Sj(o)gren's综合征的口腔表现

Sj(o)gren's综合征(简称SS)是一种以口腔干燥症、干燥性角、结膜炎和胶原病为特征的慢性全身性自身免疫性疾病[1].原发患者有干燥症状,伴发于胶原病者称为继发性SS.胶原病多为类风湿性关节炎、系统性红斑狼疮、硬皮病、多发性结节性动脉炎等,还可有其他外分泌腺的表现,如甲状腺炎、间质性肾炎等[2].随着现代生物技术的发展,其病因、临床病理、检诊、治疗的研究已取得了可喜的进展,现就其口腔表现作一综述.     

Sjgren综合征的心血管表现一例

患者男,65岁。因5天来心悸、胸闷伴气促、纳呆、尿少、恶心间有呕吐入院。前月余来腹泻频数,有脓血便伴里急后重,近5日每日稀便1～2次。既往有慢性咳嗽、咯痰、气促10余年,加重20天,咯白粘痰不易吐出。长期来泪少,双眼干燥,1年前患“点状角膜炎”并“虹膜睫状体炎”,右眼遗留角膜白斑。平时唾液少,口干,涎痰粘稠不易吐出,语音低哑,并有双膝、踝、趾关节僵痛史。否认“心脏病”及心悸、胸闷等病症史。体查:体温37.2℃、脉搏66次、呼吸20次、血压测不到。发育可、一般差、神清。皮肤弹性差,无黄疸及皮疹。浅淋巴结不大,毛发正常,巩膜不黄、双瞳等反射可,右眼角膜     

涎腺Sjgren's综合征的灰阶和彩色超声诊断

本文应用灰阶超声和彩色多普勒超声对１１例Ｓｊｇｒｅｎｓ综合征作了研究，所有病例均经临床和病理证实。结果显示：弥漫型Ｓｊｇｒｅｎｓ综合征的灰阶图表现为双侧腺体内弥漫性多个低回声区；彩色血流强度在Ⅰ～Ⅱ级，表现为在整个的腺体内出现随机分布的点状血流信号。结节型的灰阶图表现为腺体内椭圆形低回声区，境界清晰；彩色血流的强度为Ⅱ级，分布形式为内部分支型。本文结果表明：超声表现虽无特异性，但紧密结合临床，仍有助于Ｓｊｇｒｅｎｓ综合征诊断     

Sjgren综合征的诊断研究进展

1933年瑞典的眼科医生Herrik Sjogren首先报道了原因不明的干燥性角膜炎、口干、涎腺肿大和多发性关节炎为主征的病例19例。此后人们把这一征候群称为Sjogren综合征(简称SS)。它是一种慢性炎症性自身免疫性疾病。目前SS在临床上比较多见。其诊断手段越来越多,现综述如下。     

Sjögren's syndrome

Sj?gren's syndrome(SS) is a chronic inflammatory disease characterized by keratoconjunctivitis sicca and xerostomia. According to the epidemiological survey done by the Japan Ministry of Health and Welfare(MHW) in 1994, about 17,000 SS patients visit hospitals and the prevalence rate is approximately 0.06% for females. SS is diagnosed mainly by the criteria of MHW in 1977, and the revised ones will be published in the near future. Several candidates for the corresponding antigens for SS autoimmunity have discovered, such as alpha-fodrin and 203-211 amino acid residues of Ro/SSA-52 kDa. The sicca complex is treated with fluid replacement, but corticosteroids or immunosuppressive drugs are sometimes indicated in the treatment of extraglandular involvement. Several new drugs are now under investigation.     

原发性进行性多发性硬化合并Sjgren综合征

多发性硬化和Sj gren综合征之间的关系是有争论的。为了弄清原发性进行性多发性硬化 (pri mary progressivemultiplesclerosis ,PPMS )和(Sj grensyndrome ,SS)之间的关系 ,我们对 4 0例PPMS患者伴发SS的情况用修订的欧洲SS标准进行了评价。现将结果报告如下。1　资料与方法1.1　一般资料　本组PPMS患者男性 2 0例 ,女性2 0例 ,年龄 19～ 4 9岁 ,平均年龄 4 4.4岁。病程 10个月～ 10年 ,平均 3.6年。 4 0例PPMS患者的诊断 ,采用Thompson的最新建议标准[1] ,77.5 %的患者确诊PPMS ,2 0 %的患者为可能PPMS ,2 .5 %患者为可疑PP…     

Primary Sjögren's syndrome

This article aims to raise awareness of the chronic autoimmune rheumatological disorder Sj?gren's syndrome. It describes the main symptoms, diagnosis and management of patients with primary Sj?gren's syndrome. It is applicable to nurses working in primary and secondary care settings and offers advice on how they can support such patients with protective and preventive measures.     

