Baseline characteristics and survival of patients of idiopathic pulmonary fibrosis: a longitudinal analysis of the Swedish IPF Registry.
Baseline characteristics and survival of patients of idiopathic pulmonary fibrosis: a longitudinal analysis of the Swedish IPF Registry.
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特发性肺纤维化患者的基线特征和生存率：瑞典IPF登记研究的纵向分析
DOI：
10.1186/s12931-021-01634-x
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发表时间：
2021-02-05
影响因子：
5.8
通讯作者：
Sköld CM
中科院分区：
文献类型：
--
作者：
Gao J;Kalafatis D;Carlson L;Pesonen IHA;Li CX;Wheelock Å;Magnusson JM;Sköld CM
特发性肺纤维化（IPF）在现实生活条件下的观察数据很少。我们探讨了瑞典IPF登记研究（SIPFR）中IPF患者的抗纤维化治疗、疾病严重程度和表型。对2014年9月至2020年4月期间入组并随访≥ 6个月的患者进行了研究。评估了人口统计学、合并症、肺功能、复合变量、6分钟步行试验（6 MWT）、生活质量和抗纤维化治疗。使用kappa值及其对死亡率的影响和风险比，评估轻度生理损害（定义为性别-年龄-生理学（GAP）1期）与生理和复合严重程度指标之间的一致性。采用因子分析和两步聚类分析对表型进行鉴定。在变量或组间进行单变量和多变量生存分析。在662例有基线数据的患者中（中位年龄72.7岁，74.0%为男性），480例随访≥ 6个月，5年生存率为48%。肺功能、6 MWT、年龄和BMI是生存的预测因素。在调整年龄、性别、BMI、吸烟状态、用力肺活量（FVC）和一氧化碳弥散量（DLCO）后，接受抗纤维化治疗≥ 6个月的患者的生存率优于未接受治疗的患者[p = 0.007，HR（95% CI）：1.797（1.173-2.753）]。在调整年龄、性别、BMI和吸烟状况及治疗后，轻度生理损害（GAP 1级，复合生理指数（CPI）≤ 45，DLCO ≥ 55%，FVC ≥ 75%，总肺容量（TLC）≥ 65%）的患者生存率更高。第1组患者的生存率最差，主要由患有中重度疾病的男性患者组成，基线时心脏病患病率增加;第2组患者的特征是轻度疾病，女性超过50%，合并症很少，生存率最高;第3组患者年龄较轻，患有中重度疾病，合并症很少。疾病严重程度、表型和抗纤维化治疗与IPF结局密切相关，接受治疗的患者存活时间更长。表型可能有助于预测IPF患者的结局，并提示患者需要特殊管理，而单一或复合变量作为疾病预测因子具有一定的局限性。
Observational data under real-life conditions in idiopathic pulmonary fibrosis (IPF) is scarce. We explored anti-fibrotic treatment, disease severity and phenotypes in patients with IPF from the Swedish IPF Registry (SIPFR). Patients enrolled between September 2014 and April 2020 and followed ≥ 6 months were investigated. Demographics, comorbidities, lung function, composite variables, six-minute walking test (6MWT), quality of life, and anti-fibrotic therapy were evaluated. Agreements between classification of mild physiological impairment (defined as gender-age-physiology (GAP) stage 1) with physiological and composite measures of severity was assessed using kappa values and their impact on mortality with hazard ratios. The factor analysis and the two-step cluster analysis were used to identify phenotypes. Univariate and multivariable survival analyses were performed between variables or groups. Among 662 patients with baseline data (median age 72.7 years, 74.0% males), 480 had a follow up ≥ 6 months with a 5 year survival rate of 48%. Lung function, 6MWT, age, and BMI were predictors of survival. Patients who received anti-fibrotic treatment ≥ 6 months had better survival compared to untreated patients [p = 0.007, HR (95% CI): 1.797 (1.173–2.753)] after adjustment of age, gender, BMI, smoking status, forced vital capacity (FVC) and diffusion capacity of carbon monoxide (DLCO). Patients with mild physiological impairment (GAP stage 1, composite physiological index (CPI) ≤ 45, DLCO ≥ 55%, FVC ≥ 75%, and total lung capacity (TLC) ≥ 65%, respectively) had better survival, after adjustment for age, gender, BMI and smoking status and treatment. Patients in cluster 1 had the worst survival and consisted mainly of male patients with moderate-severe disease and an increased prevalence of heart diseases at baseline; Cluster 2 was characterized by mild disease with more than 50% females and few comorbidities, and had the best survival; Cluster 3 were younger, with moderate-severe disease and had few comorbidities. Disease severity, phenotypes, and anti-fibrotic treatment are closely associated with the outcome in IPF, with treated patients surviving longer. Phenotypes may contribute to predicting outcomes of patients with IPF and suggest the patients’ need for special management, whereas single or composite variables have some limitations as disease predictors.
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影响因子：
9.6
作者：
Erbes, R;Schaberg, T;Loddenkemper, R
通讯作者：
Loddenkemper, R
影响因子：
3.1
作者：
Kalafatis, Dimitrios;Gao, Jing;Ferrara, Giovanni
通讯作者：
Ferrara, Giovanni
影响因子：
24.3
作者：
Behr, Juergen;Prasse, Antje;Kreuter, Michael
通讯作者：
Kreuter, Michael
影响因子：
168.9
作者：
Noble, Paul W.;Albera, Carlo;du Bois, Roland M.
通讯作者：
du Bois, Roland M.
DOI：
10.1164/rccm.2009-040gl
发表时间：
2011-03-15
影响因子：
24.7
作者：
Raghu, Ganesh;Collard, Harold R.;Schuenemann, Holger J.
通讯作者：
Schuenemann, Holger J.