14 July 2011 (Thursday) - Churg Strauss Syndrome

A new diagnosis. All sorts of allergies and asthma in someone who’s never previously had them: Churg–Strauss syndrome is a medium and small vessel autoimmune vasculitis which can lead to necrosis. It involves mainly the blood vessels of the lungs, gastrointestinal system, and peripheral nerves, but also affects the heart, skin and kidneys.
It is a rare disease that is non-inheritable and non-transmissible. Differentiation from Wegener's granulomatosis is not difficult. Wegener's is closely associated with c-ANCA, while Churg-Strauss shows elevations of p-ANCA.

The French Vasculitis Study Group has developed a five-point score that predicts the risk of death in Churg–Strauss syndrome

  • reduced renal function (creatinine >1.58 mg/dL or 140 μmol/l)
  • proteinuria (>1 g/24h)
  • gastrointestinal hemorrhage, infarction or pancreatitis
  • involvement of the central nervous system
  • cardiomyopathy.
Presence of one of these indicates severe disease (five-year mortality 26%) and two or more very severe disease (five-year mortality 46%). Clearly the blood science lab is crucial here...

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