BACKGROUND: Diagnosing primary Sjogren's syndrome (pSS)-associated interstitial lung disease (ILD) is complex and can be very challenging. In addition, information about the prognostic factors is limited.
AIMS: We aimed to determine the clinical characteristics and prognostic factors that impact pSS-ILD survival.
METHODS: This retrospective review included 84 consecutive patients diagnosed with pSS-ILD. The information analyzed included the clinical characteristics, laboratory findings, and physiological and hemodynamic data. Prognostic factors were identified using a Cox proportional hazards regression model.
RESULTS: The mean age was 60.5 years, and 61.9% were females. The mean time between the onset of symptoms and diagnosis was 21 months (range, 1–98 months). Minor salivary gland biopsy (MSGB) was positive for pSS in 92.3% of the cohort. Fifty percent of the patients had negative autoimmune serology related to pSS. Based on the available hemodynamic data, 40% had pulmonary hypertension (PH), and 20% had severe PH. During follow-up, acute exacerbation was noted in 38% of the cohort. The 5-year survival rate for all patients was 56%. Male sex, usual interstitial pneumonia pattern, and a reduced forced vital capacity were independent predictors of mortality in the pSS-ILD patients.
CONCLUSIONS: A significant delay between the onset of symptoms and diagnosis was noted in our cohort. Importantly, our study highlights the importance of MSGB and emphasizes that clinicians should not rely solely on serological tests to diagnose pSS in ILD patients. The overall survival was poor, and more efforts are needed to diagnose pSS-ILD at an early stage and refer patients to experienced centers.


中文翻译：

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原发性干燥综合征综合症间质性肺疾病的临床特征和结局


背景：原发性干燥综合征（pSS）相关的间质性肺病（ILD）的诊断非常复杂，并且可能非常具有挑战性。另外，关于预后因素的信息是有限的。
目的：我们旨在确定影响pSS-ILD生存的临床特征和预后因素。
方法：这项回顾性回顾性研究纳入了84例被诊断为pSS-ILD的连续患者。分析的信息包括临床特征，实验室检查结果以及生理和血液动力学数据。使用Cox比例风险回归模型确定预后因素。
结果：平均年龄为60.5岁，女性为61.9％。从出现症状到诊断的平均时间为21个月（范围1–98个月）。在92.3％的队列中，小唾液腺活检（MSGB）对pSS呈阳性。50％的患者与pSS相关的自身免疫血清学阴性。根据现有的血液动力学数据，40％的患者患有肺动脉高压（PH），20％的患者患有严重的PH。在随访过程中，有38％的人群发现急性加重。所有患者的5年生存率均为56％。男性，通常的间质性肺炎模式和强迫肺活量降低是pSS-ILD患者死亡率的独立预测指标。
结论：在我们的队列研究中，注意到在症状发作和诊断之间存在明显的延迟。重要的是，我们的研究突出了MSGB的重要性，并强调临床医生不应仅依靠血清学检查来诊断ILD患者的pSS。总体生存率很差，需要更多的努力来尽早诊断pSS-ILD并将患者转至经验丰富的中心。