Infliximab treatment in a patient with systemic sclerosis associated with lung fibrosis and pulmonary hypertension

Abstract

This is the first report of the efficacy of anti-TNFalpha treatment in a patient with lung fibrosis and pulmonary hypertension associated with advanced systemic sclerosis, refractory to conventional therapies. The patient was treated with infliximab (5 mg/kg) and methotrexate (10 mg/week) for 1 year. After 6 months of therapy, the echocardiogram showed a reduction in pulmonary pressure, confirmed after 1 year. During treatment, the patient's quality of life improved significantly and high-resolution computed tomography of the chest, lung function tests and blood gas analysis remained stable. After 1 year, the patient decided to stop infliximab therapy (for family reasons related to the distance to our hospital). Lung function tests, pulmonary arterial pressures and blood gas analysis progressively worsened and the patient died 11 months later. Few open-label studies have been conducted on the efficacy of anti-TNFalpha therapy in patients with systemic sclerosis. Here we report our experience in a case of systemic sclerosis complicated by pulmonary fibrosis and hypertension. Infliximab treatment seemed effective, suggesting that controlled randomized trials to evaluate infliximab efficacy in these patients and to compare infliximab with other anti-TNFalpha treatments would be worthwhile.