De la exclusión a la certidumbre. El recorrido hacia el diagnóstico de la fibrosis pulmonar idiopática

Marcos, Pedro J.; Valenzuela, Claudia; Ancochea, Julio

doi:10.1016/S0300-2896(12)70045-5

Archivos de Bronconeumología

ISSN: 0300-2896

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Vol. 48. Issue S2.

Actualización en fibrosis pulmonar idiopática

Pages 7-12 (October 2012)

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Actualización en fibrosis pulmonar idiopática

Pages 7-12 (October 2012)

Actualización en fibrosis pulmonar idiopática

DOI: 10.1016/S0300-2896(12)70045-5

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De la exclusión a la certidumbre. El recorrido hacia el diagnóstico de la fibrosis pulmonar idiopática

From exclusion to uncertainty: the route to diagnosing idiopathic pulmonary fibrosis

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Pedro J. Marcosa, Claudia Valenzuelab,c, Julio Ancocheab,c,

Corresponding author

julio.ancochea@uam.es

Autor para correspondencia.

a Servicio de Neumología, Complexo Hospitalario Universitario A Coruña, A Coruña, España

b Servicio de Neumología, Hospital Universitario de La Princesa, Madrid, España

c Instituto de Investigación Sanitaria Princesa (iP), Universidad Autónoma de Madrid, Madrid, España

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Resumen

La fibrosis pulmonar idiopática (FPI) es una enfermedad diferenciada dentro de las neumonías intersticiales idiopáticas. La FPI es progresiva y fibrosante, y está limitada a los pulmones. Afecta generalmente a adultos mayores de 50 años y se asocia con un patrón radiológico y/o histológico de neumonía intersticial usual. Clínicamente cursa con disnea de esfuerzo progresiva y tos seca. Se auscultan estertores crepitantes de predominio en bases en la mayoría de los pacientes y hay acropaquias en el 50%. No existen alteraciones analíticas específicas. El lavado broncoalveolar y la biopsia transbronquial no proporcionan el diagnóstico de fibrosis pulmonar idiopática, pero son útiles para descartar otras entidades. El diagnóstico definitivo requiere: a) la exclusión de otras entidades clínicas definidas o enfermedades pulmonares difusas de causa conocida, y b) la presencia de un patrón histológico de neumonía intersticial usual en el examen de tejido pulmonar obtenido mediante biopsia quirúrgica, la evidencia radiológica de patrón definido de neumonía intersticial usual en la tomografía computarizada de alta resolución, o ambos.

Palabras clave:

Fibrosis pulmonar idiopática

Neumonía intersticial usual

Diagnóstico radiológico e histológico

Abstract

Idiopathic pulmonary fibrosis (IPF) is a differentiated disease within the idiophatic interstitial pneumonias. IPF is progressive and fibrosing and is limited to the lungs. This entity generally affects persons older than 50 years old and is associated with the radiological and/or histological pattern of usual interstitial pneumonia (UIP). Clinically, IPF causes progressive exertional dyspnea and nonproductive cough. In most patients, physical examination reveals fine bibasilar inspiratory crackles and 50% of patients have digital clubbing. There are no specific laboratory alterations. Bronchoalveolar lavage and transbronchial biopsy will not establish the diagnosis of IPF but are useful to exclude other entities. Definitive diagnosis requires: a) exclusion of other, defined clinical entities or diffuse pulmonary diseases of known cause, and b) the presence of a histological pattern of UIP on analysis of pulmonary tissue from surgical biopsy, radiological evidence of the defined pattern of UIP on high-resolution computed tomography, or both.

Keywords:

Idiopathic pulmonary fibrosis

Usual interstitial pneumonia

Radiological and histological diagnosis

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