前瞻性登记研究中进行性纤维化间质性肺疾病的患病率及特征

Prevalence and characteristics of progressive fibrosing interstitial lung disease in a prospective registry.

作者信息

Hambly Nathan, Farooqi M Malik, Dvorkin-Gheva Anna, Donohoe Kathryn, Garlick Kristopher, Scallan Ciaran, Chong Sy Giin, MacIsaac Sarah, Assayag Deborah, Johannson Kerri A, Fell Charlene D, Marcoux Veronica, Manganas Helene, Morisset Julie, Comes Alessia, Fisher Jolene H, Shapera Shane, Gershon Andrea S, To Teresa, Wong Alyson W, Sadatsafavi Mohsen, Wilcox Pierce G, Halayko Andrew J, Khalil Nasreen, Cox Gerard, Richeldi Luca, Ryerson Christopher J, Kolb Martin

机构信息

Dept of Medicine, McMaster University, Hamilton, ON, Canada.

Denotes equal contribution.

出版信息

Eur Respir J. 2022 Oct 6;60(4). doi: 10.1183/13993003.02571-2021. Print 2022 Oct.

DOI:10.1183/13993003.02571-2021

PMID:35273032

Abstract

BACKGROUND

Progressive fibrosing interstitial lung disease (PF-ILD) is characterised by progressive physiological, symptomatic and/or radiographic worsening. The real-world prevalence and characteristics of PF-ILD remain uncertain.

METHODS

Patients were enrolled from the Canadian Registry for Pulmonary Fibrosis between 2015 and 2020. PF-ILD was defined as a relative forced vital capacity (FVC) decline ≥10%, death, lung transplantation or any two of: relative FVC decline ≥5% and <10%, worsening respiratory symptoms or worsening fibrosis on computed tomography of the chest, all within 24 months of diagnosis. Time-to-event analysis compared progression between key diagnostic subgroups. Characteristics associated with progression were determined by multivariable regression.

RESULTS

Of 2746 patients with fibrotic ILD (mean±sd age 65±12 years; 51% female), 1376 (50%) met PF-ILD criteria in the first 24 months of follow-up. PF-ILD occurred in 427 (59%) patients with idiopathic pulmonary fibrosis (IPF), 125 (58%) with fibrotic hypersensitivity pneumonitis (HP), 281 (51%) with unclassifiable ILD (U-ILD) and 402 (45%) with connective tissue disease-associated ILD (CTD-ILD). Compared with IPF, time to progression was similar in patients with HP (hazard ratio (HR) 0.96, 95% CI 0.79-1.17), but was delayed in patients with U-ILD (HR 0.82, 95% CI 0.71-0.96) and CTD-ILD (HR 0.65, 95% CI 0.56-0.74). Background treatment varied across diagnostic subtypes, with 66% of IPF patients receiving antifibrotic therapy, while immunomodulatory therapy was utilised in 49%, 61% and 37% of patients with CHP, CTD-ILD and U-ILD, respectively. Increasing age, male sex, gastro-oesophageal reflux disease and lower baseline pulmonary function were independently associated with progression.

CONCLUSIONS

Progression is common in patients with fibrotic ILD, and is similarly prevalent in HP and IPF. Routinely collected variables help identify patients at risk for progression and may guide therapeutic strategies.

摘要

背景

进行性纤维化间质性肺疾病（PF-ILD）的特征是生理功能、症状和/或影像学表现进行性恶化。PF-ILD在现实世界中的患病率和特征仍不明确。

方法

研究对象为2015年至2020年期间从加拿大肺纤维化登记处招募的患者。PF-ILD定义为相对用力肺活量（FVC）下降≥10%、死亡、肺移植，或在诊断后24个月内出现以下任意两项情况：相对FVC下降≥5%且<10%、呼吸症状恶化或胸部计算机断层扫描显示纤维化加重。采用事件发生时间分析比较关键诊断亚组之间的病情进展情况。通过多变量回归确定与病情进展相关的特征。

结果

在2746例纤维化间质性肺疾病患者（平均年龄±标准差为65±12岁；51%为女性）中，1376例（50%）在随访的前24个月内符合PF-ILD标准。PF-ILD发生于427例（59%）特发性肺纤维化（IPF）患者、125例（58%）纤维化性过敏性肺炎（HP）患者、281例（51%）无法分类的间质性肺疾病（U-ILD）患者和402例（45%）结缔组织病相关间质性肺疾病（CTD-ILD）患者。与IPF患者相比，HP患者的病情进展时间相似（风险比[HR]为0.96，95%置信区间为0.79-1.17），但U-ILD患者（HR为0.82，95%置信区间为0.71-0.96）和CTD-ILD患者（HR为0.65，95%置信区间为0.56-0.74）的病情进展时间延迟。不同诊断亚型的背景治疗方法各异，66%的IPF患者接受抗纤维化治疗，而CHP、CTD-ILD和U-ILD患者接受免疫调节治疗的比例分别为49%、61%和37%。年龄增加、男性、胃食管反流病和较低的基线肺功能与病情进展独立相关。