[Pulmonary artery hypertension caused by carcinomatous lymphangitis ]

A 58 year old man without significant past medical history developed a cough and effort dyspnoea of a few weeks. A computed tomographic scan showed bilateral interstitial disease with linear thickening of the septa and also a ground glass effect in the lung parenchyma. Echocardiography and right heart catheterisation confirmed the existence of pulmonary arterial hypertension (mean pulmonary artery pressure 45 mmHg) with a normal pulmonary artery wedge pressure (12 mmHg). There was a rapidly progressive worsening with an unfavourable outcome. A necropsy examination showed the development of bilateral pulmonary lymphangitis carcinomatosis secondary to an adenocarcinoma of the head of the pancreas. There was no metastatic emboli of the vessels but there was lymphatic infiltration and the physiopathological hypothesis suggested a compression of the pulmonary arterial vessels secondary to lymphatic obstruction.