血栓症と循環のジャーナル: オープンアクセス

概要

Coronary Arteries Involvement in Churg-Strauss Syndrome Simulating an Acute Coronary Syndrome: An Emblematic Case and Literature Review

Buccheri D, Chirco PR, Piraino D, Carella M, Franca EL, Cortese B and Andolina G

Churg-Strauss syndrome is a rare vasculitis, affecting small to medium vessels, recently renamed Eosinophilic Granulomatosis with Polyangiitis. The American College of Rheumatology proposed the following diagnostic criteria: asthma, eosinophils on the leukocyte count greater than 10%, mononeuropathy or polyneuropathy, migratory or transient pulmonary opacities detected radiographically, paranasal sinus abnormality and evidence of eosinophilic infiltration in extravascular tissues. At least, four out of previous six criteria are needed for the diagnosis. Cardiac involvement has been documented in 16-50% of cases and it often takes the form of acute coronary syndrome or mimic it. Furthermore, it can cause about half of the deaths. The eosinophil-mediated heart damage can evolve through three stages: the acute necrotic, intermediate thrombotic and, finally, fibrotic one. It is know that infiltrating eosinophils can damage the endocardium and vascular endothelium. Rarer but equally important are the vasculitis affecting small myocardial vessels and coronary arteries, which can lead to myocardial ischemia, and ectasia and aneurysms of the coronary arteries due to the eosinophil infiltration and to a direct cytotoxic damage mediated by eosinophilic proteins. Furthermore, an uncommon manifestation of Churg-Strauss syndrome is coronary artery vasospasm that can lead to angina pectoris, acute coronary syndromes or even to cardiogenic shock. Corticosteroids (prednisone or its equivalents) remain the cornerstone of treatment of Churg-Strauss syndrome and the addition of azathioprine or cyclophosphamide is indicated for the treatment of patients with adverse prognostic factors or otherwise prone to relapse.The prognosis of Churg-Strauss syndrome is good, with an overall 10-year survival of 81-92% of patients. Coronary involvement in this disease may however be diffuse and a life-threatening condition. In this light, antiplatelet drugs (aspirin at first) may be evaluated in the field of coronary primary prevention. Here, we are describing a case report and literature review from cardiology point of view, highlighting coronary involvement.