Primary Sjgren's syndrome related optic neuritis

AIM:To determine the clinical features,diagnosis and treatment of the primary Sjogren syndrome(SS)related optic neuritis.METHODS:The clinical data of 8 patients(12 eyes)with primary SS related optic neuritis were analyzed retrospectively.RESULTS:Eight of 128 consecutive patients with optic neuritis resulted from varied causes fulfilled the diagnostic criteria for the primary SS.They presented initially with the signs and symptoms of non-specific optic neuritis,and 5 patients presenting without dryness showed a chronic inflammation of submandibular gland or parotid gland,and lymphocyte infiltration was demonstrated by labial gland biopsy in 2 patients.There were serum positive titers for anti-Sjogren syndrome A(SSA)in 7 patients and anti-Sjogren syndrome B(SSB)in 8 patients.Anti-aquaporin-4(AQP4)antibody was negative in all the 8 patients.Both glucocorticoids and immunosuppressive agent were administered,and visual acuity elevated in 8 eyes(66.7%),3 patients(37.5%)recurred in the follow-up.CONCLUSION:Primary SS related optic neuritis is less common and easily misdiagnosed.The conventional therapies for optic neuritis could not control the recurrence.

Cutaneous mucosa-associated lymphoid tissue lymphoma complicating Sjögren's syndrome:A case report and review of literature

BACKGROUND The association of Sjögren's syndrome(SS)and lymphoma is similar.Mucosaassociated lymphoid tissue(MALT)or extranodal marginal zone B-cell lymphoma was the most common lymphomatous histology in SS patients.MALT in SS patients is frequently located in the parotid gland,while MALT lymphoma of the skin with SS is an exceedingly rare entity that needs to be recognized.CASE SUMMARY A 60-year-old woman presented with a 3-year history of progressive dry mouth associated with a 1-year history of enlarging cutaneous nodules.Physical examination revealed two hard subcutaneous nodules on her right lower leg.The results of Schirmer’s test were positive,despite the absence of dry eyes.Labial salivary gland biopsy revealed lymphocytic infiltration and chronic inflammation with a focus score of 2.The patient was diagnosed with SS.She underwent resection of one cutaneous nodule,and histopathological analysis identified the nodule as MALT lymphoma.Her dry mouth symptoms improved,and the nodules decreased after 6 mo of treatment with hydroxychloroquine sulfate and chemotherapy(thalidomide,cyclophosphamide,and dexamethasone).CONCLUSION Lymphoma is a severe complication of SS,shown by the reported unique case of cutaneous MALT lymphoma with SS.

Iguratimod in treatment of primary Sjögren’s syndrome concomitant with autoimmune hemolytic anemia:A case report

BACKGROUND Primary Sjögren's syndrome(pSS)concomitant with autoimmune hemolytic anemia(AIHA)but without eye and mouth dryness is exceedingly rare.Iguratimod(IGU)has been widely used in the treatment of pSS.However,there are few reports about the application of IGU in pSS concomitant with AIHA.CASE SUMMARY Here,we present the case of a patient with pSS concomitant with AIHA but without eye and mouth dryness.The patient was initially diagnosed with hyperplastic anemia and AIHA while pSS was missed,and was finally diagnosed with pSS concomitant with AIHA.The patient was treated with IGU along with prednisone and hydroxychloroquine,and her hemoglobin,reticulocytes and IgG returned to normal levels.CONCLUSION IGU was effective for and well tolerated by our patient with pSS concomitant with AIHA,and may be a promising therapy for the treatment of this disease.

AA Amyloidosis Secondary to Primary Sjögren Syndrome: Can It Be Developed without Chronic Inflammation?

Background: The association of primary Sjögren syndrome (PSS) and AA amyloidosis is a rare occurrence. Objective: To describe the phenotype of patients with this association through our two cases and a literature review. Materials and methods: A report of two cases of AA amyloidosis complicating primary Sjögren syndrome with a literature review. Results: Eight patients of Primary Sjögren’s Syndrome complicated by AA amyloidosis were studies. Six cases were reported in the literature by consulting several databases. 50% of patients had a positive immunological assessment, three cases with kidney damage, and three cases lung damage. Conclusion: The immunological activity in the Primary Sjogren’s Syndrome requires the search not only a lymphoma but also AA amyloidosis apart from any clinical or biological chronic inflammation.

