Liang Peixuan, Huang Yanli, Hu Ziwei, Zhou Liang, Cai Shaozhe, Zhong Jixin, Dong Lingli
Department of Rheumatology and Immunology, Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology, Wuhan, China.
Department of Rheumatology and Immunology, Yueyang Central Hospital, Yueyang, 414000, Hunan, China.
Clin Rheumatol. 2025 Mar;44(3):1225-1236. doi: 10.1007/s10067-024-07273-z. Epub 2025 Jan 18.
To investigate the clinical and laboratory features of Sjögren's syndrome-associated autoimmune liver disease (SS-ALD) patients and identify potential risk and prognostic factors.
SS patients with or without ALD, who visited Tongji Hospital between the years 2011 and 2021 and met the 2012 American College of Rheumatology (ACR) classification criteria for Sjögren's syndrome, were retrospectively enrolled. The clinical and laboratory data of the enrolled patients, including autoimmune antibodies, were collected and analyzed with principal component analysis, correlation analysis, LASSO regression, and Cox regression.
A total of 117 SS-ALD patients were confirmed out of 568 SS patients. Compared to SS-non-ALD patients (n = 451), SS-ALD patients exhibited more severe involvement of the hepatic and hematologic systems, albeit with less pronounced typical SS symptoms. Disease activity was higher in SS-ALD patients, as indicated by elevated ESR, CRP, and IL-6 levels, particularly in the SS-overlap subgroup. Furthermore, SS-AIH patients without AIH-specific autoantibody testing or with negative testing results had higher AST and ALT levels than those who were autoantibody-positive. Our predictive model, incorporating IgG, IgM, AST, GGT, ALT, and C4, effectively identified ALD complications in SS patients, achieving an AUC of 0.924. Additionally, a grimmer prognosis was associated with higher baseline AST and ALT levels.
SS-ALD patients often manifest with an insidious onset and atypical SS symptoms, yet frequently exhibit severe systemic involvement, intense inflammatory and immune responses, and a poor prognosis. To improve the clinical outcomes in SS-ALD patients, regular monitoring, early identification, and active treatment should be applied. Key Points • The study provided a detailed profile of clinical and laboratory features of SS-ALD and SS-non-ALD patients, contributing to a predictive model of ALD complications in SS patients • SS-ALD patients manifested with an insidious onset but exhibited severe systemic involvement, robust inflammatory and immune responses, and poor prognosis • SS-AIH patients without available testing for AIH-specific autoantibodies or with negative results demonstrated worse liver function, thus routine screening for autoimmune liver antibodies is recommended in SS patients • More severe baseline liver function status was associated with poorer therapeutic responses to routine medications, so early detection and timely intervention are essential for SS patients.
探讨干燥综合征相关自身免疫性肝病（SS-ALD）患者的临床和实验室特征，并确定潜在的风险和预后因素。
回顾性纳入2011年至2021年间就诊于同济医院、符合2012年美国风湿病学会（ACR）干燥综合征分类标准的有或无ALD的SS患者。收集纳入患者的临床和实验室数据，包括自身抗体，并采用主成分分析、相关分析、LASSO回归和Cox回归进行分析。
568例SS患者中，共确诊117例SS-ALD患者。与非ALD的SS患者（n = 451）相比，SS-ALD患者的肝脏和血液系统受累更严重，尽管典型的SS症状不太明显。SS-ALD患者的疾病活动度更高，表现为血沉、C反应蛋白和白细胞介素-6水平升高，尤其是在SS重叠亚组中。此外，未进行AIH特异性自身抗体检测或检测结果为阴性的SS-AIH患者的谷草转氨酶和谷丙转氨酶水平高于自身抗体阳性患者。我们纳入IgG、IgM、谷草转氨酶、γ-谷氨酰转肽酶、谷丙转氨酶和补体C4的预测模型有效地识别了SS患者的ALD并发症，曲线下面积为0.924。此外，基线谷草转氨酶和谷丙转氨酶水平越高，预后越差。
SS-ALD患者常起病隐匿，SS症状不典型，但常表现为严重的全身受累、强烈的炎症和免疫反应，预后较差。为改善SS-ALD患者的临床结局，应进行定期监测、早期识别和积极治疗。要点• 本研究提供了SS-ALD和非ALD的SS患者详细的临床和实验室特征，有助于建立SS患者ALD并发症的预测模型• SS-ALD患者起病隐匿，但表现为严重的全身受累、强烈的炎症和免疫反应，预后较差• 未进行AIH特异性自身抗体检测或检测结果为阴性的SS-AIH患者肝功能较差，因此建议对SS患者常规筛查自身免疫性肝病抗体• 基线肝功能状态越严重，对常规药物的治疗反应越差，因此早期发现和及时干预对SS患者至关重要。
