干燥综合征治疗应答评估工具的研究与初步验证结果发表

过去20年间, EULAR制定了广为应用的EULAR干燥综合征疾病活动指数(European league against rheumatism Sj?gren’’s syndrome patient reported index, ESSDAI)和EULAR干燥综合征患者报告指数(European league against rheumatism Sj?gren′s syndrome patient reported index, ESSPRI), 分别作为衡量系统活动和患者症状的参考指标。ESSDAI在监测疾病活动变化和评估治疗效果中表现良好, 但也存在局限性。作为RCT中的主要终点, ESSDAI未能涵纳pSS所有重要的疾病特征, 如患者症状和腺体功能, 因此, 目前应该建立一个可以更全面评估疾病的工具。     

干燥综合征睡眠质量及其相关因素

目的调查原发性干燥综合征患者睡眠质量及影响睡眠质量的相关因素,为改善干燥综合征患者睡眠质量提供依据。方法采用匹兹堡睡眠质量指数(Pittsburgh sleep quality index,PSQI)、医院焦虑抑郁量表(hospital anxiety and depression scale,HADS)、疲劳严重度量表(ftigue severity scale,FSS)、欧洲干燥综合征疾病活动指数(EULAR Sj?gren’s syndrome disease activity index,ESSDAI)、欧洲干燥综合征患者报告指数(EULAR Sj?gren’s syndrome patient reported index,ESSPRI)及自制一般情况调查表对门诊的68例原发性干燥综合征患者进行问卷调查,分析睡眠障碍与人口学、临床资料、实验室检查、疾病活动性的相关性。结果患者PSQI总分(7.7±3.6)分,明显高于健康人群(3.9±2.5);不同性别患者睡眠障碍的检出率差异无统计学意义(P0.05)。伴睡眠障碍组在年龄、HADS、FSS、ESSPRI、ESSDAI、IL-6、IgG、血沉方面与不伴睡眠障碍组差异具有统计学意义(P0.05)。睡眠障碍与年龄、HADS、FSS、ESSPRI、IL-6水平均呈正相关(r=0.521、0.756、0.503、0.294、0.915,P0.05),与ESSDAI、血沉、IgG水平负相关(r=-0.345、-0.343、-0.429,P0.05),与CRP无明显相关性(P=0.834)。结论原发性干燥综合征的睡眠障碍明显高于健康人群,相关的因素有年龄、焦虑抑郁状态、疲劳、高IL-6水平、高ESSPRI等。与系统损害的程度呈负相关,提示较高的针对原发病的治疗强度可能改善患者的睡眠情况。     

系统性红斑狼疮趋化因子CXCL13水平升高的临床意义

目的 探讨趋化因子CXCL13在系统性红斑狼疮(systemic lupus erythematosus,SLE患者血浆中的表达及临床意义。方法 收集SLE患者60例,健康对照20例,采用酶联免疫吸附法(ELISA)检测两组血浆中CXCL13的表达水平,并分析与SLE患者实验室指标及疾病活动度的关系;同时比较血浆CXCL13水平在狼疮性肾炎(lupus nephritis,LN)和非LN患者间的表达差异;受试者工作特征曲线(ROC曲线)判断血浆CXCL13对诊断LN的敏感性和特异性。采用t检验、Kruskal-Wallis H检验、Pearson相关分析进行统计学数据分析。结果 SLE患者血浆CXCL13为(272. 1±232. 7) pg/ml,明显高于健康对照组(52. 1±31. 0) pg/ml (P 0. 05)。LN患者血浆CXCL13水平为(340. 9±248. 6) pg/ml,明显高于非LN患者[(134. 5±106. 9) pg/ml; Z=3. 895,P 0. 01]。血浆CXCL13水平与SLE患者SLEDAI评分呈正相关(r=0. 267,P 0. 05),与补体C3 (r=-0. 294,P 0. 05)、外周血血红蛋白含量(r=-0. 299,P 0. 05)、血小板计数(r=-0. 300,P 0. 05)、淋巴细胞计数(r=-0. 309,P 0. 05)呈负相关。疾病活动组患者血浆CXCL13水平明显高于疾病稳定组[(335. 7±248. 2) pg/ml比(144. 9±127. 5) pg/ml,t=3. 223,P 0. 01]。抗ds DNA抗体阳性组血浆CXCL13水平明显高于抗ds DNA抗体阴性组[(367. 0±285. 4) pg/ml比(204. 3±158. 6) pg/ml,t=2. 824,P 0. 01]; AHA阳性组血浆CXCL13水平明显高于AHA阴性组[(375. 3±276. 8) pg/ml比(216. 5±186. 3) pg/ml; t=2. 645,P 0. 05]。LN患者血浆CXCL13水平与血清尿素氮呈正相关(r=0. 425,P 0. 01),与e GFR呈负相关(r=-0. 385,P 0. 05)。血浆CXCL13水平≥116. 95 pg/ml,对LN诊断的敏感性为92. 5%,特异性为60. 0%。结论 SLE患者血浆CXCL13水平明显升高,可能与自身抗体产生及血液系统和肾脏系统损害有关,CXCL13可作为判断SLE疾病活动性的指标之一。     

