Pathophysiology of Sjögren's syndrome

The term Sj?gren's syndrome refers to keratoconjunctivitis sicca and xerostomia due to lymphocytic infiltrates of lachrymal and salivary glands. The current used criteria for diagnosis of primary Sj?gren's syndrome is the American-European consensus. Primary Sj?gren's syndrome is an autoimmune disorder characterized by lymphocytic infiltrates and destruction of the salivary and lachrymal glands and systemic production of autoantibodies to the ribonucleoprotein particles SS-A/Ro and SS-B/La. The infiltrating cells (T- and B-cells, dendritic cells) interfere with glandular function at several points: destruction of glandular elements by cell-mediated mechanisms; secretion of cytokines that activate pathways bearing the signature of type 1 and 2 interferons; production of autoantibodies that interfere with muscarinic receptors; and secretion of metalloproteinases (MMPs) that interfere with the interaction of the glandular cell with its extracellular matrix, which is necessary for efficient glandular function. As the process progresses, the mucosal surfaces become sites of chronic inflammation and the start of a vicious circle. Despite extensive study of the underlying cause of Sj?gren's syndrome, the pathogenesis remains obscure. In broad terms, pathogenesis is multifactorial; environmental factors are thought to trigger inflammation in individuals with a genetic predisposition to the disorder.     

Predictive value of SS-B precipitating antibodies in Sjo?gren's syndrome

As part of a screening programme several patients were identified with antibodies to the nuclear antigen SS-B. Fifteen were examined and 11 found to have Sj?gren's syndrome, though this had not been suspected by most of the referring physicians. In contrast, among a group of 17 patients with overt Sj?gren's syndrome, most of whom also had rheumatoid arthritis, only one had antibodies to SS-B. Patients presenting with polyarthralgia found to be SS-B positive may be likely to develop Sj?gren's syndrome but unlikely to develop rheumatoid arthritis. The detection of SS-B antibodies may antedate clinical evidence of Sj?gren's syndrome by months or even years. These results emphasise the clinical heterogeneity of Sj?gren's syndrome.     

干燥综合征与恶性淋巴瘤

为了探索早期诊断干燥综合征患者合并恶性淋巴瘤，作者采用Ｂ细胞恶性淋巴瘤基因探针检测了４５例干燥综合征患者的泪腺组织及患眼附属器Ｂ恶性淋巴瘤组织。结果４例干燥综合征患者泪腺组织阴性，而５例恶性淋巴瘤患者的瘤组织扩增阳性。结果表明，一般的原发或继发性干燥综合征患者泪腺中的淋巴细胞中无恶性Ｂ淋巴瘤基因。     

Clinical manifestations and immunological features of primary Sj?gren's syndrome with liver involvement: analysis of thirty cases.

OBJECTIVE: To evaluate the incidence, severity, clinical manifestations and immunological features relevant to liver involvement in 135 cases of primary Sj?gren's syndrome. METHODS: One hundred and thirty-five patients with definite primary Sj?gren's syndrome were analyzed retrospectively for liver involvement by the abnormalities of the liver enzymes, bilirubin level and liver biopsied section. RESULTS: The liver involvement in 30 patients (22.2%) could be etiologically ascribed to Sj?gren's syndrome itself. The clinical spectrum and severity of this entity differed widely, 36.6% showed no relevant clinical symptoms, however jaundice was found in 46.7% of patients. Six patients showed pathological changes of chronic active hepatitis. 73.3% of all patients with liver involvement responded to steroid and immunosuppressive drugs, yet with a tendency to relapse (two cases). Liver cirrhosis was developed in five cases. The spectrum of serum autoantibodies in the patients with liver involvement showed no difference from those without liver involvement. Most of them were compatible with the serum profile of autoimmune hepatitis type-1. CONCLUSIONS: Liver involvement was complicated in 22.2% patients of primary Sj?gren's syndrome. Clinical manifestations were non-specific and the main pathological change was chronic active hepatitis. The differential diagnosis between Sj?gren's syndrome with liver involvement and type-1 autoimmune hepatitis could be only ascribed to other systemic clinical manifestations of Sj?gren's syndrome.     

唾液β2微球蛋白浓度检测对Sjogren综合征的诊断价值

In this paper we measured salivary beta 2 microglobulin (beta 2 m) concentration in 40 patients with Sj?gren's syndrome, including 20 patients with systemic lupus erythematosus, 12 patients with recurrent aphthous ulceration and 16 patients with single xerostomia, and in 40 normal controls. The results showed that salivary beta 2 m concentration was more significantly elevated in Sj?gren's syndrome patients, compared with that in both normals and other patients. The measurement of beta 2 m concentration in saliva may offer a simple and valuable method for diagnosis of Sj?gren's syndrome.     

