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Vol. 46. Issue 12.
Pages 646-651 (January 2010)
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Vol. 46. Issue 12.
Pages 646-651 (January 2010)
Review Article
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Combined Pulmonary Fibrosis and Emphysema
Combinación de fibrosis pulmonar y enfisema
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Karina Portillo Carroza,
Corresponding author
karisoe@yahoo.es

Corresponding author.
, Juan Roldán Sánchezb, Josep Morera Prata
a Servei de Pneumologia, Hospital Germans Trias i Pujol, Badalona, Barcelona, Spain
b Servei de Pneumologia, Hospital Santa Caterina, Salt, Girona, Spain
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Abstract

The combination of pulmonary fibrosis and emphysema (CPFE) is a recently defined syndrome, in which an upper lobe emphysema and lower lobe fibrosis coexist in a single patient. These patients have a characteristic lung function profile, with apparently normal or minimally altered dynamic and static lung volumes, contrasting with a significant reduction of carbon monoxide transfer (DLco), and hypoxemia, which worsens with exercise. Pulmonary hypertension is highly prevalent and is the principal negative prognostic factor for this condition. High resolution computed axial tomography (HRCT) is the main tool to confirm the diagnosis. Cigarette smoking has been proposed as the main factor in its etiology; however, neither pathogenic mechanisms nor the sequence of events involved in this syndrome has been clarified yet. Experimental studies in animal models are providing information on the involvement of some inflammatory mediators in the pathogenesis. There is currently no consensus on the therapeutic approach to be followed in these patients, since the studies published to date on this subject are limited to wellcharacterized series of cases. Therefore, it is a pathology with many unknowns yet to be resolved and highly likely to be underdiagnosed, unless its functional clinical characteristics are taken into account.

Key words:
Emphysema
Pulmonary fibrosis
Carbon monoxide diffusion
Pulmonary hypertension
Smoking
Resumen

La combinación de fibrosis pulmonar y enfisema (CFPE) es un síndrome definido recientemente, en el cual coexisten en un mismo individuo enfisema en lóbulos superiores y fibrosis en lóbulos inferiores. Estos pacientes presentan un perfil funcional respiratorio característico, con volúmenes pulmonares dinámicos y estáticos aparentemente normales o mínimamente alterados que contrastan con una grave alteración de la difusión del monóxido de carbono (DLCO) e hipoxemia arterial, la cual empeora durante el esfuerzo. La prevalencia de hipertensión pulmonar es elevada y representa la principal condición que determina el pronóstico. La tomografía axial computarizada de alta resolución (TCAR) constituye la herramienta primordial para confirmar su diagnóstico. Se ha postulado al humo del tabaco como el principal agente etiológico; sin embargo, ni los mecanismos fisiopatológicos ni la secuencia de eventos involucrados en este síndrome han sido aún dilucidados. Estudios experimentales en modelos animales están proporcionando información sobre la participación de algunos mediadores inflamatorios en su patogenia. Actualmente, no existe un consenso sobre la actitud terapéutica que seguir en estos pacientes, puesto que lo publicado hasta la fecha sobre esta entidad se limita a series de casos bien caracterizadas. Es, por tanto, una patología con múltiples incógnitas todavía por resolver y con alta probabilidad de ser infradiagnosticada si no se tienen en cuenta sus particularidades clínico-funcionales.

Palabras clave:
Enfisema
Fibrosis pulmonar
Difusión del monóxido de carbono
Hipertensión pulmonar
Tabaquismo
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