Silva Araújo Catarina, Laranjeira Francisco, Simão de Magalhães Mariana, Oliveira Ana, Vale Martinha M, Furtado Inês, Farinha Fátima, Brandão Mariana, Valadão Ivone
Internal Medicine, Unidade Local de Saúde de Braga, Braga, PRT.
Internal Medicine, Hospital da Luz Lisboa, Lisboa, PRT.
Cureus. 2024 Nov 15;16(11):e73734. doi: 10.7759/cureus.73734. eCollection 2024 Nov.
Background Sjögren's syndrome (SS) is a chronic systemic autoimmune disorder characterized by lymphocytic infiltration of the exocrine glands. Although primarily affecting these glands, the syndrome can also involve several organs. Interstitial lung disease is one of the most severe complications associated with SS. Objectives This study aims to analyze the characteristics of lung involvement in patients with primary SS from a reference medical center's population. Methods Retrospective, longitudinal study of primary SS patients diagnosed until December 2022. Clinical and laboratory data were collected and subjected to statistical analysis using SPSS®. A 95% confidence interval was considered, with statistical significance set at p<0.05. Results A total of 126 patients were included, 95.2% of whom were females, with a mean age at diagnosis of 50 years. The median EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) at diagnosis was 3, which decreased to 1 at the last evaluation. Based on the ESSDAI lung domain, nine patients presented with lung disease at diagnosis. This number increased to 18 by the last assessment. Thoracic tomography showed lung disease in 30 patients, with bronchiectasis being the most common finding. Ten patients presented with interstitial lung disease. Pulmonary function tests were abnormal in 18 patients, and the diffusing capacity of the lungs for carbon monoxide was reduced in 32. Patients with lung disease were more likely to experience a dry cough (p<0.01), have positive SSA antibodies (p<0.05), antinuclear antibody (ANA) >1/320 (p<0.01), positive rheumatoid factor (p<0.01), and low C4-complement during follow-up (p<0.05). Conclusions The authors found an association between clinical and analytical findings and the presence of lung disease in SS patients. Further research is essential to identify optimal predictors of SS-associated lung disease, facilitating early diagnosis and intervention for both SS and its related lung complications.
干燥综合征（SS）是一种慢性全身性自身免疫性疾病，其特征是外分泌腺淋巴细胞浸润。尽管该综合征主要影响这些腺体，但也可能累及多个器官。间质性肺疾病是与SS相关的最严重并发症之一。目的：本研究旨在分析一家参考医疗中心原发性SS患者肺部受累的特征。方法：对截至2022年12月诊断的原发性SS患者进行回顾性纵向研究。收集临床和实验室数据，并使用SPSS®进行统计分析。考虑95%置信区间，设定统计学显著性为p<0.05。结果：共纳入126例患者，其中95.2%为女性，诊断时平均年龄为50岁。诊断时的欧洲抗风湿病联盟干燥综合征疾病活动指数（ESSDAI）中位数为3，在最后一次评估时降至1。根据ESSDAI肺部领域，9例患者在诊断时患有肺部疾病。到最后一次评估时，这一数字增加到18例。胸部断层扫描显示30例患者患有肺部疾病，其中支气管扩张是最常见的发现。10例患者患有间质性肺疾病。18例患者肺功能测试异常，32例患者肺一氧化碳弥散量降低。患有肺部疾病的患者更有可能出现干咳（p<0.01）、抗SSA抗体阳性（p<0.05）、抗核抗体（ANA）>1/320（p<0.01）、类风湿因子阳性（p<0.01）以及随访期间C4补体水平低（p<0.05）。结论：作者发现SS患者的临床和分析结果与肺部疾病的存在之间存在关联。进一步研究对于确定SS相关肺部疾病的最佳预测指标至关重要，有助于对SS及其相关肺部并发症进行早期诊断和干预。