舍格伦综合征病因和发病机制及AQP5在其中的作用

舍格伦综合征是一种具有独特特征且临床症状多样的慢性自身免疫性疾病。该病在临床上分为原发和继发两类,从局部外分泌腺病变到系统性免疫疾病、淋巴瘤等均可发生,严重影响患者生活质量。关于该病的病因和发病机制尚未完全明确,有许多问题有待进一步研究。     

干燥综合征毒邪致病研究进展

近年来毒邪致病理论在干燥综合征的认识和治疗中得到越来越多的应用和研究.对干燥综合征毒邪致病学说的中医观点和现代学研究进行总结,探讨了中医毒邪致病理论与现代医学干燥综合征发病机制的联系,以期为干燥综合征的治疗与研究提供新思路、新方法.     

Challenges in Treatment of Primary Sjögren''s Syndrome and Opportunities for Chinese Medicine

Primary Sj?gren's syndrome is a chronic autoimmune disease that can lead to systemic manifestations. At present, immunomodulatory agents have not shown good ef?cacy, many patients in China seek Chinese medicine treatment. Chinese medicine can comprehensively improve the symptoms of patients through Chinese pattern diagnosis and individualized treatment. Fundamental researches are providing scienti?c bases for the therapeutic effect of Chinese medicine. Professional Chinese medicine treatment can be integrated into the conventional management of primary Sj?gren's syndrome.     

以主动脉瓣膜病变为突出表现的干燥综合征1例报道

干燥综合征（Sj-gren’s syndrome,SS）是一种以泪腺和唾液腺慢性炎症为主要特征的系统性自身免疫性疾病,上皮炎症是其最基本的病理改变。在干燥综合征病程中,心脏损害并不多见,而以主动脉瓣狭窄为主要表现的干燥综合征更是罕见。本文报道1例以主动脉瓣狭窄为突出表现的干燥综合征患者。     

干燥综合征患者外周血CD5~+ B细胞、CD5 mRNA变化的研究

目的:了解干燥综合征患者外周血中CD5+B细胞的变化及其B淋巴细胞CD5在基因水平上的表达,探讨CD5+B细胞与干燥综合征的关系。方法:采用免疫荧光双标记技术和流式细胞仪检测了20例干燥综合征患者和10例健康对照者外周血中CD5+B细胞(CD5+CD19+)的百分率。应用流式细胞仪分选B淋巴细胞的基础上,运用Real-time RT-PCR方法检测7例干燥综合征患者外周血CD5 mRNA的表达水平。结果:干燥综合征患者外周血CD5+B(CD5+CD19+)细胞的百分率高于健康对照组(P<0.05)。86%的患者CD5 mRNA高于正常人水平。结论:干燥综合征患者外周血CD5+B细胞及CDS转录水平和干燥综合征的发病有一定的关联。     

Yoghurt biotherapy: contraindicated in immunosuppressed patients?

A fatal case of Lactobacillus rhamnosus septicaemia after prolonged oral vancomycin for recalcitrant Clostridium difficile infection is reported. The patient was immunosuppressed with cyclophosphamide and steroids for Sj?gren's syndrome. The administration of Lactobacillus spp as "biotherapy" may be hazardous in such circumstances.     

Middle lobe syndrome as the pulmonary manifestation of primary Sjögren's syndrome

Middle lobe syndrome - recurrent atelectasis and/or bronchiectasis involving the right middle lobe and/or lingula - has, up to now, not been reported as the pulmonary manifestation of primary Sj?gren's syndrome. We describe a patient in whom lymphocytic bronchiolitis in the atelectatic lobes was proved histologically from two separate transbronchial biopsies. The atelectasis responded well to glucocorticoid treatment, suggesting that the peribronchiolar lymphocytic infiltrates may have played an important role in the development of middle lobe syndrome in this patient.     

