Abstract：
Few data are available on the extent to which clinical practice is aligned with international guidelines for the management of idiopathic pulmonary fibrosis (IPF). We investigated the extent to which management guidelines for IPF have been implemented in the US IPF-PRO Registry and associations between implementation of guidelines and clinical outcomes.
We assessed the implementation of eight recommendations in clinical practice guidelines within the 6 months after enrollment: visit to a specialized clinic; pulmonary function testing; use of oxygen in patients with resting hypoxemia and exercise-induced hypoxemia; referral for pulmonary rehabilitation; treatment of gastro-esophageal reflux disease; initiation of anti-fibrotic therapy; referral for lung transplant evaluation. An implementation score was calculated as the number of recommendations achieved divided by the number for which the patient was eligible. Associations between implementation score and outcomes were analyzed using logistic regression and Cox proportional hazards models.
Among 727 patients, median (Q1, Q3) implementation score was 0.6 (0.5, 0.8). Patients with an implementation score >0.6 had greater disease severity than those with a lower score. Implementation was lowest for referral for pulmonary rehabilitation (19.5%) and lung transplant evaluation (22.3%). In unadjusted models, patients with higher implementation scores had a greater risk of death, death or lung transplant, and hospitalization, but no significant associations were observed in adjusted models.
Management guidelines were more likely to be implemented in patients with IPF with greater disease severity. When adjusted for disease severity, no association was found between implementation of management guidelines and clinical outcomes.
We assessed the implementation of eight recommendations in clinical practice guidelines within the 6 months after enrollment: visit to a specialized clinic; pulmonary function testing; use of oxygen in patients with resting hypoxemia and exercise-induced hypoxemia; referral for pulmonary rehabilitation; treatment of gastro-esophageal reflux disease; initiation of anti-fibrotic therapy; referral for lung transplant evaluation. An implementation score was calculated as the number of recommendations achieved divided by the number for which the patient was eligible. Associations between implementation score and outcomes were analyzed using logistic regression and Cox proportional hazards models.
Among 727 patients, median (Q1, Q3) implementation score was 0.6 (0.5, 0.8). Patients with an implementation score >0.6 had greater disease severity than those with a lower score. Implementation was lowest for referral for pulmonary rehabilitation (19.5%) and lung transplant evaluation (22.3%). In unadjusted models, patients with higher implementation scores had a greater risk of death, death or lung transplant, and hospitalization, but no significant associations were observed in adjusted models.
Management guidelines were more likely to be implemented in patients with IPF with greater disease severity. When adjusted for disease severity, no association was found between implementation of management guidelines and clinical outcomes.
摘要：
关于临床实践与特发性肺纤维化（IPF）管理的国际指南一致的程度，几乎没有数据可用。我们调查了美国IPF-PRO注册中心实施IPF管理指南的程度以及指南实施与临床结果之间的关联。
我们评估了纳入后6个月内临床实践指南中8项建议的执行情况：就诊于专科门诊；肺功能检测；静息低氧血症和运动性低氧血症患者使用氧气；转诊肺康复；胃食管反流疾病的治疗；开始抗纤维化治疗；转诊肺移植评估。实施得分计算为实现的建议数量除以患者符合条件的数量。使用逻辑回归和Cox比例风险模型分析实施得分与结果之间的关联。
在727名患者中，中位数（Q1，Q3）实施评分为0.6（0.5，0.8）。实施评分>0.6的患者的疾病严重程度高于评分较低的患者。转诊肺康复（19.5％）和肺移植评估（22.3％）的实施率最低。在未调整的模型中，实施得分较高的患者死亡风险较大,死亡或肺移植，住院，但在校正模型中未观察到显著关联.
治疗指南更有可能在疾病严重程度较高的IPF患者中实施。当调整疾病严重程度时，未发现管理指南的实施与临床结局之间存在关联.
我们评估了纳入后6个月内临床实践指南中8项建议的执行情况：就诊于专科门诊；肺功能检测；静息低氧血症和运动性低氧血症患者使用氧气；转诊肺康复；胃食管反流疾病的治疗；开始抗纤维化治疗；转诊肺移植评估。实施得分计算为实现的建议数量除以患者符合条件的数量。使用逻辑回归和Cox比例风险模型分析实施得分与结果之间的关联。
在727名患者中，中位数（Q1，Q3）实施评分为0.6（0.5，0.8）。实施评分>0.6的患者的疾病严重程度高于评分较低的患者。转诊肺康复（19.5％）和肺移植评估（22.3％）的实施率最低。在未调整的模型中，实施得分较高的患者死亡风险较大,死亡或肺移植，住院，但在校正模型中未观察到显著关联.
治疗指南更有可能在疾病严重程度较高的IPF患者中实施。当调整疾病严重程度时，未发现管理指南的实施与临床结局之间存在关联.
