Brucato A, Baudo F, Barberis M, Redaelli R, Casadei G, Allegri F, De Juli E, De Cataldo F
Divisione Medica Brera, Ospedale Niguarda Ca, Granda Milano, Italy.
J Rheumatol. 1994 May;21(5):942-4.
Recurrent pulmonary emboli or microthromboses are hypothesized as possible causes of pulmonary hypertension in the antiphospholipid syndrome (APS), but thrombosis of the pulmonary vessels has been rarely documented. We describe the case of a 45-year-old Caucasian man affected by thrombocytopenia, recurrent deep venous thrombosis, recurrent pulmonary embolism and fatal chronic pulmonary hypertension (systolic pressure: 85 mm Hg). Anticardiolipin antibodies were highly positive, and the lupus anticoagulant was present. At autopsy, recent thromboses of small vessels were observed in the lung, with organized clots and recanalized channels. Furthermore, friable and firm vegetations and nodules were observed on the cusps of the mitral and tricuspid valves, intermingled with recent surface fibrinous thrombi. In the adrenals we found vascular thrombotic lesions similar to those in the lungs. The pathological lesions suggest pulmonary hypertension secondary to pulmonary arterial microthromboses. Moreover, this is the first documentation of tricuspid valve pathology in a patient with APS.
复发性肺栓塞或微血栓形成被认为是抗磷脂综合征（APS）中肺动脉高压的可能原因，但肺血管血栓形成鲜有文献记载。我们描述了一名45岁的白人男性病例，他患有血小板减少症、复发性深静脉血栓形成、复发性肺栓塞和致命的慢性肺动脉高压（收缩压：85 mmHg）。抗心磷脂抗体高度阳性，且存在狼疮抗凝物。尸检时，在肺中观察到小血管近期血栓形成，伴有机化血栓和再通的血管腔。此外，在二尖瓣和三尖瓣瓣叶上观察到易碎且坚实的赘生物和结节，夹杂着近期的表面纤维蛋白血栓。在肾上腺中，我们发现了与肺部相似的血管血栓性病变。病理病变提示肺动脉微血栓形成继发肺动脉高压。此外，这是首例记录APS患者三尖瓣病变的病例。
