Read more about this disease, some with Classification – Types – Signs and symptoms – Genetics – Pathophysiology – Diagnosis – Screening – Prevention – Treatment and management – Cures and much more, some including pictures and video when available.
Churg-Strauss syndrome (also known as allergic granulomatosis) 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. It is a rare disease that is non-inheritable, non-transmissible and often mis-diagnosed. Churg-Strauss syndrome was once considered a type of Polyarteritis nodosa (PAN) due to their similar morphologies.
The syndrome was first described by Drs. Jacob Churg and Lotte Strauss at Mount Sinai Hospital in New York City in 1951.[1][2]
Diagnostic markers include eosinophil granulocytes and granulomas in affected tissue and Anti-neutrophil cytoplasmic antibodies (ANCA) against neutrophil granulocytes. Differentiation from Wegener’s granulomatosis can be difficult, though the increasing use of ANCA assays has made the distinction more routine. Wegener’s is closely associated with c-ANCA, unlike Churg-Strauss, which shows elevations of p-ANCA.
This disease has three distinct stages.
People can live for many years in the first two stages before progressing to stage three.
The French Vasculitis Study Group has developed a five-point score (“five-factor score” or FFS) that predicts the risk of death in Churg-Strauss syndrome. These are (1) reduced renal function (creatinine >1.58 mg/dL or 140 µmol/l), (2) proteinuria (>1 g/24h), (3) gastrointestinal hemorrhage, infarction or pancreatitis, (4) involvement of the central nervous system or (5) cardiomyopathy. Presence of 1 of these indicates severe disease (5-year mortality 26%) and 2 or more very severe disease (mortality 46%), while absence of any of these 5 indicates a milder case (mortality 11.9%).[3]
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