Saturday, August 30, 2008

Churg-Strauss Syndrome

There is a patient now on the renal consult service with Churg-Strauss Syndrome. This disease falls under the category of "small vessel vasculitis" and most commonly is thought of as a pulmonary disease, as the characteristic triad consists of allergic rhinitis, asthma, and peripheral eosinophilia. However it may involve any organ system, including the kidney, where it can cause a nephritic urine sediment and kidney biopsy generally reveals a pauci-immune glomerulonephritis. The ANCA titer is usually positive and is typically a p-ANCA (perinuclear) pattern against myeloperoxidase.

This particular patient had a number of features consistent with Churg-Strauss: a long-standing history of steroid-requiring asthma, peripheral eosinophilia, a skin rash (with a biopsy showing extensive eosinophilic infiltrate), mononeuritis multiplex, and acute kidney injury requiring dialysis with a renal biopsy demonstrating a necrotizing vasculitis. She is being treated with pulse steroids and oral Cytoxan.

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