PULMONARY-HYPERTENSION SECONDARY TO THROMBOSIS OF THE PULMONARY VESSELS IN A PATIENT WITH THE PRIMARY ANTIPHOSPHOLIPID SYNDROME

Recurrent pulmonary emboli or microthromboses are hypothesized as poss ible causes of pulmonary hypertension in the antiphospholipid syndrome (APS), but thrombosis of the pulmonary vessels has been rarely docume nted. We describe the case of a 45-year-old Caucasian man affected by thrombocytopenia, recurrent deep venous thrombosis, recurrent pulmonar y 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 smal l vessels were observed in the lung, with organized clots and recanali zed channels. Furthermore, friable and firm vegetations and nodules we re observed on the cusps of the mitral and tricuspid valves, interming led with recent surface fibrinous thrombi. In the adrenals we found va scular thrombotic lesions similar to those in the lungs. The pathologi cal lesions suggest pulmonary hypertension secondary to pulmonary arte rial microthromboses. Moreover, this is the first documentation of tri cuspid valve pathology in a patient with APS.