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Giant coronary artery aneurysm in Behçet’s disease, a case report

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Giant coronary artery aneurysm in Behçet’s disease, a case report

부산대학교 병원

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양소리, 최정현

Background: Behcet’s disease, also known as Behcet’s syndome, is a rare, autoinflammatory disease representing the multi-systemic involvement includ- ing eyes, skin, genital organs, oral mucosa, nerves and vessels. Vascular involvement counts 20-25% of disease population and this occurs more in veins than arteries. The arterial involvement usually affects abdominal aorta, pulmonary arteries, femoral arteries and rarely, coronary arteries. The cardiac in- volvement is very uncommon, but they are potentially life-threatening, especially when ruptured. Case report: We report a case of 48-year-old male pa- tient, who visited a medical center due to his leg pain at first, and later diagnosed as giant coronary artery aneurysm on Behçet’s disease. The patient under- went resection of Rt. popliteal artery aneurysm and graft interposition in the vascular surgery department, and soon referred to the cardiology department as he complained the symptom of palpitation. His treadmill test was positive, cardiac enzymes were elevated and coronary angiography showed huge aneur- ysmal sac on proximo-middle right coronary artery and proximal left anterior descending artery. Transthoracic echocardiography showed giant coronary ar- tery aneurysm with strong spontaneous echo contrast and biventricular dysfunction was noted. Considering that aneurysmal growth was significantly fast on chest radiography, patient had aneurysmectomy and coronary artery bypass graft surgery. Afterwards, patient also referred to nephrology, rheumatology and neurology department and clinically diagnosed as Behçet’s disease while he also represented oral ulcers. After a 2-year follow up of echocardiography, ejection fraction has improved from 45 to 59% and there was no ventricular dysfunction. Conclusion: In patients with Behçet’s disease, as our case shows, it is important for patient to have a regular cardiovascular examination even if one does not present any symptoms of coronary invasion. Cardiac involve- ment of Behçet’s disease is very rare, but life-threatening so that clinicians should consult to cardiology when they meet patients with Behçet’s disease.

Sat-188

A rare case of Churg-Strauss syndrome presenting with pericardial effusion in Korea

부천세종병원

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유지웅, 서보미, 정지현

Introduction: Churg-Strauss syndrome (CSS) is a rare systemic necrotizing vasculitis that affects many organs. Heart involvement occurs in approx- imately 15% to 60% of patients with CSS and signifies a poor prognosis. CSS primarily presents as a cardiac failure due to myocarditis and myocardial is- chemia as a result of coronary arteritis. Here, we report a rare case of CSS presenting with a pericardial effusion and preserved myocardial function. Case report: A 21-year-old female with no history of cardiac disease and asthma was admitted for dyspnea and chronic cough that began two months ago. Her vital signs were stable. An electrocardiogram showed normal sinus rhythm. Chest X-ray showed marked cardiomegaly. Echocardiography displayed the normal left ventricular ejection fraction and an extensive pericardial effusion (Fig. A), but the right atrium and right ventricle were not collapsed. The left ventricular cavity was not dilated. We performed pericardiocentesis. Cytologic examination of the pericardial fluid showed eosinophil-dominant exudates (37%), but no malignant cells. The histopathology of the pericardium revealed acute and chronic inflammation with elevated eosinophil infiltration (Fig.

B). Laboratory findings revealed marked peripheral eosinophilia (28.2%). Tests for antinuclear antibodies, anti-neutrophil cytoplasmic antibody, bacteria, fungi, viruses, parasites, and tuberculosis were negative. Maxillary sinusitis was found by x-ray in both sinuses. A pulmonary function test showed an ob- structive pattern, and the change in FEV1 in response to bronchodilator was 28% and 480 ml. The result was consistent with asthma. The presence of eosi- nophilia, pathological evidence of eosinophilic infiltration, asthma, and a paranasal sinus abnormality fulfilled the criteria for CSS. She began im- munosuppressive therapy. Conclusion: We report a rare case of CSS presenting with pericardial effusion without myocardial dysfunction in Korea. CSS should be considered as one of the differential diagnoses of pericardial effusions.

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