Syndrome of Combined Pulmonary Fibrosis and Emphysema: An Official ATS/ERS/JRS/ALAT Research Statement

Abstract

Background. The presence of emphysema is relatively common in patients with fibrotic interstitial lung disease. This has been designated combined pulmonary fibrosis and emphysema (CPFE). The lack of consensus over definitions and diagnostic criteria has limited CPFE research. Goals.The objectives of this taskforce were to review the terminology, definition, characteristics, pathophysiology, and research priorities of CPFE, and to explore whether CPFE is a syndrome.Methods. This research statement was developed by a committee including 19 pulmonologists, 5 radiologists, 3 pathologists, 2 methodologists, and 2 patient representatives. The final document was supported by a focused systematic review that identified and summarized all recent publications related to CPFE.Results. This taskforce identified that patients with CPFE are predominantly male, with history of smoking, severe dyspnea, relatively preserved airflow rates and lung volumes on spirometry, severely impaired diffusion capacity for carbon monoxide, exertional hypoxemia, frequent pulmonary hypertension, and a dismal prognosis. The committee proposes to identify CPFE as a syndrome given the clustering of pulmonary fibrosis and emphysema, shared pathogenetic pathways, unique considerations related to disease progression, increased risk of complications (pulmonary hypertension, lung cancer, mortality), and implications for clinical trial design. There are varying features of interstitial lung disease and emphysema in CPFE. The committee offers a research definition and classification criteria, and proposes that studies on CPFE include a comprehensive description of radiologic and, when available, pathological patterns including some recently described patterns such as smoking-related interstitial fibrosis.Conclusions. This statement delineates the syndrome of CPFE and highlights research priorities.

New trends in pulmonary hypertension

“…These patients also had a low D LCO and worse survival, further emphasising the prognostic role of D LCO . This is not restricted to group 1 PAH, as similar findings have also been observed in subjects with group 2 [5], 3 [6] and 4 PH.…”

Síndrome combinado de fibrosis pulmonar y enfisema: albores del tratamiento antifibrótico en el Paraguay

“…Hasta la fecha ningún parámetro funcional se considera óptimo para monitorear el curso del SCFPE. Un grupo de expertos propone identificar a esta entidad como un síndrome, dado que la progresión y las complicaciones (hipertensión pulmonar, cáncer de pulmón exacerbación aguda y mortalidad) devienen de vías patogénicas símiles al enfisema y a la fibrosis pulmonar con quien comparte además el pronóstico sombrío (20) . La sospecha de hipertensión pulmonar debe ser siempre alta en este grupo de pacientes como se vio en dos de los casos relatados.…”

“…Salud. Diciembre 2022; 20(3):148-154 149 combinado de fibrosis pulmonar y enfisema (SCFPE) presenta algunas características llamativas que invitan a estudiarla con mayor profundidad. Dos eventos inauguran una nueva era en el manejo de las EPID: la aprobación de la pirfenidona y al nintedanib para el tratamiento de la fibrosis pulmonar idiopática (3) y la definición de consenso de la fibrosis pulmonar progresiva o FPP, que comprende a una EPID con evidencia radiológica de fibrosis pulmonar que cumpla al menos con dos de los siguientes criterios: empeoramiento de los síntomas respiratorios y evidencia fisiológica o radiológica de progresión de la enfermedad (4) .…”

Pathogenesis, clinical features, and phenotypes of pulmonary hypertension associated with interstitial lung disease: A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative ‐ Group 3 Pulmonary Hypertension

“…including their extent and distribution. 41 It is unknown whether these different presentations have an influence on the development or severity of PH; in other words, whether they constitute distinct phenotypes within the broader phenotype of PH associated with CPFE.…”