Role of JAK-STAT signaling pathway in pathogenesis and treatment of primary Sjögren’s syndrome

Primary Sjögren’s syndrome(pSS)is a systemic autoimmune disease with high prevalence and possible poor prognosis.Though the pathogenesis of pSS has not been fully elucidated,B cell hyperactivity is considered as one of the fundamental abnormalities in pSS patients.It has long been identified that Janus kinases-signal transducer and activator of transcription(JAK-STAT)signaling pathway contributes to rheumatoid arthritis and systemic lupus erythematosus.Recently,increasing numbers of studies have provided evidence that JAK-STAT pathway also has an important role in the pathogenesis of pSS via direct or indirect activation of B cells.Signal transducer and activator of transcription 1(STAT1),STAT3,and STAT5 activated by various cytokines and ribonucleic acid contribute to pSS development,respectively or synergically.These results reveal the potential application of Janus kinase inhibitors for treatment of pSS,which may fundamentally improve the quality of life and prognosis of patients with pSS.

原发性干燥综合征并肝损害13例临床特征及中医证型分析

目的:分析原发性干燥综合征并肝损害临床特征及中医证型状况,为早期改善患者预后提供参考。方法:选择2018年9月至2022年1月广州中医药大学东莞医院风湿科诊治的原发性干燥综合征患者72例作为研究对象,调查、记录患者的电子病历系统,随访患者的预后,重点记录临床特征及中医证型。结果:72例患者中肝损害13例,占比18.1%,女性13例,临床上首发主要症状以口干、眼干为主。肝损害以GGT、ALP升高为主,ALT、AST升高不明显,13例患者均出现抗核抗体(ANA)阳性,中医分型判定为肝郁脾虚型6例、脾肾不足型4例、气阴两虚型2例、阴虚津亏型1例。13例患者经过中西医结合治疗后都顺利出院,2~3周后复查肝功能恢复至正常。结论:临床医师应对原发性干燥综合征肝损害、自身免疫性肝病的异同有充分认识,分析各种实验室检查结果,及早明确诊断、分析中医证型并给予相应治疗方案。

五运六气与干燥综合征发病特点及中医证候的相关性研究

目的:探讨原发性干燥综合征(pSS)患者出生时的五运六气等相关信息与pSS发病所累及的系统损害及中医证候的相关性。方法:采用回顾性研究方法,从门诊及住院部收集2020年1月—2021年12月就诊的pSS患者,收集其出生时的运气学相关信息资料,统计运、气因素出现的频次,分析其与pSS发病所累及的系统损害及中医证候相关的运气因素,并运用中医理论进行分析、总结、探讨。结果:岁运因素中“土不及”是pSS患者的易患禀赋因素;岁运因素中“土不及”、六气因素中的“太阴司天、太阳在泉”及“厥阴司天、少阳在泉”是pSS气阴两虚证的易患禀赋因素;岁运因素中“土不及”“木太过”“金太过”“水太过”“水不及”,六气因素中“少阴司天、阳明在泉”及“少阳司天、厥阴在泉”为阴虚津亏证pSS的易患禀赋因素;岁运因素中“火太过”,六气因素中“太阴司天、太阳在泉”“少阴司天、阳明在泉”是阴虚热毒证pSS的易患禀赋因素;岁运因素中“木太过”“金不及”“木不及”,六气因素中“厥阴司天、少阳在泉”为阴虚血瘀证pSS的易患禀赋因素;岁运因素中“土不及”“金不及”,六气因素中“太阴司天、太阳在泉”为pSS患者关节受累的易患禀赋因素;岁运因素中“土不及”“金不及”为pSS患者肺受累的易患禀赋因素;岁运因素中“火太过”“土太过”“土不及”,六气因素中“厥阴司天、少阳在泉”为pSS患者血液系统受累的易患禀赋因素。结论:原发性干燥综合征的发病特点及其中医证候与五运六气具有一定的相关性。

