背景:间质性肺病(ILD)是原发性干燥综合征(pSS)发病和死亡的常见原因。然而,pSS-ILD的患病率和行为仍然不完整,主要基于回顾性异质性研究。
目的:研究pSS-ILD患者多中心队列中进行性肺纤维化(PPF)的患病率。此外,这项研究探讨了PPF与临床之间可能的相关性,人口统计学,和pSS的血清学特征。
方法:所有pSS-ILD患者均在6个月的时间内入选。临床,人口统计学,和pSS的血清学特征,除了功能和放射性肺特征,被收集。还研究了除PPF以外的ILD的临床行为。
结果:纳入72例患者。在65.3%的pSS-ILD患者中观察到纤维化ILD模式;其中,55.3%显示PPF。放射学模式(NSIP,UIP,或其他)与PPF无关;特别是,PFF患者的UIP占42.3%,NSIP占57.7%,与非PPF组没有显着差异(p=0.29)。pSS病程越短,pSS诊断年龄较高,抗核抗体的频率较低与PPF亚组相关。然而,多变量分析未证实这些关联。
结论:本研究为pSS-ILD中PPF的患病率和特征提供了有价值的见解。特别是,在pSS患者中,UIP和NSIP对PPF的演变相似;对于NSIP,这种行为比其他风湿性疾病更常见。我们的结果强调了早期识别PPF对于及时干预和仔细随访的重要性。
结论:本研究为pSS-ILD中PPF的患病率和特征提供了有价值的见解。特别是,在pSS患者中,UIP和NSIP对PPF的演变相似;对于NSIP,这种行为比其他风湿性疾病更常见。我们的结果强调了早期识别PPF对于及时干预和仔细随访的重要性。
BACKGROUND: Interstitial lung disease (ILD) represents a frequent cause of morbidity and mortality in primary Sjogren syndrome (pSS). However, the prevalence and behavior of pSS-ILD remains incomplete, largely based on retrospective heterogeneous studies.
OBJECTIVE: To investigate the prevalence of progressive pulmonary fibrosis (PPF) in a multicentric cohort of patients with pSS-ILD. Additionally, this study explored possible correlations between PPF and clinical, demographic, and serological features of pSS.
METHODS: All consecutive patients with pSS-ILD were enrolled in a 6-month period. Clinical, demographic, and serological features of pSS, other than functional and radiological lung features, were collected. Clinical behaviors of ILD other than PPF were also investigated.
RESULTS: Seventy-two patients were enrolled. A fibrosing ILD pattern was observed in 65.3% of patients with pSS-ILD; among them, 55.3% showed a PPF. The radiologic pattern (NSIP, UIP, or others) was not associated with PPF; in particular, patients with PFF had UIP in 42.3% of cases and NSIP in 57.7%, without a significant difference with respect to the non-PPF group (p = 0.29). Shorter pSS disease duration, higher age at pSS diagnosis, and lower frequency of antinuclear antibodies were correlated with the PPF subgroup. However, multivariate analysis did not confirm these associations.
CONCLUSIONS: This study provides valuable insights into the prevalence and characteristics of PPF in pSS-ILD. In particular, UIP and NSIP showed a similar evolution towards PPF in patients with pSS; for NSIP, this behavior was more frequent than for other rheumatic diseases. Our results emphasize the importance of early recognition of PPF for timely intervention and careful follow-up.
CONCLUSIONS: This study provides valuable insights into the prevalence and characteristics of PPF in pSS-ILD. In particular, UIP and NSIP showed a similar evolution towards PPF in patients with pSS; for NSIP, this behavior was more frequent than for other rheumatic diseases. Our results emphasize the importance of early recognition of PPF for timely intervention and careful follow-up.