Antiphospholipid syndrome presenting as portopulmonary hypertension

The association of pulmonary hypertension with portal hypertension, also called portopulmonary hypertension, is a well-described condition. The pathogenesis of this association remains unclear. We describe a 34-year-old female patient with "primary antiphospholipid syndrome" and portopulmonary hypertension. Our finding supports that in situ microthrombosis associated with the presence of anticardiolipin antibodies could be the pathophysiologic explanation for both portal and pulmonary hypertension.