Identification and functional analysis of shared gene signatures between systemic lupus erythematosus and Sjogren's syndrome

Background:Systemic lupus erythematosus(SLE)is an autoimmune disease that can affect multiple systems.Sjogren's syndrome(SS)is an autoimmune disease that may be primary SS(pSS)or occur together with other autoimmune diseases,including SLE.This study aimed to explore the shared gene signatures in SLE and pSS.Methods:Gene expression data sets of SLE(GSE50772 and GSE81622)and pSS(GSE84844 and GSE48378)were obtained and analyzed for differentially expressed genes(DEGs)in peripheral blood mononuclear cells(PBMCs).A protein–protein interaction(PPI)network was constructed.Gene ontology(GO)and KEGG pathway enrichment analysis were carried out for the DEGs.Results:We screened 232 and 110 DEGs from the SLE and pSS data sets,respectively.We found 32 shared DEGs,which were all upregulated in patients compared with controls.Among these 32 DEGs,11 genes showed a more than twofold change in all data sets(IFI27,IFI44L,RSAD2,IFIT1,IFI44,USP18,IFI6,HERC5,EPSTI1,OAS1,and OAS3).PPI analysis showed that 29 genes interacted with each other.GO analysis showed that these 32 shared DEGs were mainly enriched in biological processes associated with the type Ⅰ interferon signaling pathway,defense response to viruses,response to viruses,negative regulation of viral genome replication,and the immune response.Kyoto Encyclopedia of Genes and Genomes pathway analysis showed that these 32 DEGs were related to virus infection.Conclusion:This study showed that alterations to biological processes associated with the response to virus infection play critical roles in both SLE and pSS.

原发性干燥综合征继发免疫性血小板减少症的临床特点及危险因素分析

目的:了解原发性干燥综合征(pSS)相关继发免疫性血小板减少症(ITP)的发生率和临床特点。方法:回顾性收集498例pSS病人的临床资料及实验室检查结果,比较pSS继发ITP病人(观察组)和血小板计数正常病人(对照组)间临床表现、实验室检查及免疫学指标特征。结果:498例pSS病人中,有17.86%(89/498)病人继发ITP。与对照组比较,观察组病人表现出更高的疾病活动度、更低的补体C3水平(P<0.01)。此外,pSS继发ITP病人较少发生关节炎(15.8%vs 5.6%,P<0.05)及间质性肺病(16.5%vs 4.5%,P<0.05)。结论:继发性ITP是pSS病人常见并发症。其主要临床特点有更低的补体C3水平及更高的疾病活动度评分,而间质性肺病和关节炎的发生是继发ITP的独立保护因素。

IL-10-producing regulatory B cells restrain the T follicular helper cell response in primary Sjögren’s syndrome

Increased numbers of T follicular helper(Tfh)cells have been implicated in the development of autoimmune diseases including primary Sjögren’s syndrome(pSS),but how the Tfh cell response is regulated during autoimmune pathogenesis remains largely unclear.Here,we first found negative correlations between IL-10^(+)regulatory B(Breg)cell numbers and Tfh cell responses and disease activity in patients with pSS and mice with experimental Sjögren’s syndrome(ESS).Moreover,we detected high expression of IL-10 receptor on Tfh cells and their precursors in both humans and mice.In culture,IL-10 suppressed human and murine Tfh cell differentiation by promoting STAT5 phosphorylation.By using an adoptive transfer approach and two-photon live imaging,we found significantly increased numbers of Tfh cells with enhanced T cell homing into B cell follicles in the draining cervical lymph nodes of RAG-2−/−mice transferred with IL-10-deficient B cells during ESS development compared with those of RAG-2−/−mice transferred with wild-type B cells.In ESS mice,CD19^(+)CD1d^(hi)CD5^(+)Breg cells with decreased IL-10 production exhibited severely impaired suppressive effects on T cell proliferation.Consistently,CD19^(+)CD24^(+)CD38^(hi) Breg cells from pSS patients showed significantly reduced IL-10 production with defective inhibitory function in the suppression of autologous Tfh cell expansion.Furthermore,the adoptive transfer of IL-10-producing Breg cells markedly suppressed the Tfh cell response and ameliorated ESS progression in ESS mice.Together,these findings demonstrate a critical role for IL-10-producing Breg cells in restraining the effector Tfh cell response during pSS development.

