[Pulmonary veno-occlusive disease in a patient with scleroderma and the CREST syndrome]

Pulmonary veno-occlusive disease is a rare and poorly understood condition that affects the postcapillary pulmonary vasculature, posing diagnostic problems and treatment dilemmas.

We present a patient with veno-occlusive disease and give a short review on the disease.

The patient was a 54-year-old female with a history of the CREST variant of scleroderma. Admitted with dyspnoea, she was treated with epoprostenol in addition to oxygen, diuretics and warfarin. Epoprostenol improved her condition initially; her symptoms grew worse during further medical escalation. With an attempt to stop epoprostenol, however, she became even more dyspnoeic and tolerated best an intermediate dose. She died after three months of treatment with signs of progressive right heart failure.

Veno-occlusive disease may be difficult to diagnose and treat. Clinical signs of pulmonary hypertension without evidence of left ventricular failure may give rise to suspicion of the disease, and high-resolution CT of the lungs with relatively specific findings can be helpful. The prognosis is poor and lung transplantation is the only form of effective treatment. Vasodilators as a bridge to transplantation must be used with caution because of the risk of intolerance and development of pulmonary oedema.