The interstitial lung diseases (ILD) are a heterogenous group of disorders with similar clinical presentation, but widely varying prognoses. The use of a pragmatic disease behaviour classification (DBC), first proposed in international guidelines in 2013, categorises diseases into five behavioural classes based on their predicted clinical course. This study aimed to determine the prognostic utility of the DBC in an ILD cohort. Consecutive patients presented at the weekly multidisciplinary meeting (MDM) of a specialist ILD centre were included. MDM consensus was obtained for diagnosis and DBC category (1–5). Baseline and serial clinical and physiological data were collected over the study period (median 3.9 years, range 0–5.4 years). The relationship between DBC and prognostic outcomes was explored. 137 ILD patients, [64 (47%) female] were included with mean age 67.0 ± 1.1 years, baseline FVC% 72.7 ± 1.7, and baseline DLco% 57.8 ± 1.6%. Patients were stratified into DBC by consensus at MDM: DBC1 n = 0 (0%), DBC2 n = 16 (12%), DBC3 n = 10 (7.3%), DBC4 n = 55 (40%), and DBC5 n = 56 (41%). On univariable Cox regression, increasing DBC class was associated with poorer progression-free survival (HR 1.6, 95% CI 1.2–2.0, p < 0.001). On multivariable Cox regression, DBC remained predictive of PFS when combined with age and gender (HR 1.4, 95% CI 1.1–1.9, p = 0.011), baseline FVC% (HR 1.5, 95% CI 1.1–1.8, p = 0.003) and ILD diagnosis (HR 1.6, 95% CI 1.2–2.2, p < 0.0001). DBC as determined at ILD multidisciplinary meeting may be a useful prognostic tool for the management of ILD patients.

中文翻译：

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疾病行为分类：预测间质性肺疾病结果的实用模型

间质性肺疾病（ILD）是一组具有相似临床表现但预后差异很大的异质性疾病。实用疾病行为分类 (DBC) 于 2013 年在国际指南中首次提出，根据预测的临床病程将疾病分为 5 种行为类别。本研究旨在确定 DBC 在 ILD 队列中的预后效用。连续参加 ILD 专业中心每周多学科会议 (MDM) 的患者也被纳入其中。诊断和 DBC 类别获得了 MDM 共识 (1-5)。在研究期间（中位数 3.9 年，范围 0-5.4 年）收集基线和连续临床和生理数据。探讨了 DBC 与预后结果之间的关系。纳入 137 名 ILD 患者，[64 名 (47%) 女性]，平均年龄 67.0 ± 1.1 岁，基线 FVC% 72.7 ± 1.7，基线 DLco% 57.8 ± 1.6%。在 MDM 上通过共识将患者分为 DBC：DBC1 n = 0 (0%)、DBC2 n = 16 (12%)、DBC3 n = 10 (7.3%)、DBC4 n = 55 (40%) 和 DBC5 n = 56（41%）。在单变量 Cox 回归中，增加 DBC 类别与较差的无进展生存期相关（HR 1.6，95% CI 1.2–2.0，p < 0.001）。在多变量 Cox 回归中，结合年龄和性别（HR 1.4，95% CI 1.1–1.9，p = 0.011）、基线 FVC%（HR 1.5，95% CI 1.1–1.8，p = 0.003），DBC 仍然可以预测 PFS。和 ILD 诊断（HR 1.6，95% CI 1.2–2.2，p < 0.0001）。ILD 多学科会议确定的 DBC 可能是 ILD 患者管理的有用预后工具。