Churg-Strauss syndrome

Hippokratia 2005, 9(2):65-72

A Papadopoulou, P Boura
2nd Dpt Internal Medicine, Hippokratio General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece


Churg- Strauss syndrome (CSS) is a form of primary systemic vasculitis, which mainly affects the lower respiratory tract, the nervous system, the gastrointestinal system , heart, skin and kidneys. The incidence of CSS in asthma populations is high. Its exact pathogenesis remains unknown; hyperresponsivness to an antigenic stimulus seems to initiate disease process. In asthmatics, cysteinyl leykotriene receptor type antagonists are reported to trigger the disease. Its pathophysiology is not clearly understood. Stydies concerning the cytokine profile of lymphocytes involved in CSS, suggests that it may be a Th2 mediated disease, although it seems that all types of hypersensitivity reactions take part. Leading laboratory findings are eosinophilia and normochromic normocytic anemia together with elevated IgE, while ANCA are found in approximately 50% of all patients with CSS, but their clinical significance is under researh. Therapeutic protocols include corticosteroids, immunosuppressive regimens and interferon-a, while future alternative treatment protocols target to cytokine inhibition, such as IL-5 and TNF- a.