类肿瘤型Sjgren综合征误诊四例

类肿瘤型Sj gren综合征 (Sj grenSyndrome ,SS)较为少见 ,在临床上易误诊为腮腺肿瘤 ,尤其是症状不明显者。 1996 2 0 0 2年我科收治的 4例术前均误诊为腮腺肿瘤 ,因此也未能按SS进行手术处理 ,结果 2例术后 1年复发。为吸取教训 ,现报告如下。1　病例资料【例 1】　女 ,72岁。因双侧腮腺区肿物 1年入院 ,病程中无口干、眼干、关节疼痛症状。查体 :右侧腮腺直径约 3cm大小肿物 ,外突明显 ;左侧腮腺直径约 2cm肿物。肿物质地较硬 ,边界清楚。入院诊断为腮腺区良性肿物 ,局麻下行腮腺肿物及浅叶切除术并保留双侧面神经 ,术中行冷冻切片病理…     

NF-κB在Sjgren综合征中的表达及意义

目的:观察核转录因子κB(NF-κB)在SjOgren综合征(SS)涎腺组织中的表达,探讨NF-κB在SS发病过程中的调控作用。方法:采用免疫组化法检测20例SS患者和8例正常唇腺组织中NF-κB的表达情况。结果:(1)SS组唇腺组织中 NF-κB阳性表达水平显著高于对照组,有显著差异(P<0．05)。(2)SS组唇腺组织中NF-κB表达水平与浸润的淋巴细胞灶数／4 mm2呈正相关。结论:NF-κB参与调控SS的发病过程。     

Sjögren's syndrome--implications for perioperative practice

Sj?gren's syndrome (SS) is a chronic autoimmune rheumatic disorder in which lymphocytes invade the exocrine glands, leading to destruction of the glandular tissue. Systemic inflammation also may occur and involve multiple organs. Ninety percent of patients with SS are female, Caucasian, and middle-aged. The characteristic symptoms of xerostomia and xerophthalmia often are trivialized, and the disorder is significantly underdiagnosed. Early diagnosis and management can prevent devastating oral and ocular complications and internal organ damage. Treatment modalities are aimed at providing comfort and preventing complications and progression of the disease. In the perioperative practice setting, nurses play an important role in the health care team involved in providing surgical care to patients with SS.     

Gene therapeutics in Sjögren's syndrome

Sj?gren's syndrome (SS) is a complex autoimmune disorder, characterised by mononuclear cell infiltration of exocrine glands, principally the lacrimal and salivary glands. Both cellular, in the form of autoreactive immune cells, and humoral factors, such as autoantibodies, contribute to the expression of the disease. SS can also occur as a systemic disease affecting several organs, and approximately 5% of the patients develop malignant lymphoproliferation. Today SS is considered uncurable. The treatment available is only palliative, and the treatment goals are to manage symptoms and prevent or limit tissue damage. This may involve both local and systemic measures. However, the existing systemic treatment of chronic inflammatory autoimmune diseases has several limitations and unwanted side effects. In recent years the possibility to treat diseases with gene therapy has gained interest and has become a subject of investigation. Given the multitude of factors contributing to the pathogenesis of SS, gene therapy is a major challenge, but may elicit great benefits if successful. Keeping this in mind, the possibility for gene therapeutics in SS in general and potential targets for gene therapy are discussed.     

Sjoegren‘s综合征的口腔表现

Sj(o)gren's综合征(简称SS)是一种以口腔干燥症、干燥性角、结膜炎和胶原病为特征的慢性全身性自身免疫性疾病[1].原发患者有干燥症状,伴发于胶原病者称为继发性SS.胶原病多为类风湿性关节炎、系统性红斑狼疮、硬皮病、多发性结节性动脉炎等,还可有其他外分泌腺的表现,如甲状腺炎、间质性肾炎等[2].随着现代生物技术的发展,其病因、临床病理、检诊、治疗的研究已取得了可喜的进展,现就其口腔表现作一综述.     

