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Vol. 148. Issue 4.
Pages 170-175 (February 2017)
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Vol. 148. Issue 4.
Pages 170-175 (February 2017)
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DOI: 10.1016/j.medcle.2016.11.035
Idiopathic pulmonary fibrosis
Fibrosis pulmonar idiopática
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Antoni Xaubeta,
Corresponding author
axaubetmir@gmail.com

Corresponding author.
, Julio Ancocheab, María Molina-Molinac
a Servicio de Neumología, Hospital Clínic, Barcelona, Spain
b Servicio de Neumología, Hospital de La Princesa, Madrid, Spain
c Servicio de Neumología, Hospital de Bellvitge, L’Hospitalet de Llobregat, Barcelona, Spain
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Table 1. Factors associated with the etiology of idiopathic pulmonary fibrosis.
Table 2. Prognostic factors of idiopathic pulmonary fibrosis.
Table 3. Complications and comorbidities of idiopathic pulmonary fibrosis.
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Abstract

Idiopathic pulmonary fibrosis is a fibrosing interstitial pneumonia associated with the radiological and/or histological pattern of usual interstitial pneumonia. Its etiology 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 etiologies. 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.

Keywords:
Idiopathic pulmonary fibrosis
Treatment
Interstitial pneumonia
Resumen

La fibrosis pulmonar idiopática es una neumonía intersticial fibrosante asociada al patrón radiológico y/o histológico de la neumonía intersticial usual. No se conoce su etiología, aunque es probable que consista en la acción de diversos factores microambientales exógenos y/o endógenos en sujetos con predisposición genética. El diagnóstico se basa en los hallazgos característicos en la tomografía axial computarizada de alta resolución y biopsia pulmonar en ausencia de enfermedades pulmonares intersticiales de otras causas. La evolución es variable, aunque la supervivencia media es de 2-5 años desde el inicio de los síntomas. Los pacientes con fibrosis pulmonar idiopática pueden presentar complicaciones y comorbilidades, que modifican el curso clínico y el pronóstico. En la enfermedad leve-moderada el tratamiento consiste en la administración de fármacos antifibróticos, y en la enfermedad grave, la mejor opción terapéutica es el trasplante pulmonar. En el presente artículo, se revisan los aspectos diagnósticos y terapéuticos de la enfermedad.

Palabras clave:
Fibrosis pulmonar idiopática
Tratamiento
Neumonía intersticial

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