산후 확장심근병과 미만성 폐포출혈로 나타난 항인지질증후군 1예
Possible Antiphospholipid Syndrome Presenting as Peripartum Cardiomyopathy and Diffuse Alveolar Hemorrhage
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초록
항인지질증후군(antiphospholipid syndrome, APS)은 항인지질항체인 루푸스 항응고인자(lupus anticoagulant, LAC), 항카디오리핀항체(anticardiolipin antibody, aCL Ab), 베타2당단백-I 항체(ß2-glycoprotein-I antibody, ß2GPI Ab)가 양성이면서 혈관의 혈전이나 반복적인 유산을 특징으로 한다[1]. APS는 혈관염과 유사한 임상양상을 가지므로 전신을 침범 가능한 자가면역질환이며 심혈관계 침범 시에는 대부분 판막이상이나 심혈관 폐쇄, 심장내 혈전, 그리고 드물게 확장심근병으로 나타날 수 있다[1]. 그러나 폐포출혈이 동반된 산후 확장심근병으로 나타나는 경우는 흔치 않다.
Abstract
Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the presence of circulating antiphospholipid antibodies in combination with at least one other clinical manifestation, such as vascular thrombosis or recurrent pregnancy loss. APS is further characterized by the presence of vasculitis affecting multiple organ systems, resulting in a wide variety of clinical outcomes. Common cardiac manifestations of APS include valve abnormalities, occlusive arterial disease, intra-cardiac emboli, ventricular dysfunction, and pulmonary hypertension, along with rare instances of dilated cardiomyopathy. Simultaneously diffuse alveolar hemorrhage and peripartum cardiomyopathy have also been reported, though these manifestations remain extremely rare. Here, we report a case of a 34-year-old woman with possible APS who presented with peripartum cardiomyopathy and diffuse alveolar hemorrhage.
참고문헌 (10)
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- 김조은, "파국 항인지질 증후군 의증 환자의 파종혈관내응고 관련 급성 신손상 1예", 대한내과학회지, 82(6), pp.739~743 (2012), 대한내과학회[DOI]
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