 8000/μL.
  • Introduction
    • Definition
      • a multisystem small and medium-sized artery necrotizing vasculitis characterized by
        • rhinosinusitis
        • asthma
        • peripheral blood eosinophilia
  • Epidemiology
    • Demographics
      • 20-40 years of age
      • male > female
  • ETIOLOGY
    • Pathogenesis
      • unknown
  • Presentation
    • Symptoms/physical exam
      • disease phases
        • prodromal
          • atopic disease
          • asthma
          • allergic rhinitis
        • eosinophilic
          • peripheral blood eosinophilia
          • eosinophils infiltrate organs (e.g., lungs)
        • vasculitic
          • vascular and extravascular granulomatosis
            • e.g., fever, weight loss, and skin lesions (tender subcutaneous nodules)
    • Other findings
      • peripheral neuropathy
      • gastrointestinal involvement
  • Studies
    • Labs
      • positive MPO-ANCA/p-ANCA antibodies
      • ↑ IgE level
      • peripheral blood eosinophilia (characteristic finding)
  • Differential
    • Granulomatosis with polyangiitis
      • differentiating factors
        • positive PR3-ANCA/c-ANCA antibody
        • upper and lower respiratory and renal involvement
    • Temporal arteritis
      • differentiating factors
        • occurs in elderly patients with temporal headaches and jaw claudication
        • high risk of blindness if untreated
  • Treatment
    • Medical
      • high-dose glucocorticoids
        • indication
          • for induction of remission
  • Complications
    • Necrotizing glomerulonephritis
    • Purpura
    • Parenchymal disease
  • Prognosis
    • Improved with immunosuppressive treatment

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