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 task force 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 task force identified that patients with CPFE are predominantly male, with a history of smoking, severe dyspnea, relatively preserved airflow rates and lung volumes on spirometry, severely impaired DlCO, 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, and/or 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.

中文翻译：

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肺纤维化和肺气肿联合综合征：ATS/ERS/JRS/ALAT 官方研究声明。

背景：肺气肿在纤维化间质性肺病患者中相对常见。这被称为合并肺纤维化和肺气肿（CPFE）。定义和诊断标准缺乏共识限制了 CPFE 研究。目标：该工作组的目标是回顾 CPFE 的术语、定义、特征、病理生理学和研究重点，并探讨 CPFE 是否是一种综合征。方法：本研究声明由一个委员会制定，该委员会包括 19 名肺科医师、5 名放射科医师、3 名病理学家、2 名方法学家和 2 名患者代表。最终文件得到了重点系统审查的支持，该审查确定并总结了与 CPFE 相关的所有最新出版物。结果：该工作组发现 CPFE 患者主要为男性，有吸烟史、严重呼吸困难、肺活量测定中气流速率和肺容量相对保留、DICO 严重受损、劳力性低氧血症、频繁发生肺动脉高压和预后不良。考虑到肺纤维化和肺气肿的聚集、共同的发病途径、与疾病进展相关的独特考虑因素、并发症风险增加（肺动脉高压、肺癌和/或死亡）以及对以下疾病的影响，委员会建议将 CPFE 确定为一种综合征：临床试验设计。CPFE 的间质性肺疾病和肺气肿有不同的特征。该委员会提供了研究定义和分类标准，并建议 CPFE 研究包括对放射学模式和病理学模式（如果有）的全面描述，包括一些最近描述的模式，例如与吸烟相关的间质纤维化。结论：本声明描述了 CPFE 综合征并强调了研究重点。