趋化因子CXCL13在系统性红斑狼疮患者的血浆表达水平及临床意义

目的 检测系统性红斑狼疮(SLE)患者血浆趋化因子CXCL13表达,分析CXCL13表达与SLE疾病活动性及脏器受累的关系,探讨CXCL13在SLE发病机制中的可能作用.方法 酶联免疫吸附法(ELISA)检测60例SLE患者血浆CXCL13水平,并与21名健康体检者对照.组间计量资料比较采用Mann-Whitney检验,组间计量资料相关性分析采用Pearson相关性分析.结果 血浆趋化因子CXCLl3在SLE患者组表达水平明显高于健康对照组[(501±446)和(102±121) pg/ml,P＜0.01].有肾脏及中枢神经系统受累的SLE患者较无相应器官受累的SLE患者有着较高水平的血浆CXCL13表达(P=0.038,0.026).另外,分析发现血浆CXCL13表达水平与SLE疾病活动指数呈正相关(r=0.585,P=0.001).结论 趋化因子CXCL13在SLE发病机制中具有重要作用,可能参与了SLE肾脏及中枢神经系统受累过程;CXCL13可作为判断SLE病情活动性的指标.     

自身免疫性肝炎患者血清趋化因子水平与生物化学指标的相关性

目的 探讨自身免疫性肝炎(AIH)患者外周血中趋化因子(CCL2、CCL5、CXCL8、CXCL9和CXCL10)水平及其与肝脏生物化学指标的相关性. 方法 检测46例AIH患者血清中趋化因子(CCL2、CCL5、CXCL8、CXCL9和CXCL10)水平,选择12例健康体检者为对照组. 结果 AIH组CCL2、CXCL9和CXCL10水平分别为11.79 pg/ml、11.31 pg/ml和15.85 pg/ml,高于健康对照组的8.39 pg/ml、2.69 pg/ml、4.64 pg/ml,两组比较,Z值分别为-1.958、-4.527、-3.84,P值均＜ 0.05,差异均有统计学意义.AIH活动期组血清CCL2、CXCL8、CXCL9和CXCL10水平分别为29.69 pg/ml、7.2 pg/ml、16.02 pg/ml和90.01 pg/ml,明显高于激素治疗后缓解期组的11.16 pg/ml、5.38 pg/ml、5.47 pg/ml、13.24 pg/ml,两组比较,统计值分别为t=2.985、Z=-2.547、Z=-3.187、t=2.12,P值均＜0.05,差异均有统计学意义.AIH患者血清CXCL8与IgG水平呈正相关关系(r2=0.291,P＜0.05);CXCL9与ALT、AST水平均呈正相关关系(r2值分别为0.5324、0.3352,P值均＜0.05);CXCL10与ALT、AST、GGT水平均呈正相关关系(r2值分别为0.9551、0.8960、0.8271,P值均＜0.05). 结论 活动期AIH患者外周血中CCL2、CXCL8、CXCL9和CXCL10水平明显升高,CXCL9和CXCL10水平与肝脏炎症活动指标呈明显正相关关系,提示趋化因子水平可反映AIH肝脏炎症活动程度,在AIH病理损伤中发挥重要作用.     

