Updated: 6/23/2018

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
  • Pathogenesis
    • unknown
  • Prognosis
    • improved with immunosuppressive treatment
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

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