干燥综合征(Sjgren’s syndrome)口干治疗进展

早在1933年HenrickSj gren对本病就有报道[1] 。故命名为Sj grensyndrome ,简称SS ,分为原发性干燥综合征(Pss)和继发性干燥综合征(Sss)。原发性干燥综合征主要是腺泡间局部管周围单核细胞浸润和腺泡萎缩,使唾液流速率和唾液成分发生改变[2 ] 的全身性自身免疫性疾病。继发性主要来自于自身免疫性疾病,如风湿性关节炎等。本病目前主要是对症治疗,控制和延缓因免疫反应而引起的组织器官损害的进展以及继发性感染。1　全身治疗1 1　中医治疗　本症在祖国医学中属燥证范畴[3] 。其特点是津枯内燥,阴血不足。采用活血生津药来改善口干症状,如…     

Renal involvement in primary Sjögren's syndrome

Renal involvement was evaluated in 62 patients with primary Sj?gren's syndrome, classified according to criteria proposed by The European Classification Criteria Group. Urine concentration capacity was tested using intranasal 1-desamino-8-D-arginine-vasopressin. For patients with urine pH>5.5 without metabolic acidosis (n=28), an acidification test with ammonium chloride was performed. Urinary citrate, albumin, NAG, ALP and beta2-microglobulin were measured and creatinine clearance was calculated. Maximum urine concentration capacity and creatinine clearance were reduced in 13 (21%). Albumin excretion was >30 microg/min in only one patient (1.6%). Seven patients (11.3%) had complete or incomplete distal renal tubular acidosis (dRTA), four had reduced creatinine clearance and five had reduced maximum urine concentration capacity. The ratio of citrate/creatinine in spot urine was below the 2.5 percentile in all patients with complete or incomplete dRTA. The prevalence of dRTA was lower than in previous studies. There were also few patients with signs of glomerular disease (1.6%). The use of citrate:creatinine ratio in spot urine can be a helpful method in identifying patients with complete or incomplete dRTA.     

Biological therapies in primary Sjögren's syndrome

INTRODUCTION: Primary Sj?gren's syndrome (PSS) is a relatively common immune-mediated condition characterized by oral and ocular dryness, fatigue, musculoskeletal pain and poor health-related quality of life. Other extra-glandular organs can also be affected and PSS is associated with a markedly increased risk of lymphoma. Furthermore, the health-economic cost for PSS is substantial. There is currently no effective treatment available. With better understanding of the pathophysiology of PSS and advances in technologies, it is now possible to develop biological therapies to target specific molecules or molecular pathways that are important in PSS pathogenesis. Indeed, a limited number of biological therapies have already been tested in PSS with mixed successes. AREAS COVERED: Published data on the use of biological therapies in PSS, the possible roles for other biological therapies and the potential challenges for their use. EXPERT OPINION: The use of biological agents targeting key cellular and molecular pathways in PSS pathogenesis represents a promising therapeutic strategy. Clinical trials assessing the efficacy of biological therapies in PSS should be encouraged but patient selection and outcome measures used in these studies must be carefully considered to ensure that the true effects of biological therapies on the outcomes of PSS are being appropriately evaluated.     

Stomatological manifestations in Sj?gren's disease and syndrome

For diagnosis of SS and SD and the detection of early stages of disease one should necessarily take into account the symptom complex of "major" (salivary gland enlargement, xerostomia, exacerbation of parotitis) and "minor" stomatological signs (multiple cervical caries, dry lips, perlèche, mycotic and herpetic stomatitis, lymphadenopathy). The initial, marked and late stages were defined according to a degree of expression of stomatological manifestations. The initial stage prevailed in SS, the late stage in SD. The chief method of examination were sialometry, sialography and minor salivary gland biopsy. Sialography was widely used as a less traumatic diagnostic procedure. In addition to common signs with SD, salivary gland involvement was characterized by changes typical of SS combined with rheumatic disease (sclerosis in sclerodermia, vasculitis in RA and SLE, nuclear pathology in SLE).     

State of microcirculation and the system of hemostasis in Sj?gren's disease, Sj?gren's syndrome and chronic parenchymatous parotitis

Microcirculation and hemocoagulation were examined in 88 patients with Sj?gren's disease and Sj?gren's syndrome (SD and SS) in 20 patients with chronic parenchymatous parotitis (CPP) without a "dry syndrome", and in 20 healthy donors by conjunctival angioscopy, capillaroscopy of the nail matrix, and histological study of the salivary glands. Hemostasis was explored with the use of 37 tests. SD and SS patients manifested significantly more pronounced microcirculatory disorders versus donors (P less than 0.001) and CPP patients (P less than 0.001). In patients with hypergammaglobulinemic purpura, microcirculatory disorders were found to be enhanced (P less than 0.05). An indirect relationship between these disorders and the degree of lacrimation lowering was established. Intravascular disturbances increased as the process activity rose and diminished under the effect of prednisolone therapy. It is assumed that microcirculatory and hemocoagulation disorders play an important role in the pathogenesis of SD and SS.