血清增殖诱导配体在原发性干燥综合征中的临床意义

目的 探讨血清增殖诱导配体(APRIL)对原发性干燥综合征(pSS)患者的临床意义.方法 采用ELISA法分别检测38例pSS确诊患者和40名正常健康体检者的血清APRIL水平,并分析其与pSS患者临床指标相关性.结果 PSS患者的血清APRIL高于健康对照组[(26.37±16.18)ng/ml比(16.33±15.61)ng/ml,P＜0.05],且与pSS患者ESR(r=0.427,P＜0.05) 、唇腺淋巴细胞灶数(r=0.446,P＜0.05)呈一定程度相关性,与外周血白细胞(WBC)、血小板(PLT)计数呈负相关(P＜0.05).结论 pSS患者血清APRIL水平异常增高,与临床指标的相关性,提示该系统可能参与pSS的发病过程.     

原发性干燥综合征患者血清B淋巴细胞刺激因子水平的检测

目的检测原发性干燥综合征(pSS)患者血清中B淋巴细胞刺激因子(BLys)的水平,探讨其在pSS发病机制中的作用。方法pSS患者50例,采用酶联免疫吸附试验(ELISA)检测血清BLyS水平,速率散射比浊法检测类风湿因子(RF)、免疫球蛋白IgG、IgM、IgA及补体C3、C4水平,间接免疫荧光法及免疫印迹法检测抗核抗体(ANA)、抗SSA抗体、抗SSB抗体的水平。对照组30名为我院门诊的健康体检者,性别、年龄构成与pSS患者组匹配。分析BLyS在pSS患者血清中的水平及其与RF、免疫球蛋白、补体、ANA、抗SSA抗体、抗SSB抗体的相关性。结果血清BLyS平均水平在pSS患者组为(560±380)pg/ml,在对照组为(195±93)pg/ml,pSS患者组明显高于对照组(P＜0．01),血清BLyS水平与RF、IgG、丙种球蛋白呈正相关(r=0．598,P＜0．01；r=0．522,P＜0．01；r=0．547,P＜0．01),与IgA、IgM、C3、C4无明显相关性。ANA和抗SSA抗体、抗SSB抗体阳性者血清BLyS水平高于上述指标正常者。结论pSS患者的血清BLyS水平升高,且与免疫球蛋白定量、自身抗体表达有相关性。BLys的异常表达可能是pSS发病的重要环节。     

白细胞介素-17在原发性干燥综合征患者唇腺组织和外周血的表达

目的 探讨白细胞介素(IL)-17在原发性干燥综合征(pSS)患者唇腺组织及外周血及唾液中的表达及意义.方法 采用免疫组织化学的方法对30例DSS患者及5名对照组的唇腺石蜡组织的连续切片进行IL-17、CD45RO和CD20染色.用双抗体夹心酶联免疫吸附试验(ELISA)法检测30例pSS患者和20名健康对照组外周血及唾液中IL-17水平.结果 pSS患者唇腺组织均有IL-17阳性细胞表达;而对照组无表达.在pSS患者唇腺组织少量淋巴细胞浸润组,IL-17阳性细胞数(15±5)个低于多量淋巴细胞浸润组(21±8)个(P＜0.05);但在少量淋巴细胞浸润的pSS组,IL-17阳性的淋巴细胞占总淋巴细胞的比率(14±5)%,高于多量淋巴细胞浸润组(10±4)%(P＜0.05).在大量淋巴细胞聚集的部位,IL-17主要表达在腺体外周的淋巴细胞上.呈胞质表达.相关性分析显示:IL-17阳性细胞数与红细胞沉降率(ESR)呈显著正相关(r=0.557,P＜0.05).血清中IL-17的浓度在多量淋巴细胞浸润组(7.8±0.6)pg/ml与少量淋巴细胞浸润组(7.6±1.0)pg/ml及健康对照组(7.4±1.0)pg/ml相比差异无统计学意义(P＞0.05).结论 IL-17在pSS患者中可能参与局部的炎症损伤过程.     

