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Recent developments in the diagnosis and management of Sjogrens syndrome

机译:Sjogrens综合征的诊断和治疗的最新进展

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Sjogren's syndrome (SS) is a common immune disease that mainly affects the exocrine glands, which clinically present xerostomia, keratoconjuctivitis sicca and serological autoantibodies including rheumatoid factor, antinuclear antibody, anti-SS-A and anti-SS-B. The prevalence of SS is high but underestimated in the general population. SS may involve many other organs and tissues including lung (interstitial lung disease), kidney (renal tubular acidosis and hypokalemia), nerve (peripheral neuropathy), vessel (vasculitis, Raynauld's phenomenon), bladder (interstitial cystitis), lymph node (lymphaenopathy), liver (autoimmune hepatitis), pancreas (pancreatitis) and Gl (reflux esophagitis, peptic ulcer). In 2010, a new disease activity, the European League Against Rheumatism (EULAR) Sjogren Syndrome Disease Activity Index (ESSDAI) was proposed. Diagnosis of SS is based upon the 2002 classification criteria of SS, which originated from the 1996 European classification criteria. Methods for diagnosis of SS include salivary flow assessment, salivary gland scintigraphy, Schimer's test. Rose Bengal test and minor salivary gland biopsy. Treatment of dry mouth and dry eye requires muscarinic agonists, for which two drugs are now available; pilocarpine and cevimeline. Hydroxychloroquine is not useful for sicca syndrome, however, it may be effective to relieve arthralgia. Active immunotherapy is considered when the patient has pulmonary, neurologic and renal involvement. Corticosteroid, cyclophosphamide, azathioprine, mycophenolate mofetal (cellcept), mizoribine and cydosporine are the immunosuppressive drugs that have been used for SS with systemic manifestations. TNF-alpha blocker has been used in SS but, in general, it demonstrated no benefit to relieve oral or eye dryness. More recently, rituximab, a B cell directed therapy, was reported to have efficacy for sicca symptoms or pulmonary, neurologic involvement. In this article, we demonstrate current diagnosis and therapy f...
机译:干燥综合征(SS)是一种常见的免疫疾病,主要影响外分泌腺,临床上表现为口干症,干燥性角膜结膜炎和血清学自身抗体,包括类风湿因子,抗核抗体,抗SS-A和抗SS-B。 SS的患病率很高,但在普通人群中被低估了。 SS可能涉及许多其他器官和组织,包括肺(间质性肺病),肾(肾小管酸中毒和低血钾),神经(周围神经病变),血管(血管炎,雷纳德现象),膀胱(间质性膀胱炎),淋巴结(淋巴结病) ,肝脏(自身免疫性肝炎),胰腺(胰腺炎)和Gl(反流性食管炎,消化性溃疡)。 2010年,提出了一项新的疾病活动,即欧洲抗风湿病联盟(EULAR)干燥综合征综合症活动指数(ESSDAI)。 SS的诊断基于2002年的SS分类标准,该分类标准源自1996年的欧洲分类标准。 SS的诊断方法包括唾液流量评估,唾液腺闪烁显像,Schimer检验。孟加拉玫瑰试验和小唾液腺活检。干口和干眼的治疗需要毒蕈碱激动剂,目前有两种药物可供使用。毛果芸香碱和西维美林。羟氯喹不能用于干燥综合征,但是,它可能对缓解关节痛有效。当患者有肺,神经和肾脏受累时,可考虑采用主动免疫疗法。皮质类固醇,环磷酰胺,硫唑嘌呤,霉酚酸酯(细胞受体),咪唑啉碱和环孢菌素是已被用于具有全身性表现的SS的免疫抑制药物。 TNF-α阻滞剂已被用于SS,但总的来说,它对减轻口腔或眼干无益处。最近,据报道利妥昔单抗是一种B细胞定向疗法,对干燥症状或肺,神经系统受累具有疗效。在本文中,我们演示了当前的诊断和治疗方法。

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