Churg-Strauss syndrome pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Editor-in-Chief: Alexandra M. PalmerAssociate Editor(s)-in-Chief: Krzysztof Wierzbicki M.D. [2]

Pathophysiology

Churg–Strauss syndrome is a medium and small vessel autoimmune vasculitis, leading to necrosis. It involves mainly the blood vessels of the lungs (it begins as a severe type of asthma), gastrointestinal system, and peripheral nerves, but also affects the heart, skin, and kidneys.

Associated Diseases

• Asthma

  • >95%
  • Usually precedes the vasculitic phase by 8-10 years (but can be coincident)

• Nasal and Sinus disease
• Skin Disease

  • 66%
  • Subcutaneous nodules on the extensor surfaces of the arm

• Cardiovascular disease

  • Pericarditis (32%)
  • Pericardial constriction
  • Heart failure (47%)
  • Myocardial infarction (MI)

• Neurologic Disease

  • 75% mononeuritis multiplex
  • If untreated may progress to polyneuropathy

• Kidney Disease

  • 85% with focal segmental glomerulonephritis (FSGN) on biopsy

    • Unclear total incidence of renal disease.

  • 70% P-anca positive

    • But renal failure is rare (in contrast to Wegener’s)

• GI Disease

  • Eosinophilic gastroenteritis with abdominal pain (59%)
  • Diarrhea (33%)
  • GI bleeding (18%)

Microscopic Pathology

• Eosinophilic infiltrates with necrosis (sometimes quite extensive)
• Giant cell vasculitis with eosinophils
• Interstitial and perivascular necrotizing granulomas
• Eosinophilic lymphadenopathy

Necrotizing and granulomatous arteritis in the lung of a patient with Churg-Strauss syndrome.