原发性干燥综合征合并间质性肺疾病97例临床特点分析

干燥综合征(SS)是一种主要累及外分泌腺体的慢性炎症性自身免疫病,常侵犯泪腺和唾液腺,也可侵犯呼吸系统、消化系统、泌尿系统等。SS可分为原发性干燥综合征(pSS)和继发性干燥综合征。其中pSS常合并间质性肺疾病(ILD),早期多无明显症状和体征,晚期可因呼吸衰竭而死亡。本文回顾性分析我院97例住院pSS患者的临床资料,以期提高对pSS合并ILD的诊治水平。     

原发性干燥综合征患者外周血B细胞FcγRⅡb的表达及临床意义

目的 探讨原发性干燥综合征(pSS)患者外周血B细胞FcγRⅡb的表达及其临床意义.方法 流式细胞术检测19例pSS患者、15名健康对照外周血B细胞FcγRⅡb的平均荧光强度(MFI);酶联免疫吸附试验( ELISA)方法测定19例pSS患者血清抗SSA、SSB抗体水平,统计学分析采用t检验、单因素方差分析、SNK-q检验及Pearson相关分析.结果 pSS患者CD 19+CD27+记忆性B细胞亚群的百分率[(20.8±2.7)％,19例]显著低于健康对照组[(37.8±2.2)％,15名](t=-4.002,P＜0.01);活动期pSS患者外周血CD19+CD27+记忆性B细胞FcγRⅡb的MFI[( 74±8),13例]低于非活动期组[(132±11),6例]及健康对照组[(139±12),15名](F=10.699,P＜0.01);pSS患者外周血CD19+CD27+记忆性B细胞FcγRⅡb的MFI与pSS疾病活动指数(SSDAI)呈负相关(r=-0.744,P=0.0003);抗SSA、SSB抗体阳性组pSS患者CD19+CD27+记忆性B细胞FcγRⅡb的MFI[分别为(75±3),12例;(48±7),5例]显著低于抗SSA、SSB抗体阴性组[分别为(122±11),7例;(108±9),14例](t分别为-4.336和-3.776,P均＜0.01);抗SSA抗体阳性组pSS患者CD19+CD27+记忆性B细胞FcγRⅡb的MFI与抗SSA抗体滴度呈负相关(r=-0.685,P=0.014).结论 pSS患者活动期外周血记忆性B细胞FcyRⅡb表达与SSDAI呈负相关,并与抗SSA抗体呈负相关.FcγR Ⅱb表达异常可能在pSS免疫发病机制中起重要作用.     

Clinical predictors of silent but substantial liver fibrosis in primary Sjogren’s syndrome

Objectives: To investigate the prevalence and the predictors of silent but substantial liver fibrosis in patients with primary Sjogren’s syndrome (pSS).

Methods: We enrolled 101 pSS patients with normal liver function and structures, and without significant liver diseases or other conditions affecting liver fibrosis. The European league against rheumatism (EULAR) SS patients reported index (ESSPRI) and the EULAR SS disease activity index (ESSDAI) were analyzed. Liver stiffness (LS) was measured using transient elastography and 7.4 kPa was determined as the cutoff value for significant liver fibrosis.

Results: The median age of patients (91women) was 53 years and the median LS value was 4.7 kPa. The median ESSPRI and ESSDAI showed no correlation with LS values. Twelve patients (11.9%) had significant liver fibrosis. In multivariate logistic regression, white blood cells count ≤4000.0/mm³ (Odds ratio [OR] 9.821), serum albumin?≤3.8?mg/dL (OR 16.770) and aspartate aminotransferase (AST) ≥?27.0 IU/L (OR 20.858) independently predicted silent but substantial liver fibrosis in pSS patients.

Conclusions: The prevalence of silent but substantial liver fibrosis was 11.9% in pSS and its predictors were leukopenia, decreased serum albumin and increased AST levels.     

