Piette J C, Cacoub P, Karmochkine M, Godeau P
Service de Médecine Interne, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
Rev Pneumol Clin. 1994;50(3):99-105.
The antiphospholipid syndrome (APS) is usually defined by the association of a clinical manifestation (venous or arterial thrombosis or miscarriage) with the presence of antiphospholipid antibodies (lupus anticoagulant and/or anticardiolipin antibodies). It frequently occurs in the course of systemic lupus erythematosus but is also encountered as a "primary" disease. APS is responsible for diverse respiratory manifestations. Pulmonary embolism is common. The site of the causal venous thrombosis is frequently unusual. Pulmonary hypertension may be a consequence of repeated embolism or may belong to the primary idiopathic variety. Pulmonary manifestations may also result from left-sided heart failure due to mitral or aortic valve abnormalities, myocardial infarction or a specific myocardiopathy. APS is probably involved in the occurrence of some cases of adult respiratory distress syndrome. Long term secondary prevention of recurrent thrombosis is a central point in the management of APS.
抗磷脂综合征(APS)通常定义为临床表现(静脉或动脉血栓形成或流产)与抗磷脂抗体(狼疮抗凝物和/或抗心磷脂抗体)的存在相关联。它常发生于系统性红斑狼疮病程中,但也可作为一种“原发性”疾病出现。APS可导致多种呼吸系统表现。肺栓塞很常见。导致血栓形成的静脉部位常常不寻常。肺动脉高压可能是反复栓塞的结果,也可能属于原发性特发性类型。肺部表现也可能由二尖瓣或主动脉瓣异常、心肌梗死或特定心肌病导致的左心衰竭引起。APS可能与某些成人呼吸窘迫综合征病例的发生有关。复发性血栓形成的长期二级预防是APS管理的核心要点。