原发性干燥综合征合并肌炎患者临床特征分析

分析原发性干燥综合征（pSS）合并肌炎患者的临床特点,并与原发性多发性肌炎（PM）患者比较。结果示202例pSS患者中有4例（2．0％）在诊断pSS后5—20年出现肌炎表现,其中2例无明显肌痛和肌无力症状;肌酸肌酶（CK）最高值为（480—2702）IU／L,中位数为789．5IU／L,抗Jo-抗体均阴性;糖皮质激素治疗1周后CK下降13．9％～71．5％,中位数为48．9％;随访8个月至5年,无一例肌炎病情反复。与15例PM患者资料比较分析,两组在肌痛发生率、CK最高值、激素治疗后CK下降率、抗Jo-1抗体阳性率等方面差异有统计学意义。提示pSS患者合并肌炎较为少见,多在干燥综合征多年之后出现,肌痛和肌无力表现较轻,使用激素效果较好。     

干燥综合征与病毒感染的关系研究进展

干燥综合征是一种以口眼干燥为特征,主要累及外分泌腺的弥漫性结缔组织病。其发病可能与病毒感染、遗传和性激素异常等多种因素有关,在这些因素的共同作用下,机体免疫异常,组织损伤。与该病关系较密切的病毒有EB病毒、丙型肝炎病毒、巨细胞病毒、反转录病毒等。病毒在干燥综合征的发病机制中可能起重要的作用。     

Focus on Effects of Chinese Medicine on Improving Anxiety-Depression and Quality of Life of Patients with Primary Sjögren''s Syndrome

Primary Sj?gren's syndrome(pSS) is a chronic autoimmune disease with a long duration of illness, for which there is no cure. Patients often suffer from anxiety and depression due to various reasons, exhibiting a decline in their quality of life. Chinese medicine(CM) has certain advantages in the treatment of pSS, which not only helps relieve clinical symptoms and improve treatment outcomes, but also reduces anxiety and depression and improves the quality of life. Therefore, CM should be considered as early as possible given its effectiveness and synergistic effects in treating p SS.     

原发性干燥综合征合并感染的临床特点及危险因素分析

目的探讨原发性干燥综合征(primary Sj?gren's syndrome,pSS)合并感染的临床特点及危险因素。方法197例pSS病人根据是否合并感染分为感染组(52例)和非感染组(145例),了解感染组的感染部位和病原体类型,并对感染相关的可疑危险因素,运用二元logistic回归逐步进行单因素和多因素分析。结果本研究中pSS病人感染发生率为26.4%(52/197),最常见的感染部位是呼吸道(52.7%)和泌尿道(21.8%),其余分别是皮肤、软组织(12.7%)、败血症(5.5%)、中枢神经系统(3.6%)、消化道(1.8%)及生殖系统(1.8%)。感染病原体中最常见的是细菌(67.3%),其余依次是病毒(25%)、真菌(7.7%)、支原体(7.7%),混合感染4例。细菌感染中又以大肠埃希菌和肺克雷伯杆菌等革兰阴性杆菌最为常见,且对喹诺酮类或头孢菌素类抗生素的耐药率均较高。感染组的年龄、病程、血清胆固醇、三酰甘油、C反应蛋白、pSS病情活动度评分病人主观症状评分(ESSPRI)和病人客观表现评分(ESSDAI)、干燥程度及疲乏程度、肺脏受累发生率、糖皮质激素的使用率均高于非感染组(P < 0.05~P < 0.01),且感染组的总蛋白及白蛋白含量均低于非感染组(P < 0.01),pSS疾病高度活动组感染率明显高于病情稳定组与轻度活动组(P < 0.05)。logistic单因素回归分析显示年龄大、ESSDAI评分高、ESSPRI评分高、疲乏程度高、干燥程度高、血清总蛋白低、白蛋白低、血清胆固醇高、三酰甘油高、C反应蛋白高、肺脏受累是pSS合并感染的危险因素(P < 0.05~P < 0.01)。多因素回归分析显示ESSDAI评分高、干燥程度高、血清三酰甘油高、肺脏受累是pSS合并感染的独立危险因素(P < 0.05~P < 0.01)。结论pSS病人感染的发病率较高,最常见的感染部位是呼吸道和泌尿道,最常见的感染病原体是大肠埃希菌和克雷伯杆菌等革兰阴性杆菌。ESSDAI评分高、干燥程度高、血清三酰甘油高、肺脏受累是原发性干燥综合征合并感染的独立危险因素,临床工作中应予以重视。     

