The therapeutic management of Sjögren syndrome (SjS) has not changed substantially in recent decades: treatment decisions remain challenging in clinical practice, without a specific therapeutic target beyond the relief of symptoms as the most important goal. In view of this scenario, the European League Against Rheumatism (EULAR) promoted and supported an international collaborative study (EULAR SS Task Force) aimed at developing the first EULAR evidence and consensus-based recommendations for the management of patients with SjS with topical and systemic medications. The aim was to develop a rational therapeutic approach to SjS patients useful for healthcare professionals, physicians undergoing specialist training, medical students, the pharmaceutical industry and drug regulatory organisations following the 2014 EULAR standardised operating procedures. The Task Force (TF) included specialists in rheumatology, internal medicine, oral health, ophthalmology, gynaecology, dermatology and epidemiology, statisticians, general practitioners, nurses and patient representatives from 30 countries of the 5 continents. Evidence was collected from studies including primary SjS patients fulfilling the 2002/2016 criteria; when no evidence was available, evidence from studies including associated SjS or patients fulfilling previous sets of criteria was considered and extrapolated. The TF endorsed the presentation of general principles for the management of patients with SjS as three overarching, general consensus-based recommendations and 12 specific recommendations that form a logical sequence, starting with the management of the central triplet of symptoms (dryness, fatigue and pain) followed by the management of systemic disease. The recommendations address the use of topical oral (saliva substitutes) and ocular (artificial tear drops, topical non-steroidal anti-inflammatory drugs, topical corticosteroids, topical CyA, serum tear drops) therapies, oral muscarinic agonists (pilocarpine, cevimeline), hydroxychloroquine, oral glucocorticoids, synthetic immunosuppressive agents (cyclophosphamide, azathioprine, methotrexate, leflunomide and mycophenolate), and biological therapies (rituximab, abatacept and belimumab). For each recommendation, levels of evidence (mostly modest) and TF agreement (mostly very high) are provided. The 2019 EULAR recommendations are based on the evidence collected in the last 16 years in the management of primary 2002 SjS patients and on discussions between a large and broadly international TF. The recommendations synthesise current thinking on SjS treatment in a set of overarching principles and recommendations. We hope that the current recommendations will be broadly applied in clinical practice and/or serve as a template for national societies to develop local recommendations.

中文翻译：

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EULAR 建议通过局部和全身治疗来管理干燥综合征

近几十年来，干燥综合征 (SjS) 的治疗管理并未发生重大变化：治疗决策在临床实践中仍然具有挑战性，除了缓解症状之外，没有特定的治疗目标作为最重要的目标。鉴于这种情况，欧洲抗风湿病联盟 (EULAR) 推动并支持了一项国际合作研究 (EULAR SS Task Force)，旨在为局部和全身性 SjS 患者的管理制定首个 EULAR 证据和基于共识的建议药物。目的是为 SjS 患者开发一种合理的治疗方法，对医疗保健专业人员、接受专科培训的医生、医学生、遵循 2014 EULAR 标准化操作程序的制药行业和药品监管机构。工作组 (TF) 包括来自五大洲 30 个国家的风湿病学、内科、口腔健康、眼科、妇科、皮肤科和流行病学专家、统计学家、全科医生、护士和患者代表。从包括满足 2002/2016 年标准的原发性 SjS 患者在内的研究中收集证据；当没有可用的证据时，来自研究的证据，包括相关的 SjS 或符合先前标准的患者被考虑和推断。TF 赞同将 SjS 患者管理的一般原则表述为三个总体原则，基于共识的一般建议和形成逻辑顺序的 12 条具体建议，首先是对中枢三重症状（干燥、疲劳和疼痛）的管理，然后是全身性疾病的管理。这些建议涉及使用局部口服（唾液替代品）和眼部（人工泪液、局部非甾体抗炎药、局部皮质类固醇、局部 CyA、血清泪液）疗法、口服毒蕈碱激动剂（毛果芸香碱、西维美林）、羟氯喹、口服糖皮质激素、合成免疫抑制剂（环磷酰胺、硫唑嘌呤、甲氨蝶呤、来氟米特和霉酚酸酯）和生物疗法（利妥昔单抗、阿巴西普和贝利单抗）。对于每项建议，都提供了证据级别（大部分为适中）和 TF 一致性（大部分为非常高）。2019 年 EULAR 建议基于过去 16 年在 2002 年 SjS 原发性患者管理中收集的证据以及大型和广泛国际 TF 之间的讨论。这些建议在一套总体原则和建议中综合了当前对 SjS 治疗的思考。我们希望当前的建议将广泛应用于临床实践和/或作为国家学会制定地方建议的模板。