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Med Clin (Barc). 2017 Feb 23;148(4):170-175. doi: 10.1016/j.medcli.2016.11.004. Epub 2016 Dec 18.

Idiopathic pulmonary fibrosis.

[Article in English, Spanish]

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Servicio de Neumología, Hospital Clínic, Barcelona, España. Electronic address:
Servicio de Neumología, Hospital de La Princesa, Madrid, España.
Servicio de Neumología, Hospital de Bellvitge, L'Hospitalet de Llobregat, Barcelona, España.


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.


Fibrosis pulmonar idiopática; Idiopathic pulmonary fibrosis; Interstitial pneumonia; Neumonía intersticial; Tratamiento; Treatment

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