French Vasculitis Study Group

Figure 2 See color insert.) ENT manifestations bilateral maxillary sinusitis. Source Courtesy of the French Vasculitis Study Group.

Heart involvement develops in up to 60% of CSS patients (1). In the French Vasculitis Study Group (FVSG) experience, heart disease was found in 39 of 112 (35%) patients, including 28 patients with pericarditis and 27 with cardiomyopathy (3). Heart disease can involve the myocardium, pericardium, and at lesser degree, endocardium (28) and, in earlier studies, represented the major cause of death, accounting for about 48% of deaths (28,29) and morbidity. Myocarditis (caused by different or perhaps associated mechanisms, including eosinophilia, coronary arteritis, and fibrosis) can lead to restrictive (29), congestive (30), or dilated cardiomyopathy (30,31). Pericardial effusion occurs in up to 22% of CSS patients. CS usually control pericarditis but relapses may occur. 

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