Castro Pablo, Venegas Reinaldo, Fardella Patricia, Pérez Osvaldo, Jalil Roberto, Gutiérrez Miguel A
Departamento de Enfermedades Cardiovasculares, Hospital Clínico Pontificia Universidad Católica de Chile.
Rev Med Chil. 2003 Sep;131(9):1037-41.
A 33 years old woman was admitted to the hospital after four days with cough, dyspnea, orthopnea and hemoptysis. Blood pressure was 170/90 mmHg, pulse was 112 and temperature was normal. She had cyanosis and a left ventricular gallop, without heart murmurs. A chest radiograph revealed pulmonary edema and echocardiogram showed a global left ventricular systolic disfunction. Oxygen and furosemide were started, but cardiopulmonary collapse ensued. The patient was supported with mechanical ventilation and treated with inotropic drugs. A right sided cardiac catheterization showed pulmonary wedge pressure of 18 mmHg and a cardiac index of 3 l/min/m2. The levels of creatinine and urea nitrogen were elevated and a urine protein was 97 mg/dl. Coagulation tests were normal except by a positive lupic anticoagulant. Markers of connective tissue diseases or vasculitis were negatives. The clinical evolution suggested that a catastrophic antiphospholipid syndrome was ongoing. Intravenous corticoids, gammaglobulin and cyclophosphamide were administered with transient improvement. On her fourth day of treatment, the patient presented sudden pulmonary bleeding and embolism. A plasmapheresis was performed with improvement of renal, cardiac and pulmonary function. After this episode, the patient has been treated with prednisone and oral anticoagulants treatment for the last two years, without further clinical events.
一名33岁女性在出现咳嗽、呼吸困难、端坐呼吸和咯血四天后入院。血压为170/90 mmHg,脉搏112次/分,体温正常。她有发绀和左心室奔马律,无心脏杂音。胸部X线片显示肺水肿,超声心动图显示左心室整体收缩功能障碍。开始给予氧气和呋塞米治疗,但随后出现心肺功能衰竭。患者接受机械通气支持并使用强心药物治疗。右侧心导管检查显示肺楔压为18 mmHg,心脏指数为3 l/min/m²。肌酐和尿素氮水平升高,尿蛋白为97 mg/dl。除狼疮抗凝物阳性外,凝血检查正常。结缔组织病或血管炎标志物均为阴性。临床进展提示正在发生灾难性抗磷脂综合征。给予静脉注射皮质类固醇、丙种球蛋白和环磷酰胺后病情有短暂改善。在治疗的第四天,患者突然出现肺出血和栓塞。进行了血浆置换,肾功能、心脏功能和肺功能均有改善。在此事件之后,患者在过去两年中一直接受泼尼松和口服抗凝剂治疗,未再发生进一步的临床事件。