PDF(Pubmed)
Abstract：
BACKGROUND: Patients with fibrotic hypersensitivity pneumonitis (f-HP) have varied clinical and radiologic presentations whose associated phenotypic outcomes have not been previously described. We conducted a study to evaluate mortality and lung transplant (LT) outcomes among clinical clusters of f-HP as characterized by an unsupervised machine learning approach.
METHODS: Consensus cluster analysis was performed on a retrospective cohort of f-HP patients diagnosed according to recent international guideline. Demographics, antigen exposure, radiologic, histopathologic, and pulmonary function findings along with comorbidities were included in the cluster analysis. Cox proportional-hazards regression was used to assess mortality or LT risk as a combined outcome for each cluster.
RESULTS: Three distinct clusters were identified among 336 f-HP patients. Cluster 1 (n = 158, 47%) was characterized by mild restriction on pulmonary function testing (PFT). Cluster 2 (n = 46, 14%) was characterized by younger age, lower BMI, and a higher proportion of identifiable causative antigens with baseline obstructive physiology. Cluster 3 (n = 132, 39%) was characterized by moderate to severe restriction. When compared to cluster 1, mortality or LT risk was lower in cluster 2 (hazard ratio (HR) of 0.42; 95% CI, 0.21-0.82; P = 0.01) and higher in cluster 3 (HR of 1.76; 95% CI, 1.24-2.48; P = 0.001).
CONCLUSIONS: Three distinct phenotypes of f-HP with unique mortality or transplant outcomes were found using unsupervised cluster analysis, highlighting improved mortality in fibrotic patients with obstructive physiology and identifiable antigens.
METHODS: Consensus cluster analysis was performed on a retrospective cohort of f-HP patients diagnosed according to recent international guideline. Demographics, antigen exposure, radiologic, histopathologic, and pulmonary function findings along with comorbidities were included in the cluster analysis. Cox proportional-hazards regression was used to assess mortality or LT risk as a combined outcome for each cluster.
RESULTS: Three distinct clusters were identified among 336 f-HP patients. Cluster 1 (n = 158, 47%) was characterized by mild restriction on pulmonary function testing (PFT). Cluster 2 (n = 46, 14%) was characterized by younger age, lower BMI, and a higher proportion of identifiable causative antigens with baseline obstructive physiology. Cluster 3 (n = 132, 39%) was characterized by moderate to severe restriction. When compared to cluster 1, mortality or LT risk was lower in cluster 2 (hazard ratio (HR) of 0.42; 95% CI, 0.21-0.82; P = 0.01) and higher in cluster 3 (HR of 1.76; 95% CI, 1.24-2.48; P = 0.001).
CONCLUSIONS: Three distinct phenotypes of f-HP with unique mortality or transplant outcomes were found using unsupervised cluster analysis, highlighting improved mortality in fibrotic patients with obstructive physiology and identifiable antigens.
摘要：
背景：纤维化过敏性肺炎（f-HP）患者具有不同的临床和放射学表现，其相关的表型结局以前没有描述过。我们进行了一项研究，以评估以无监督机器学习方法为特征的f-HP临床集群中的死亡率和肺移植（LT）结果。
方法：对根据最近的国际指南诊断的f-HP患者的回顾性队列进行共识聚类分析。人口统计,抗原暴露，放射学,组织病理学,和肺功能的结果以及合并症被纳入聚类分析。Cox比例风险回归用于评估死亡率或LT风险作为每个集群的综合结果。
结果：在336例f-HP患者中发现了三个不同的簇。第1组（n=158，47％）的特征是对肺功能测试（PFT）的轻度限制。第2组(n=46，14%)的特点是年龄较小，较低的BMI,和较高比例的可识别的致病抗原与基线阻塞性生理。第3组（n=132，39％）的特征为中度至重度限制。与第1组相比，第2组的死亡率或LT风险较低（风险比（HR）为0.42；95％CI，0.21-0.82；P=0.01），第3组较高（HR为1.76；95％CI，1.24-2.48；P=0.001）。
结论：使用无监督聚类分析发现了具有独特死亡率或移植结局的三种不同的f-HP表型，强调阻塞性生理学和可识别抗原的纤维化患者死亡率改善。
方法：对根据最近的国际指南诊断的f-HP患者的回顾性队列进行共识聚类分析。人口统计,抗原暴露，放射学,组织病理学,和肺功能的结果以及合并症被纳入聚类分析。Cox比例风险回归用于评估死亡率或LT风险作为每个集群的综合结果。
结果：在336例f-HP患者中发现了三个不同的簇。第1组（n=158，47％）的特征是对肺功能测试（PFT）的轻度限制。第2组(n=46，14%)的特点是年龄较小，较低的BMI,和较高比例的可识别的致病抗原与基线阻塞性生理。第3组（n=132，39％）的特征为中度至重度限制。与第1组相比，第2组的死亡率或LT风险较低（风险比（HR）为0.42；95％CI，0.21-0.82；P=0.01），第3组较高（HR为1.76；95％CI，1.24-2.48；P=0.001）。
结论：使用无监督聚类分析发现了具有独特死亡率或移植结局的三种不同的f-HP表型，强调阻塞性生理学和可识别抗原的纤维化患者死亡率改善。