原发性干燥综合征的临床首发症状分析

目的:了解原发性干燥综合征(pSS)的临床首发症状的特点,提高口腔科医生对此病的认识水平,以减少误诊漏诊。方法:对66例pSS的临床资料进行回顾性分析。结果:口腔首发症状出现频率为:口干47/66例,猛性龋19/66例,口腔溃疡6/66例,腮腺肿大6/66例;眼干38/66例,关节肿痛46/66例,唇腺活检阳性率最高为59/66,ANA、抗SSA、抗SSB、ESR、RF阳性分别为28/66,29/66,26/66,32/66,28/66,高γ-球蛋白血症57/66,IgG、IgA异常率为16/66,10/66。结论:pSS为全身性免疫性疾病,80%以上有高γ-球蛋白血症,口腔表现为口干、猛性龋。口干、眼干、关节肿痛依旧是pSS最常见首诊症状,唇腺活检,腮腺造影及抗核抗体谱对诊断有重要意义。     

Rare association of chronic lymphocytic thyroiditis with dermatomyositis

Autoimmune thyroid disease (AITD) has been associated with other autoimmune diseases such as chronic urticaria, insulin-dependent diabetes mellitus, Sj?gren's syndrome, inflammatory bowel disease, rheumatoid arthritis, multiple sclerosis, systemic lupus erythematosus, psoriasis, ankylosing spondylitis, and dermatomyositis (DM). AITD is a common disorder affecting primarily women, and both genetic and environmental factors are included in its pathogenesis. DM is considered an autoimmune disease of the muscles and skin. Although AITD is the most common cause of hypothyroidism, to the best of our knowledge, only three cases of DM and AITD in the same patient have been reported in the last 40 years. We consider that both are developed from the same autoimmune background. Herein, we present a case of a 30-year-old man with a 4-year history of AITD who was diagnosed as suffering as well from DM.     

中医治疗干燥综合征的研究进展

中医药治疗干燥综合征,从病因病机、辨证论治、专方治疗、中药复方治疗、针灸治疗、实验研究等方面均取得了较大进展。干燥综合征多以阴虚津亏为本,燥热瘀毒为标。中医辨证施治可调节机体免疫力及各脏腑间的功能,中医针灸治疗取穴方便、灵活,且无不良反应,能显著改善干燥综合征的临床症状。     

Mucosal administration of alpha-fodrin inhibits experimental Sjögren's syndrome autoimmunity

OBJECTIVE: To explore the effect of mucosal administration of alpha-fodrin in inhibition of autoimmunity in Sj?gren's syndrome (SS). METHODS: Thirty-four 4-week-old NOD mice were randomly divided into 4 equal groups: to be immunized by nasal administration of alpha-fodrin 1 microg/dose and 10 microg/dose respectively every two days (experimental groups), and phophate-buffered saline (PBS) or glutathion2 S-tansferase4 (GST) (control groups). The weekly volume of water drinking was calculated. The salivary flow was maintained. Serum samples were obtained to detect the anti-SSA, anti-SSB, RF, ANA, anti- -fodrin and anti-type 3 muscarinic acetylcholie receptor polypeptide (M3RP) by immunofluorescence or ELISA. The cytokines of IFN-gamma and IL-10 were measured with ELISA. The salivary glands were examined by HE staining and immunohistochemical analysis. Flow cytometry was used to detect the proportion of Foxp3+ CD4+ CD25+ T cells. RESULTS: The titers of anti- -fodrin antibody and M3RP antibody of the mice immunized with-fodrin were lower than those of the 2 control groups (all P < 0.05), however, there was not significant differences between these two a-fodrin immunized groups. Five of the 8 mice in the GST group, 5 mice in the PBS group, 2 mice in the alpha-fodrin 1 microg/dose group, and 3 mice in the alpha-fodrin 10 microg/dose showed ANA positive. The serum IFN-gamma levels in the mice of alpha-fodrin 1 microg/dose and 10 microg/dose groups, PBS group, and GST group were (42 +/- 16), (37 +/- 15), (87 +/- 18), and (72 +/- 11) pg/ml respectively, those of the fodrin groups being significantly lower than those of the control groups (all P < 0.05). There were not significant differences in the level of serum IL-10 among these four groups. The numbers of Foxp3+ CD4 CD25+ regulatory T cells were higher in the fodrin groups than in the PBS and GST control groups (all P < 0.05). The lymphocytic infiltration and expression of alpha-fodrin were decreased in the alpha-fodrin administrated groups. The volume of water drinking of the alpha-fodrin 1 microg/dose group, alpha-fodrin 10 microg/dose group, PBS group, and GST group were (39.2 +/- 2.1), (40.4 +/- 2.5), (49.3 +/- 3.1), and (51.6 +/- 2.8) ml respectively. CONCLUSION: Mucosal administration of alpha-fodrin effectively inhibits the progression of experimental Sj?gren's syndrome autoimmunity.