9. Tratamiento farmacológico en la fibrosis pulmonar idiopática. Drogas modificadoras de enfermedad

Abstract

The natural history of idiopathic pulmonary fibrosis (IPF) is heterogeneous and unpredictable. The course of the disease without treatment, is inevitably progressive, with a poor prognosis. Historical
treatments have varied from corticosteroids and immunosuppressants (azathioprine, cyclophosphamide), to colchicine and N-acetylcysteine. In the last decades, multiple failed therapeutic trials have
been carried out. However, since 2014 in the United States, Europe and other countries, two drugs, called antifibrotic therapy or disease modifiers, are approved for the treatment of IPF: nintedanib and
pirfenidone. The purpose of antifibrotic therapy is to slow down the decline in lung function in patients with IPF up to 50%.