Combined Pulmonary Fibrosis and Emphysema |
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Authors: | Karina Portillo Carroz Juan Roldán Sánchez Josep Morera Prat |
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Affiliation: | 1. Servei de Pneumologia, Hospital Germans Trias i Pujol, Badalona, Barcelona, Spain;2. Servei de Pneumologia, Hospital Santa Caterina, Salt, Girona, Spain;1. Servicio de Neumología, Complejo Hospitalario Clínico-Universitario de Santiago, Santiago de Compostela, España;2. Grupo Interdisciplinar de Investigación en Neumología, Instituto de Investigaciones Sanitarias de Santiago (IDIS), Santiago de Compostela, España;1. Sección de Epidemiología, Centro de Salud Pública de Alicante, Alicante, España;2. Servicio de Medicina Preventiva, Hospital General Universitario de Alicante, Alicante, España;3. Servicio de Microbiología, Hospital General Universitario de Alicante, Alicante, España;4. Servicio de Microbiología, Hospital Clínico Universitario de San Juan, Alicante, España;5. Servicio de Pediatría, Hospital General Universitario de Alicante, Alicante, España;1. Unidad de Patología Infecciosa e Inmunodeficiencias de Pediatría, Hospital Universitario Vall d’Hebron, Universidad Autónoma de Barcelona, Barcelona, España;2. Servicio de Urgencias de Pediatría, Hospital Universitario Vall d’Hebron, Universidad Autónoma de Barcelona, Barcelona, España;3. Servicio de Microbiología, Hospital Universitario Vall d’Hebron, Universidad Autónoma de Barcelona, Barcelona, España;4. Servicio de Medicina Preventiva y Epidemiología, Hospital Universitario Vall d’Hebron, Universidad Autónoma de Barcelona, Barcelona, España;1. Servicio de Otorrinolaringología, Hospital General de Catalunya, Universitat Internacional de Catalunya, Sant Cugat del Vallès, Barcelona, España;2. Práctica Privada, Sant Cugat del Vallès, Barcelona, España;3. Unidad del Sueño, Hospital General de Catalunya, Sant Cugat del Vallès, Barcelona, España;4. Departamento de Bioestadística, Universitat Internacional de Catalunya, Sant Cugat del Vallès, Barcelona, España;1. Hospital Universitario San Ignacio, Pontificia Universidad Javeriana, Bogotá, Colombia;2. Departamento de Epidemiología Clínica y Bioestadística, Facultad de Medicina, Pontificia Universidad Javeriana, Bogotá, Colombia |
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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. |
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