Diagnosis of Liver Involvement in Primary Sj(o)gren Syndrome

Liver involvement was one of the first extraglandular manifestations to be reported in patients with primary Sj(o)gren syndrome (SS).In the 1990s,a study of liver involvement in patients with primary SS integrated the evaluation of clinical signs of liver disease,liver function and a complete panel of autoantibodies.Recent developments in the field of hepatic and viral diseases have significantly changed the diagnostic approach to liver involvement in SS.The most recent studies have shown that,after eliminating hepatotoxic drugs and fatty liver disease,the two main causes of liver disease in primary SS are chronic viral infections and autoimmune liver diseases.The differential diagnosis of liver disease in primary SS (viral vs autoimmune) is clinically important,since the two processes require different therapeutic approaches and have different prognoses.With respect to viral infections,chronic HCV infection is the main cause of liver involvement in SS patients from the Mediterranean area,while chronic HBV infection may be the main cause of liver involvement in SS patients from Asian countries.After eliminating viral hepatitis,primary biliary cirrhosis (PBC) should be considered the main cause of liver disease in primary SS.PBC-related SS patients may have a broad spectrum of abnormalities of the liver,including having no clinical or analytical data suggestive of liver disease.Autoimmune hepatitis (AIH) is the second most frequently found autoimmune liver disease to be associated with SS (all reported cases are type I),and nearly 10% of these patients have an AIH-PBC overlap.Finally,IgG4-related disease must be investigated in patients with SS presenting with sclerosing cholangitis,especially when autoimmune pancreatitis or retroperitoneal fibrosis are also present.

Concomitant extrahepatic autoimmune diseases do not compromise the long-term outcomes of primary biliary cholangitis

Background:Primary biliary cholangitis(PBC)patients often have concomitant extrahepatic autoimmune(EHA)diseases including Sjögren’s syndrome(SS),systemic sclerosis(SSc),rheumatoid arthritis(RA),and autoimmune thyroid disease.The present study aimed to describe the prevalence of EHA diseases in PBC and explore the impact of EHA diseases on the long-term outcomes of PBC in Chinese patients.Methods:Medical records of PBC patients diagnosed in our institute were retrospectively reviewed.Pa-tients were followed up by a standardized telephone interview.The endpoints were defined as liver-related death and/or liver transplantation.Results:Totally 247 of the 985(25.1%)PBC patients enrolled in the study had at least one concomi-tant EHA disease.Sjögren’s syndrome(n=140,14.2%)was the most frequent one,followed by rheuma-toid arthritis(RA)(n=56,5.7%)and Hashimoto’s thyroiditis(n=45,4.6%).Patients with EHA dis-eases were more common in females(P<0.001)and in those with a family history of autoimmune disease(P=0.017).Overall,no differences were found between PBC patients with and without EHA dis-eases in terms of biochemical response rates to ursodeoxycholic acid,the incidence of hepatic events,or transplant-free survival.RA and EHA≥2 were protective factors for hepatic events in univariate Cox analysis,but the results became insignificant in multivariate analysis.Conclusions:Concomitant EHA diseases were common in PBC patients but did not compromise the long-term outcomes of PBC.

