系统性硬化症合并干燥综合征的临床特征分析及危险因素研究

Analysis of Clinical Characteristics and Risk Factors of Systemic Sclerosis Combined with Sjögren's Syndrome

背景系统性硬化症(SSc)是异质性疾病,常同时合并干燥综合征(SS),SSc患者部分症状与SS相似,临床诊治过程中容易漏诊SS。目的探讨SSc合并SS患者的临床和实验室特点及重叠发病的危险因素。方法回顾性纳入2019—2023年在宁波市医疗中心李惠利医院住院治疗的SSc患者为研究对象,收集患者基线资料和实验室检查结果。依据是否合并SS将患者分为SSc组(n=91)和SSc合并SS组(n=36)。采用多因素Logistic回归分析探究SSc合并SS的危险因素。结果SSc合并SS组患者女性比例、病程、血液受累、局限性皮肤型SSc(lcSSc)、自身免疫性肝病比例高于SSc组,肺部受累比例、环磷酰胺使用比例低于SSc组(P<0.05)。SSc合并SS组患者血小板分布宽度、补体C4、抗硬皮病70抗体检出率低于SSc组,碱性磷酸酶、谷氨酰转肽酶、免疫球蛋白M(IgM)、抗着丝点蛋白B抗体(抗CENP-B抗体)、抗干燥综合征A/Ro52抗体(抗SSA/Ro52抗体)、抗干燥综合征A/Ro60抗体(抗SSA/Ro60抗体)、抗干燥综合征B抗体(抗SSB抗体)、抗线粒体M2抗体(AMA-M2)检出率高于SSc组(P<0.05)。多因素Logistic回归分析结果显示,IgM升高(OR=3.796,95%CI=1.021~14.115)、抗SSA/Ro52抗体阳性(OR=15.099,95%CI=1.750~130.264)、抗CENP-B抗体阳性(OR=11.681,95%CI=1.662~82.097)是SSc合并SS的独立危险因素(P<0.05)。结论SSc合并SS患者同时具备两者的特点,当SSc患者IgM偏高,抗SSA/Ro52抗体、抗CENP-B抗体阳性并出现相应临床症状时,应进行唇腺活检等系统全面的检查,以防漏诊。

Background Systemic sclerosi(SSc)is a heterogeneous disease,often accompanied by Sjögren's syndrome(SS).Some symptoms of SSc patients are similar to SS,and SS is easily missed in the clinical diagnosis and treatment.Objective To explore the clinical and laboratory characteristics of SSc combined with SS and the risk factors of overlapping incidence.Methods A retrospective study was conducted on SSc patients hospitalized and treated at the Ningbo Medical Center Lihuili Hospital from 2019 to 2023.Baseline data and laboratory test results of the patients were collected.Patients were divided into two groups based on the presence or absence of SS:the SSc group(n=91)and the SSc with SS group(n=36).Multivariate Logistic regression analysis was used to explore the risk factors for SSc with SS.Results The proportion of female patients,disease duration,blood involvement,limited cutaneous systemic sclerosis(lcSSc),and the proportion of autoimmune liver disease in the SSc with SS group were higher than in the SSc group,while the proportion of lung involvement and the use of cyclophosphamide were lower(P<0.05).The SSc with SS group had lower platelet distribution width,complement C4,and anti-Scl-70 antibody detection rates compared to the SSc group,while the detection rates of alkaline phosphatase,glutamyl transpeptidase,immunoglobulin M(IgM),anti-centromere protein B antibodies(anti-CENP-B antibodies),anti-Sjögren's syndrome A/Ro52 antibodies(anti-SSA/Ro52 antibodies),anti-Sjögren's syndrome A/Ro60 antibodies(anti-SSA/Ro60 antibodies),anti-Sjögren's syndrome B antibodies(anti-SSB antibodies),and anti-mitochondrial M2 antibodies(AMA-M2)were higher in the SSc with SS group(P<0.05).Multivariate Logistic regression analysis showed that elevated IgM(OR=3.796,95%CI=1.021-14.115),positive anti-SSA/Ro52 antibodies(OR=15.099,95%CI=1.750-130.264),and positive anti-CENP-B antibodies(OR=11.681,95%CI=1.662-82.097)are independent risk factors for SSc with SS(P<0.05).Conclusion The SSc combined with SS patients have the characteristics of both.When SSc patients have high IgM,positive anti-SSA/Ro52 and anti-CENP-B antibodies and corresponding clinical symptoms,systematic and comprehensive examinations such as labial gland biopsy should be performed to prevent missed diagnosis.