Neuromyelitis optica--complication or comorbidity in primary Sjögren's syndrome?

We report two cases of neuromyelitis optica (NMO) associated with primary Sj?gren's syndrome (pSS), comparing the clinical and laboratory features of these predominant neurological patients and reporting their different outcome. NMO - a severe demyelinating disorder of the central nervous system - primarily affects the spinal cord and optic nerves, resulting in longitudinally extensive transverse myelitis and/or optic neuritis. Our patients had a late pSS diagnosis, due to the absence of sicca syndrome and specific Sj?gren serology in the early stages of their diseases, when the neurological symptoms prevailed. Many NMO patients have an accompanying autoimmune disease, most commonly Sj?gren syndrome and systemic lupus erythematosus or a related profile of non-organ-specific autoantibodies. Neurologic involvement occurs in approximately 20% of patients with pSS, usually preceding the diagnosis (in 75-80% of the cases) [1,2]. The frequency of both neurologic manifestations (revealing pSS) and negative autoimmune serology, especially in the event of CNS involvement, could explain why underlying pSS is misdiagnosed [3,4]. Screening for pSS should be systematically performed in cases of acute or chronic myelopathy and/or cranial nerve involvement, mainly because these patients have a severe outcome. The presence of the anti-aquaporin4 antibodies, besides anti-Ro and anti-La, in both reported cases, is intriguing and raises the question of whether we are facing two distinct diseases or the NMO is just complicating an unusually less expressive Sj?gren's syndrome subtype.     

CD5+ B lymphocytes and other lymphocyte subsets in primary Sj?gren's syndrome.

CD5+ B cells and other lymphocyte subsets were analyzed by flow cytometry in patients with primary Sj?gren's syndrome (pSS), in healthy subjects (HS) and in patients with various control diseases. When compared with HS, patients with pSS were found to have similar levels of CD5+ B cells and decreased levels of CD8+ T cells (P = 0.0003). When compared with patients with various other diseases, however, the number of CD5+ B cells in pSS was more than twice as high (P = 0.0002), whereas no difference was found between numbers of CD8+ T cells. When the number of CD5+ B cells was expressed as a percentage of total B cells, the results obtained were similar to those with absolute numbers. Determination of lymphocyte subsets may be used as a diagnostic aid for Sj?gren's syndrome in selected patients with suspected immunological diseases of unknown type.     

Tolerance and efficacy of rituximab and changes in serum B cell biomarkers in patients with systemic complications of primary Sjögren's syndrome

OBJECTIVE: To investigate the safety and efficacy of rituximab (RTX) for systemic symptoms in patients with primary Sj?gren's syndrome (pSS), and changes in B cell biomarkers. PATIENTS AND METHODS: The records of 16 patients with pSS according to the American European consensus group criteria were reviewed retrospectively. RESULTS: Patients, all women, had a median age of 58.5 (range 41-71) years and a disease duration of 9.5 (range 0-25) years. RTX was prescribed for lymphoma (n = 5), refractory pulmonary disease with polysynovitis (n = 2), severe polysynovitis (n = 2), mixed cryoglobulinaemia (n = 5), thrombocytopenia (n = 1) and mononeuritis multiplex (n = 1). The median follow-up duration was 14.5 (range 2-48) months. Three patients experienced adverse events, including one mild serum sickness-like reaction with the presence of human antichimeric antibodies. Efficacy of treatment was observed in 4 of 5 patients with lymphomas and in 9 of 11 patients with systemic involvement. Dryness was improved in only a minority of patients. Corticosteroid dose was reduced in 11 patients. RTX induced decreased rheumatoid factor, gamma-globulin and beta2-microglobulin levels, and the level of B cell activating factor of the tumour necrosis factor family (BAFF) increased concomitantly with B cell depletion. Five patients were re-treated, with good efficacy and tolerance, except for one with probable serum sickness-like reaction. CONCLUSION: This study shows good efficacy and fair tolerance of RTX for systemic features. In addition, RTX allows for a marked reduction in corticosteroid use. Except for BAFF, the level of which increases, serum B cell biomarker levels decrease after taking RTX. Controlled trials should be performed to confirm the efficacy of RTX in pSS.     

