Churg-Strauss Syndrome

Overview
  • Churg-Strauss Syndrome is a vasculitis of small and medium-sized blood vessels, including arteries, capillaries, and venules. Disease can affect virtually any organ although there appears to be a predilection for the lung.
Epidemiology
  • Churg-Strauss Syndrome does not appear to have a major predilection for age and does not have a strong gender-bias.
Pathogenesis
  • Churg-Strauss Syndrome is an ANCA-associated vasculitis, specifically with p-ANCA. The disease appears to have some allergic pathogenesis and is associated with derangements of eosinophil function.
Morphology
  • Churg-Strauss Syndrome is characterized by necrotizing inflammation of the vascular wall and accompanied by the presence of granulomas. Uniquely, granulomatous inflammation is associated with prominent infiltration of eosinophils
Clinical Consequences
  • Patients with Churg-Strauss Syndrome frequently present with severe asthma as well as allergic rhinitis and sinusitis. Characteristically, laboratory findings display a striking eosinophilia.