Long-term functional course of Sjögren's disease-associated interstitial lung disease.

IF 4.3 3区医学 Q1 RESPIRATORY SYSTEM

ERJ Open Research Pub Date : 2024-10-28 eCollection Date: 2024-09-01 DOI:10.1183/23120541.00384-2024

Caroline Diou, Marie-Pierre Debray, Raphaël Porcher, Catherine Bancal, Karime Sacre, Camille Taille, Warda Khamis, Robin Dhote, Raphaël Borie, Hilario Nunes, Yurdagül Uzunhan, Bruno Crestani

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Abstract

Background: Interstitial lung disease (ILD) is common in primary Sjögren's disease (pSD); its functional course is poorly known. Our aim was to characterise the long-term functional course and prognosis in patients with pSD-ILD. We determined the role of baseline demographic and clinical variables in the evolution of lung function and identified risk factors for death or transplantation.

Methods: In a retrospective observational cohort study, patients with pSD and ILD were retrospectively identified from two French ILD centres. Forced vital capacity (FVC) and diffusing capacity of the lungs for carbon monoxide (D _LCO) slopes were obtained from joint models. Latent class mixed models identified clusters of FVC and D _LCO trajectories.

Results: We included 73 patients (63% women, mean age 63 years), with a median follow-up of 9.3 years. At baseline, mean FVC was 73±21% and D _LCO 51±16%. On average, FVC was stable, while there was an annual decline in D _LCO of 1% of the predicted value. Male sex, a pattern of usual interstitial pneumonia (UIP) or indeterminate for UIP on high-resolution computed tomography (HRCT), and features of fibrosis on HRCT, were associated with an accelerated decline in FVC and D _LCO.

Conclusion: We identified clusters of lung function evolution. 1) Two FVC trajectories: patients with stable FVC (n=56, 78%); patients with FVC decline (n=16, 22%) of 2.4% per year, characterised by a low baseline D _LCO (39%) and a higher risk of death or transplantation (HR 52, 95% CI 10-273). 2) Three D _LCO trajectories: patients with stable D _LCO (n=44, 66%); patients with a slow decline in D _LCO (n=12, 18%) of 2.8% per year; patients with a rapid decline in D _LCO (n=11, 16%) of 4.8% per year, characterised by a low baseline D _LCO (41%) and a higher risk of death or transplantation (HR 156, 95% CI 18-1352).

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圣约翰氏病相关性间质性肺病的长期功能病程。

背景：间质性肺病（ILD）在原发性斯约格伦病（pSD）中很常见，但其功能病程却鲜为人知。我们的目的是描述 pSD-ILD 患者的长期功能病程和预后。我们确定了基线人口统计学和临床变量在肺功能演变中的作用，并确定了死亡或移植的风险因素：在一项回顾性观察队列研究中，我们从两个法国 ILD 中心回顾性地识别了 pSD 和 ILD 患者。通过联合模型获得了肺活量（FVC）和一氧化碳肺弥散容量（D LCO）斜率。潜类混合模型确定了 FVC 和 D LCO 的轨迹集群：我们纳入了 73 名患者（63% 为女性，平均年龄 63 岁），中位随访时间为 9.3 年。基线时，平均 FVC 为 73±21%，D LCO 为 51±16%。平均而言，FVC 保持稳定，而 D LCO 每年下降预测值的 1%。男性性别、高分辨率计算机断层扫描（HRCT）显示的寻常间质性肺炎（UIP）或UIP不确定模式以及HRCT显示的纤维化特征与FVC和D LCO的加速下降有关：我们发现了肺功能演变的群集。1）两种FVC轨迹：FVC稳定的患者（n=56，78%）；FVC每年下降2.4%的患者（n=16，22%），其特点是基线D LCO较低（39%），死亡或移植风险较高（HR 52，95% CI 10-273）。2）三种 D LCO 轨迹：D LCO 稳定的患者（样本数=44，66%）；D LCO 缓慢下降的患者（样本数=12，18%），每年下降 2.8%；D LCO 快速下降的患者（样本数=11，16%），每年下降 4.8%，其特征是基线 D LCO 较低（41%），死亡或移植风险较高（HR 156，95% CI 18-1352）。

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来源期刊

ERJ Open Research Medicine-Pulmonary and Respiratory Medicine

CiteScore

6.20

自引率

4.30%

发文量

273

审稿时长

8 weeks

期刊介绍： ERJ Open Research is a fully open access original research journal, published online by the European Respiratory Society. The journal aims to publish high-quality work in all fields of respiratory science and medicine, covering basic science, clinical translational science and clinical medicine. The journal was created to help fulfil the ERS objective to disseminate scientific and educational material to its members and to the medical community, but also to provide researchers with an affordable open access specialty journal in which to publish their work.