Antiphospholipid syndrome presenting as portopulmonary hypertension

The association of pulmonary hypertension with portal hypertension, also ca lled portopulmonary hypertension, is a well-described condition. The pathog enesis of this association remains unclear. We describe a 34-year-old femal e patient with "primary antiphospholipid syndrome" and portopulmonary hyper tension. Our finding supports that in situ microthrombosis associated with the presence of anticardiolipin antibodies could be the pathophysiologic ex planation for both portal and pulmonary hypertension.