Department of Respiratory Research@Alfred, Central Clinical School, Monash University, Melbourne, VIC, Australia; Department of Respiratory and Sleep Medicine, Austin Health, Heidelberg, VIC, Australia; Institute for Breathing and Sleep, Heidelberg, VIC, Australia; Department of Respiratory Medicine, Alfred Health, Melbourne, VIC, Australia.
Department of Medicine, Firestone Institute for Respiratory Health, McMaster University, Hamilton, ON, Canada.
Chest. 2023 Feb;163(2):345-357. doi: 10.1016/j.chest.2022.08.2233. Epub 2022 Sep 8.
BACKGROUND: Functional capacity, as measured by the 6-min walk test (6MWT), is often reduced in fibrotic interstitial lung disease (ILD). This study evaluated longitudinal changes and the prognostic significance of 6MWT parameters, and explored change in oxygenation status as a physiological criterion to define disease progression in patients with fibrotic ILD. RESEARCH QUESTIONS: What are the trajectories and prognostic value of 6MWT parameters in patients with fibrotic ILD? STUDY DESIGN AND METHODS: Using prospective registries in Australia and Canada, patients with idiopathic pulmonary fibrosis (IPF) and non-IPF fibrotic ILD were stratified by the presence of criteria for progressive pulmonary fibrosis (PPF). The cumulative incidence of exertional and resting hypoxemia and changes in 6-min walk distance (6MWD) and composite indices (distance-saturation product and distance-saturation-oxygen product) were determined, with prognostic significance evaluated at the time of meeting criteria for PPF. New-onset exertional or resting hypoxemia was evaluated as another potential criterion for PPF. RESULTS: Patients with IPF/PPF (n = 126) and non-IPF/PPF (n = 227) had a similar cumulative incidence of exertional hypoxemia and annualized decline in 6MWD and composite indices, which varied across each PPF criterion. Patients with IPF/non-PPF (n = 231) and non-IPF/non-PPF (n = 531) had a significantly lower incidence of hypoxemia than those with IPF/PPF, with an annualized increase in 6MWD and composite indices in the non-IPF/non-PPF group. Exertional or resting hypoxemia at the time of meeting criteria for PPF was independently associated with reduced transplant-free survival in IPF and non-IPF, adjusting for patient demographics and lung function. Adding new-onset exertional or resting hypoxemia as a physiological criterion reduced the median time to development of PPF from 11.2 to 6.7 months in IPF and from 11.7 to 5.6 months in non-IPF in patients who eventually met both definitions (P < .001 for both). INTERPRETATION: Patients with IPF/PPF and non-IPF/PPF have comparable deterioration in functional capacity. Oxygenation status provides prognostic information in PPF and may assist in defining disease progression in fibrotic ILD.
背景：纤维化间质性肺疾病（ILD）患者的 6 分钟步行试验（6MWT）测量的功能容量通常降低。本研究评估了 6MWT 参数的纵向变化和预后意义，并探讨了氧合状态的变化作为定义纤维化 ILD 患者疾病进展的生理标准。
研究问题：纤维化 ILD 患者的 6MWT 参数的轨迹和预后价值是什么？
研究设计和方法：使用澳大利亚和加拿大的前瞻性登记处，根据进展性肺纤维化（PPF）标准，将特发性肺纤维化（IPF）和非 IPF 纤维化 ILD 患者分层。确定运动性和静息性低氧血症的累积发生率，以及 6 分钟步行距离（6MWD）和综合指标（距离-饱和度产物和距离-饱和度-氧产物）的变化，并在符合 PPF 标准时评估预后意义。新出现的运动性或静息性低氧血症被评估为 PPF 的另一个潜在标准。
结果：IPF/PPF（n=126）和非 IPF/PPF（n=227）患者的运动性低氧血症发生率和 6MWD 及综合指标的年化下降率相似，每种 PPF 标准之间的变化不同。IPF/非 PPF（n=231）和非 IPF/非 PPF（n=531）患者的低氧血症发生率明显低于 IPF/PPF 患者，非 IPF/非 PPF 组的 6MWD 和综合指标呈年化增加。符合 PPF 标准时出现运动性或静息性低氧血症与 IPF 和非 IPF 患者无移植生存率降低独立相关，调整了患者人口统计学和肺功能因素。在最终符合两种定义的 IPF 和非 IPF 患者中，将新出现的运动性或静息性低氧血症作为生理标准添加后，从 11.2 个月缩短至 6.7 个月（IPF 中 P<0.001），从 11.7 个月缩短至 5.6 个月（非 IPF 中 P<0.001）。
解释：IPF/PPF 和非 IPF/PPF 患者的功能容量都有类似的恶化。氧合状态提供了 PPF 的预后信息，并可能有助于定义纤维化 ILD 的疾病进展。
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