抗着丝点抗体阳性原发性干燥综合征的临床特点

目的 通过分析抗着丝点抗体(ACA)阳性原发性干燥综合征(pSS)患者的临床特点,提高对本病的诊治水平.方法 回顾性分析北京协和医院风湿免疫科收治的60例ACA阳性pSS患者(ACA阳性组)的临床资料,并与同期收治的139例ACA阴性pSS者(ACA阴性组)相比较.结果 (1)ACA阳性组发病年龄晚于ACA阴性组[(48±11)岁比(41±12)岁,P=0.000],而2组在性别构成、口干、眼干、唇腺活检阳性率差异元统计学意义.(2)肝脏受累是ACA阳性组患者最常见的腺外脏器损害,其发生率远高于ACA阴性组(68.3%比37.0%,P=0.000),而肾脏受累(13.3%比30.9%,P=0.009)、神经系统受累(1.7%比11.5%,P=0.025)、高γ球蛋白血症的发生率(20.8%比45.7%,P=0.002)均低于ACA阴性组.(3)2组抗核抗体阳性率无差异,但ACA阳性组以散点型免疫荧光表型最为常见(占61.7%).与ACA阴性组相比,ACA阳性组中抗SSA抗体、抗SSB抗体、抗U1 RNP抗体的阳性率减低(P＜0.05),而抗线粒体抗体及其M2亚型的阳性率增高(P＜0.05).(4)ACA阳性组死亡5例,其中3例死于胃食管静脉曲张破裂出血.结论 对ACA阳性pSS者应警惕肝脏受累.由于ACA阳性pSS在发病年龄、腺外器官受累、免疫指标、自身抗体谱及预后方面均不同于经典pSS,故可能是pSS的一种独特亚型.     

A clinical analysis of primary Sjögren's syndrome with anticentromere antibodies

OBJECTIVE: To investigate the clinical manifestations, immunological features and prognosis of primary Sj?gren's syndrome (pSS) with anticentromere antibodies (ACA). METHODS: Sixty pSS patients with ACA in our hospital between 1985 and 2006 were screened retrospectively and compared with those without ACA. RESULTS: The mean age at the onset of pSS with ACA was higher than that of those without ACA [(48 +/- 11) yr vs (41 +/- 12) yr, P =0.000]. There was no difference in sex ratio, dry mouth, dry eyes and positive salivary gland biopsy between the two groups (P > 0.05). Compared with those without ACA, patients with ACA presented a higher prevalence of liver involvement (68.3% vs 37.0%, P = 0.000), while a lower prevalence of renal involvement (13.3% vs 30.9%, P = 0.009), neuropathy (1.7% vs 11.5%, P = 0.025) and hypergammaglobulinemia (20.8% vs 45.7%, P = 0.002). The difference was not significant between the two groups in Raynaud's phenomenon, articular involvement, myositis, hematologic involvement, lung involvement, and thyroiditis. While both groups showed the same prevalence of antinuclear antibody (ANA), the patterns of ANA-IF were different and the discrete speckled pattern was the most frequent in patients with ACA and occurred in 61.7%. Different from those without ACA, patients with ACA presented a lower prevalence of anti-SSA, anti-SSB, rheumatoid factor, and anti-U1RNP, while showed a higher prevalence of antimitochondrial antibodies (AMA) and AMA-M2. The most frequent cause of death was the complications associated with cirrhosis, notably bleeding varices (3/5 cases). CONCLUSION: Patients with ACA present a high risk of liver involvement. Because of the remarkable difference in the mean age of disease onset and also differences in systemic damage, immunological and antibody features, pSS with ACA may be a special subtype of pSS.     