原发性干燥综合征引发单侧胸腔积液1例

报道1例原发性干燥综合征引发的单侧胸腔积液患者的临床资料,并进行文献复习。

抗SSB抗体阳性和阴性的原发性干燥综合征患者临床及免疫学特征的比较

目的:分析抗干燥综合征抗原B(Sj?gren’s syndrome type B,SSB)抗体阳性的原发性干燥综合征(primary Sjogren’s syndrome,pSS)患者与抗SSB抗体阴性的pSS患者之间临床表现及实验室指标的差异。方法:回顾性收集2009—2019年于北京大学第三医院风湿免疫科住院的pSS患者的病历资料,采用t检验、Mann-Whitney秩和检验、卡方检验和Fisher确切概率法对抗SSB抗体阳性和抗SSB抗体阴性的pSS患者组的临床特征及实验室指标进行分析。结果:共纳入pSS患者142例,其中女性137例,男性5例,平均年龄(54.8±13.3)岁,其中抗SSB抗体阳性患者44例(31.0%)。临床特征方面,抗SSB抗体阳性的pSS患者较抗体阴性患者的就诊年龄和起病年龄更小[就诊年龄:(50.9±14.5)岁vs.(56.5±12.4)岁,P<0.05;起病年龄:(42.2±14.8)岁vs.(49.5±15.3)岁,P<0.05],出现皮疹(29.5%vs.14.3%,P<0.05)、腮腺肿大(27.3%vs.8.2%,P<0.05)、肾小管酸中毒(15.9%vs.4.2%,P<0.05)、免疫性血小板减少(9.1%vs.1.0%,P<0.05)的比例更高;两组患者在口干、眼干、雷诺现象(Raynaud phenomenon)等方面差异无统计学意义。实验室指标方面,抗SSB抗体阳性组患者与阴性组患者相比,类风湿因子(rheumatoid factor,RF)阳性率(85.0%vs.49.4%,P<0.05)及滴度(中位数89.8 IU/mL vs.20.5 IU/mL,P<0.05)、抗核抗体(antinuclear antibody,ANA)滴度(中位数320 vs.160,P<0.05)、抗干燥综合征抗原A(Sjogren’s syndrome type A,SSA)抗体阳性率(97.7%vs.64.3%,P<0.05)、IgG水平(中位数21.0 g/L vs.15.6 g/L,P<0.05)、γ球蛋白升高率(71.4%vs.38.5%,P<0.05)、CD3^(-)CD19^(+)细胞比例[(21.0±11.9)%vs.(13.7±9.6)%,P<0.05]均更高,而CD3^(+)细胞比例[(67.2±14.4)%vs.(76.6±13.1)%,P<0.05]和抗线粒体抗体M2亚型阳性率(10.5%vs.35.6%,P<0.05)相对更低,其余无明显差异。治疗方面,与抗SSB抗体阴性组患者相比,抗SSB抗体阳性组患者应用激素(90.9%vs.73.5%,P<0.05)及免疫抑制剂(54.5%vs.36.7%,P<0.05)的比例更高。结论:与抗SSB抗体阴性pSS患者相比,抗SSB抗体阳性患者的起病年龄更小,临床表现更多样,血清其他自身抗体阳性率/水平及B细胞活化程度更高,治疗上需要应用激素及免疫抑制剂的概率更大,整体倾向于表现出更重的临床表型。

原发性干燥综合征合并血液系统受累的危险因素分析

目的分析原发性干燥综合征(pSS)合并血液系统损害患者的临床特征,并建立预测模型。方法收集183例pSS患者的临床表现、实验室检查结果,对合并(n=109)或不合并(n=74)血液系统损害的pSS患者进行对比分析,采用Logistic回归方法分析pSS患者合并血液系统损害的相关因素。建立pSS合并血液系统损害的多因素逻辑回归(MLR)预测模型和人工神经网络(ANN)预测模型,绘制受试者工作特征曲线并比较2种模型的基本参数和曲线下面积(AUC),以评估模型预测效能。结果Logistic回归分析显示,肝脏受累(OR=0.191,P<0.05,95%CI:0.070~0.524)、皮肤受累(OR=0.292,P<0.05,95%CI:0.121~0.704)、低白蛋白血症(OR=0.840,P<0.05,95%CI:0.743~0.948)、低钾血症(OR=0.351,P<0.05,95%CI:0.145~0.846)、高免疫球蛋白M(IgM)(OR=1.732,P<0.05,95%CI:1.085~2.765)、高红细胞沉降率(ESR)(OR=1.028,P<0.05,95%CI:1.005~1.051)和抗SSA抗体阳性(OR=0.21,P<0.05,95%CI:0.052~0.828)是pSS患者合并血液系统损害的危险因素。在预测血小板和白细胞下降方面,MLR和ANN模型的预测效能比较,差异无统计学意义(P>0.05)。ANN模型对血红蛋白下降方面的预测效能优于MLR模型,差异有统计学意义(P<0.05)。结论pSS患者发生血液系统损害的风险较高。肝脏功能异常、皮肤受累、低白蛋白血症、低钾血症、高IgM、高ESR和抗SSA抗体阳性是pSS患者血液系统受累的危险因素。ANN和MLR模型是基于本研究的原始数据开发的2个有效预测工具,可考虑应用于临床。

