title: Idiopathic pulmonary fibrosis.
abstract: Idiopathic pulmonary fibrosis is a fibrosing interstitial pneumonia associated with the radiological and/or histological pattern of usual interstitial pneumonia. Its aetiology is unknown, but probably comprises the action of endogenous and exogenous micro-environmental factors in subjects with genetic predisposition. Its diagnosis is based on the presence of characteristic findings of high-resolution computed tomography scans and pulmonary biopsies in absence of interstitial lung diseases of other aetiologies. Its clinical evolution is variable, although the mean survival rate is 2-5 years as of its clinical presentation. Patients with idiopathic pulmonary fibrosis may present complications and comorbidities which modify the disease's clinical course and prognosis. In the mild-moderate disease, the treatment consists of the administration of anti-fibrotic drugs. In severe disease, the best therapeutic option is pulmonary transplantation. In this paper we review the diagnostic and therapeutic aspects of the disease.
authors.affiliation: Servicio de Neumología, Hospital Clínic, Barcelona, España. Electronic address: axaubetmir@gmail.com.
authors.forename: Antoni
authors.initials: A
authors.lastname: Xaubet
authors.affiliation: Servicio de Neumología, Hospital de La Princesa, Madrid, España.
authors.forename: Julio
authors.initials: J
authors.lastname: Ancochea
authors.affiliation: Servicio de Neumología, Hospital de Bellvitge, L'Hospitalet de Llobregat, Barcelona, España.
authors.forename: María
authors.initials: M
authors.lastname: Molina-Molina
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D003131:Combined Modality Therapy; D015316:Genetic Therapy; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000628:therapy; D016040:Lung Transplantation; D010102:Oxygen Inhalation Therapy; D011379:Prognosis; D033581:Stem Cell Transplantation
chemical_list: D000893:Anti-Inflammatory Agents
keywords: Fibrosis pulmonar idiopática; Idiopathic pulmonary fibrosis; Interstitial pneumonia; Neumonía intersticial; Tratamiento; Treatment