Psychological well-being in patients with primary Sjögren's syndrome

OBJECTIVES: To evaluate quality of life and psychological symptoms in patients with primary Sj?gren's syndrome and to compare this with patients with rheumatoid arthritis. METHODS: A standardised questionnaire, the Psychological General Well-Being Index (PGWB), was used to examine the quality of life and psychological symptoms in patients with primary Sj?gren's syndrome (pSS; n = 34). Patients with rheumatoid arthritis (RA; n = 32) were used as patient controls. RESULTS: The total mean score +/- SD for PGWB was 84.9 +/- 16.2 in pSS patients and significantly lower (p = 0.001) than in RA patients (97.7 +/- 17.5). Patients with pSS had an increased propensity for depressed mood (p = 0.0009), and suffered from reduced well-being (p = 0.002) and impaired vitality (p = 0.003). CONCLUSION: The results suggest that patients with pSS have a reduced quality of life, a higher degree of distress and a lower sense of well-being than patients with RA.     

Sjogren's syndrome is associated with and not secondary to systemic sclerosis

OBJECTIVES: When Sj?gren's syndrome (SS) is secondary to rheumatoid arthritis, the sicca syndrome is less serious and anti-SSA/SSB antibodies are found less frequently than in primary SS (pSS). When SS is associated with systemic lupus erythematosus, clinical and serological patterns are similar to those of pSS. We aimed to determine whether SS, accompanying systemic sclerosis (SSc), could be considered secondary to or associated with SSc and whether the coexistence of both modifies the severity and the outcome of each disease. Patients and METHODS: A retrospective multicentric study was conducted to compare (i) characteristics and complications of SS between 27 patients with SS and SSc (SS-SSc) and 202 patients with pSS, and (ii) the characteristics of SSc and complications between the SS-SSc group and 94 patients with SSc alone. RESULTS: SS features were similar in both SS-SSc and pSS patients, except for peripheral neuropathy and arthritis, which was more common in SS-SSc than in the pSS patients (P = 0.02 and 0.05, respectively). SSc appears to be less severe in patients with SS-SSc than SSc alone with a lower frequency of lung fibrosis (P = 0.05). Compared with patients with pSS or SSc alone, SS-SSc patients were more likely to have another autoimmune disorder and other autoantibodies (SS-SSc vs pSS, P = 0.02 and P = 0.03, respectively). CONCLUSION: SS seems to be associated with and not secondary to SSc. SS associated with SSc has the same features as pSS, but SSc seems to be less serious. Moreover, the association of SS and SSc is frequently accompanied by a spreading of autoimmunity.     

The efficacy and safety of total glucosides of peony in the treatment of primary Sjögren’s syndrome: a multi-center,randomized, double-blinded,placebo-controlled clinical trial

To evaluate the efficacy and safety of total glucosides of peony (TGP) in adults with primary Sjögren’s syndrome (pSS). A multi-center, randomized, double-blinded, placebo-controlled study was conducted between March 2012 and July 2014 at ten Chinese hospitals. In total, 320 pSS patients—classified according to the 2002 American-European Consensus Group Criteria—were randomized (2:1 ratio) to receive TGP(600 mg, tid) in the TGP group or placebo for 24 weeks in the placebo group. Study personnel, investigators, and patients were blinded to the treatment grouping. The primary endpoint was the improvement of EULAR Sjögren’s Syndrome Patient Reported Index (ESSPRI) at week 24. The secondary endpoints were dry eyes/mouth/skin/nose/throat/vagina visual analogue scale (VAS), pain and discomfort VAS, fatigue VAS, mental discomfort VAS, patient global assessment (PGA), EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI), Schirmer’s test, basal/stimulated salivary flow-rate values, and erythrocyte sedimentation rate (ESR). All adverse events were recorded during the trial period. ESSPRI improved more in the TGP than the placebo group (p < 0.001). Dry eyes/throat/vagina VAS, fatigue VAS, mental discomfort VAS, PGA, Schirmer’s test, and ESR also improved more in the TGP group than in the placebo group (all p < 0.05). Stimulated salivary flow-rate values increased in the TGP group at week 12 but not at week 24. Adverse events in TGP group were 10.9%. TGP can alleviate some dryness symptoms as well as disease activity in pSS patients over 24 weeks. TGP was well tolerated by study subjects. TGP seems to be an effective and safe treatment for pSS.