Manfredi Andreina, Sambataro Gianluca, Rai Alessandra, Cerri Stefania, Sambataro Domenico, Vacchi Caterina, Cassone Giulia, Vancheri Carlo, Sebastiani Marco
Rheumatology Unit, Azienda Ospedaliera Policlinico di Modena, 41100 Modena, Italy.
Department of Clinical and Experimental Medicine, Regional Referral Center for Rare Lung Disease, Policlinico "G. Rodolico-San Marco", University of Catania, 95100 Catania, Italy.
J Pers Med. 2024 Jul 1;14(7):708. doi: 10.3390/jpm14070708.
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.
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.
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.
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 ( = 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.
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.
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.
间质性肺疾病（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的患病率和表现仍不完全清楚，主要基于回顾性的异质性研究。
调查pSS-ILD多中心队列中进行性肺纤维化（PPF）的患病率。此外，本研究探讨了PPF与pSS的临床、人口统计学和血清学特征之间可能的相关性。
在6个月内纳入所有连续的pSS-ILD患者。收集pSS的临床、人口统计学和血清学特征，不包括肺功能和影像学特征。还调查了除PPF外的ILD临床行为。
共纳入72例患者。65.3%的pSS-ILD患者观察到纤维化ILD模式；其中，55.3%表现为PPF。放射学模式（非特异性间质性肺炎[NSIP]、寻常型间质性肺炎[UIP]或其他）与PPF无关；特别是，PPF患者42.3%的病例为UIP，57.7%为NSIP，与非PPF组无显著差异（P = 0.29）。pSS病程较短、pSS诊断时年龄较大以及抗核抗体频率较低与PPF亚组相关。然而，多因素分析未证实这些关联。
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，UIP和NSIP在pSS患者中向PPF的演变相似；对于NSIP，这种情况比其他风湿性疾病更常见。我们的结果强调了早期识别PPF对于及时干预和密切随访的重要性。
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，UIP和NSIP在pSS患者中向PPF的演变相似；对于NSIP，这种情况比其他风湿性疾病更常见。我们的结果强调了早期识别PPF对于及时干预和密切随访的重要性。
