Division of Rheumatology, Department of Internal Medicine, Soonchunhyang University Seoul Hospital, 59 Daesagwan-ro, Yongsan-gu, Seoul, 04401, South Korea.
Department of Radiology, Soonchunhyang University Seoul Hospital, Soonchunhyang University School of Medicine, Seoul, South Korea.
Sci Rep. 2023 Jun 6;13(1):9189. doi: 10.1038/s41598-023-35608-4.
This single-center, retrospective study aimed to investigate the course and prognostic factors of patients with primary Sjögren syndrome-associated interstitial lung disease (pSS-ILD). We included 120 pSS patients who underwent at least two high-resolution computed tomography (HRCT) scans between 2013 and 2021. Clinical symptoms, laboratory data, HRCT findings, and pulmonary function test results were collected. Two thoracic radiologists reviewed the HRCT findings. In patients with pSS without ILD at baseline (n = 81), no development of ILD was found on follow-up (median, 2.8 years). In patients with pSS-ILD (n = 39), total disease extent, extent of coarse reticulation, and traction bronchiectasis increased on HRCT, whereas the extent of ground glass opacity (GGO) decreased at follow-up (median, 3.2 years) (each p < 0.001). In progressive group of pSS-ILD (48.7%), the extent of coarse reticulation and coarseness score of fibrosis were increased at follow-up (p < 0.05). Usual interstitial pneumonia pattern on CT (OR, 15.237) and follow-up duration (OR, 1.403) were independent risk factors for disease progression in patients with pSS-ILD. In both progressive and non-progressive pSS-ILD, GGO decreased, whereas the extent of fibrosis increased even after treatment with glucocorticoid and/or immunosuppressants. In conclusion, progression occurred in approximately half of the pSS-ILD patients with slow gradual deterioration. Our study identified a definite group of progressive pSS-ILD who did not respond to current anti-inflammatory treatment.
这项单中心回顾性研究旨在探讨原发性干燥综合征相关性间质性肺病（pSS-ILD）患者的病程和预后因素。我们纳入了 120 名在 2013 年至 2021 年间至少接受过两次高分辨率计算机断层扫描（HRCT）检查的 pSS 患者。收集了临床症状、实验室数据、HRCT 结果和肺功能检查结果。两名胸部放射科医生对 HRCT 结果进行了回顾。在基线时无ILD 的 pSS 患者（n=81）中，在随访期间未发现ILD 发展（中位数为 2.8 年）。在 pSS-ILD 患者（n=39）中，HRCT 显示总疾病范围、粗网状结构范围和牵引性支气管扩张增加，而磨玻璃影（GGO）范围在随访时减少（中位数为 3.2 年）（p<0.001）。在 pSS-ILD 的进展组（48.7%）中，随访时粗网状结构范围和纤维化粗糙评分增加（p<0.05）。CT 上的寻常型间质性肺炎模式（OR，15.237）和随访时间（OR，1.403）是 pSS-ILD 患者疾病进展的独立危险因素。在进展和非进展的 pSS-ILD 中，GGO 减少，而纤维化范围增加，即使在接受糖皮质激素和/或免疫抑制剂治疗后也是如此。总之，大约一半的 pSS-ILD 患者病情进展缓慢，逐渐恶化。我们的研究确定了一组明确的进展性 pSS-ILD 患者，他们对当前的抗炎治疗没有反应。