CENP-B抗体阳性的原发性干燥综合征患者的临床和免疫学特征

目的:探讨着丝点蛋白B(centromere protein B,CENP-B)抗体阳性的原发性干燥综合征(primary Sj9gren’s syndrome,pSS)患者的临床和免疫学特征。方法:采用横断面研究的研究设计,回顾性纳入2016年1月至2022年8月就诊于北京大学口腔医院并确诊为pSS的患者,收集分析患者的一般及临床特征、唾液腺造影、唇腺活检病理、血清免疫学及生化检查等资料。将pSS患者分为CENP-B抗体阳性组和阴性组,用SPSS 23.0软件对数据进行描述性分析、单因素及相关性分析,并将CENP-B抗体阳性组分为CENP-B抗体单阳性组及合并其他抗体阳性组进行亚组分析。结果:共纳入288例pSS患者,其中CENP-B抗体阳性组75例(26.0%),阴性组213例(74.0%)。CENP-B抗体阳性组的发病年龄较大,罹患自身免疫性肝病的比例较高,唾液腺肿大的比例较低,SSA/Ro60、Ro52、SSB抗体的阳性率均较低,免疫球蛋白G、类风湿因子的水平较低,免疫球蛋白M的水平较高,总蛋白水平较低,而白蛋白/球蛋白比值、谷丙转氨酶、谷草转氨酶、碱性磷酸酶、γ-谷氨酰转移酶、乳酸脱氢酶的水平较高。亚组分析显示,CENP-B抗体单阳性组的总蛋白、免疫球蛋白A水平低于合并其他抗体阳性组。结论:CENP-B抗体阳性的pSS患者具有独特的临床和免疫学特征,表现为疾病活动性较低,较少发生唾液腺肿大,但易罹患自身免疫性肝病,肝功能生化指标水平偏高,提示CENP-B抗体可能是pSS亚型分类的特异性标志物。

干燥综合征患者发生重症新型冠状病毒肺炎的早期预测

目的:探讨血细胞比值及炎症指标对干燥综合征(primary Sj?gren’s syndrome,PSS)合并新型冠状病毒肺炎(coronavirus disease 2019,COVID-19)预后不良的预测价值。方法:选择2022年12月至2023年2月在南昌大学第一附属医院风湿免疫科就诊并具有所需完整临床资料的80例干燥综合征合并COVID-19患者进行回顾性分析,纳入标准:(1)符合2019年美国风湿病学会(American College of Rheumatology,ACR)干燥综合征诊断标准;(2)经实时逆转录聚合酶链式反应严重急性呼吸综合征冠状病毒2核酸检测或抗原检测确诊的COVID-19患者;(3)有所需完整临床资料;(4)年龄>18岁。依据《新型冠状病毒肺炎诊疗方案(试行第十版)》临床分型标准,将轻型、普通型患者合为轻症组,重型及危重型合为重症组。干燥综合征疾病活动判定方法参考欧洲抗风湿病联盟(European League Against Rheumatism,EULAR)制定的干燥综合征病情评估指数(EULAR Sj?gren’s syndrome disease activity index,ESSDAI)评分。比较两组患者感染后24~72 h内的血小板-淋巴细胞比值(platelet-lymphocyte ratio,PLR)和C反应蛋白-淋巴细胞比值(C-reactive protein-lymphocyte ratio,CLR)及红细胞沉降率(erythrocyte sedimentation rate,ESR)、C反应蛋白(C-reactive protein,CRP)等实验室资料。结果:轻症组66例,平均年龄(51.52±13.16)岁;重症组14例,平均年龄(52.64±10.20)岁。重症组患者的疾病活动度、CRP、血小板、PLR和CLR明显高于轻症组(P<0.05)。以轻、重症为因变量,分别以年龄、疾病活动度、CRP、血小板、PLR和CLR作为自变量进行单因素分析,提示疾病活动、CRP、PLR和CLR与COVID-19的严重程度相关(P<0.05)。多因素二元Logisitic回归分析进一步证实PLR(OR=1.016,P<0.05)、CLR(OR=1.504,P<0.05)是COVID-19重症患者的独立危险因素。ROC曲线分析显示PLR和CLR的曲线下面积分别为0.708(95%CI:0.588~0.828)和0.725(95%CI:0.578~0.871),敏感度分别为0.429和0.803,特异度分别为0.714和0.758,PLR和CLR的最佳分界值分别为166.214和0.870。结论:PLR和CLR,尤其是CLR,或许是预测干燥综合征患者COVID-19预后的简易而有效的指标。

