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Idiopathic pulmonary fibrosis is a progressive and fatal disease. In recent years, some factors involved in the pathophysiology of the alveolar epithelial injury and in the abnormal remodeling that lead to fibrosis have been identified, some factors involved in the pathophysiology of the alveolar epithelial injury and in the abnormal remodeling that lead to fibrosis have been identified, some of them have been evaluated for the development of specific treatments. Two disease-modifying therapies, pirfenidone and nintedanib, have demonstrated to reduce the progression of the disease, in terms of decline in forced vital capacity, with a borderline effect on mortality. These drugs have been approved for patients with mild to moderate impairment in pulmonary function tests. Real-life studies have evaluated these treatments in populations not represented in clinical trials, with similar benefits, safety and tolerance. Pirfenidone and nintedanib have different safety profiles, but none is better in terms efficacy. Decision to start treatment should weight the severity of the disease and the patient’s expectations and preferences. Combined drug treatment possibly will be the standard of treatment in the future, but further studies must assess its efficacy. Recibido 30-04-2018 Aceptado 25-10-2018

Nintedanib y pirfenidona en el tratamiento farmacológico de la fibrosis pulmonar idiopática: perspectivas actuales y futuras