Churg–Strauss syndrome

Churg–Strauss syndrome (CSS, also known as eosinophilic granulomatosis with polyangiitis [EGPA] or allergic granulomatosis) is an autoimmune condition that causes inflammation of small and medium-sized blood vessels (vasculitis) in persons with a history of airway allergic hypersensitivity (atopy). It usually manifests in three stages. The early (prodromal) stage is marked by airway inflammation; almost all patients experience asthma and/or allergic rhinitis. The second stage is characterized by abnormally high numbers of eosinophils (hypereosinophilia), which causes tissue damage, most commonly to the lungs and the digestive tract. The third stage consists of vasculitis, which can eventually lead to cell death and can be life-threatening.

Effective treatment of Churg–Strauss syndrome requires suppression of the immune system with medication. This is typically glucocorticoids followed by other agents such as cyclophosphamide or azathioprine.

The syndrome carries the name of the doctors who first described it in 1951, Jacob Churg and Lotte Strauss.