Comprehensive myocardial tissue characterization with cardiac magnetic resonance in patients with Churg Srauss Syndrome

Background Churg-Strauss syndrome (CSS) is a rare systemic necrotizing vasculitis characterized by asthma, hypereosinophilia, cardiac failure, renal damage and peripheral neuropathy. CSS typically develops in three clinical phases, beginning with asthma, followed by tissue eosinophilia and finally in a systemic small-vessel vasculitis. During the late phase, coronary vessels and myocardium are interested leading to a variety of clinical manifestations such as myocarditis, heart failure, pericarditis and coronary vasculitis. Heart involvement is found in over 50% of autopsied CSS patients with evidence of an extremely high mortality in those with cardiac disease left untreated. Cardiac MR has emerged as a promising technique not only for the early diagnosis of cardiac involvement in the setting of this insidious and complex condition, but also for a better risk stratification and tailoring of immunosuppressive therapy.