Abstract
Churg-Strauss syndrome (CSS) is a very rare small-vessel vasculitis. Clinical features include asthma, rhinitis and/or sinusitis, and peripheral eosinophilia. Although cardiac findings are observed in 50% of cases, coronary artery disease is rarely reported and even more rarely suggestive. The value of cardiac MRI for these patients is discussed here. A 52-year-old non-smoker male without family antecedents of cardiovascular disease presented with worsening of atypical asthma that developed 10 months earlier. A month before, he had been admitted to the ICU because of respiratory distress and cardiogenic shock with chest pain. The angiogram revealed stenosis of the three main coronary arteries requiring the placement of several stents. Follow-up cardiac assessments showed good results. General impairment, unstable asthma associated with rhinitis, and eosinophilia suggested a systemic disease. The diagnosis of CSS was established considering that five criteria of the American College of Rheumatology were found. Prednisolone was prescribed at 1 mg/kg/day, which completely suppressed all symptoms and eosinophilia. Cardiac MRI was performed two months later and revealed a good control of myocardial lesions characterized by fibrosis beneath the anterior endocardium and the median septum. Immunosuppressive treatment was then administered together with corticosteroid therapy. These results suggest that acute coronary artery disease can reveal CSS in some cases. Here, the patient’s cardiac assessment was normal apart from the acute episode, and cardiac MRI helped detect signs of myocarditis and establish a prognosis of CSS.
Highlights
According to the Chapel Hill classification, Churg-Strauss syndrome (CSS) is a necrotizing vasculitis of small-tomedium-sized vessels
Cardiac findings are observed in 50% of cases, coronary artery disease is rarely reported and even more rarely suggestive
Cardiac magnetic resonance imaging (MRI) was performed two months later and revealed a good control of myocardial lesions characterized by fibrosis beneath the anterior endocardium and the median septum
Summary
According to the Chapel Hill classification, Churg-Strauss syndrome (CSS) is a necrotizing vasculitis of small-tomedium-sized vessels. It is a rare disease, with 0.5 - 6.8 million cases per year. It is characterized by eosinophilia, an inflammatory disease, and by the presence of neutrophilic polynuclear anticytoplasmic antibodies in 35% - 50% of patients. Cardiac findings are observed in about 50% - 60% of cases in the form of pericarditis, myocarditis or ischemic cardiopathy. These play an important role in prognosis, as they are responsible for 48% of deaths.
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