血清转化生长因子β1与白细胞介素6水平对原发性干燥综合征并发神经系统病变的预测价值

目的:探讨血清转化生长因子β1(TGF-β1)、白细胞介素6(IL-6)水平对原发性干燥综合征(pSS)并发神经系统病变的预测价值。方法:选取80例pSS患者为研究对象,根据是否并发神经系统病变分为观察组(n=26)和对照组(n=54)。比较两组患者一般资料、病理特征、相关生化指标及自身抗体;多因素Logistic回归分析影响pSS并发神经系统病变的因素,ROC曲线分析TGF-β1和IL-6对pSS并发神经系统病变的预测价值。结果:与对照组相比,观察组患者病程更短、口干症与干眼症发生率更低、ESSDAI评分更高、TGF-β1与IL-6水平更高、补体C_4水平更低(P<0.05)。回归分析显示,TGF-β1和IL-6是pSS并发神经系统病变的独立危险因素(OR=1.759、1.791,P<0.05)。ROC曲线分析显示,TGF-β1、IL-6及二者联合预测pSS并发神经系统病变的曲线下面积(AUC)分别为0.882、0.868、0.946,二者联合预测价值更高(P<0.05)。结论:血清TGF-β1、IL-6与神经系统病变的发生密切相关,可以作为pSS并发神经系统病变的潜在生物标志物,为早期诊断和治疗提供重要依据。

基于数据挖掘、网络药理学和分子对接技术探讨中医药治疗原发性干燥综合征的用药规律和作用机制

目的探究中医药治疗原发性干燥综合征的用药规律,预测核心药物的潜在作用机制。方法检索SinoMed、CNKI、WanFang Data、VIP数据库中关于中药复方治疗原发性干燥综合征的文献,构建处方数据库,运用R 4.2.0软件进行药物和性味归经频数统计、关联规则及聚类分析,得到核心药物。利用网络药理学方法绘制中药处方-活性化合物分子-核心靶点-pSS调控网络,预测核心药物的潜在作用靶点和通路,并使用分子对接技术验证核心化合物成分与核心靶点的结合能力。结果筛选得到121首治疗原发性干燥综合征的处方,涉及162味中药,前三味高频药物为麦冬、地黄、甘草,使用频次≥10次的30味高频药物以补虚药(补气药、补阴药、补血药)占比最多,其次为清热凉血、活血调经药;性味以甘、苦、寒为主,归经以肝、肺、胃、脾、肾、心经为主;关联规则分析得到核心药对组合29个;高频中药聚类分析得到5个组合。对核心药物进行网络药理学分析得到山柰酚、槲皮素、丹参酮ⅡA、甲基麦芽酮A等核心活性成分和5个核心靶点;富集分析提示主要涉及脂质与脂肪酸代谢、动脉粥样硬化通路、NADP结合、双加氧酶活性调节、白细胞跨内皮迁移等生物学过程;分子对接表明化合物配体与蛋白受体间具备良好的结合能力。结论原发性干燥综合征多以气阴两虚为本,燥热、血瘀致病,中药复方以益气养阴润燥、清热凉血活血为主;多个核心化合物成分对应的多个靶点介入氧化、白细胞迁移等生物过程产生作用。