干燥综合征识别与治疗：口干眼干背后的自身免疫

先看懂四件事：干燥综合征是什么、怎么识别、怎么检查、怎么治疗Sjögren Syndrome

干燥综合征的治疗要分清两条线：一条是针对口干、眼干等腺体症状的对症治疗，有随机试验证据；另一条是针对系统受累的免疫抑制治疗，证据总体偏弱，需要按器官受累程度个体化决定。同时，长期随访要把淋巴瘤风险与妊娠风险一起纳入。

发病机理：腺体炎症、自身抗体与淋巴增殖风险Pathogenesis

干燥综合征的起点是外分泌腺的淋巴细胞浸润：腺体分泌减少带来口干眼干，抗体与免疫球蛋白异常提示 B 细胞活跃，而长期活跃的 B 细胞反应又与淋巴增殖风险相关，这就是为什么随访不能只看症状。

哪些因素与不良结局相关

两个容易被忽略的问题

怎么检查：抗体、活检与超声Assessment

怎么治疗：症状治疗与系统治疗Treatment

治疗与管理一览

日常管理：护理、复查与风险监测Daily Management

十个常见误区Ten Common Misconceptions

参考文献与证据来源References & Evidence

本页共 24 条文献，来自国际共识与指南、随机对照试验、荟萃分析与机制研究，链接指向 PubMed。每条结论后的编号对应下方文献；证据不足或结论不一致之处已在正文标注。

1. 分类标准（2016 ACR/EULAR 原发性干燥综合征分类标准）Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, Rasmussen A, Scofield H, Vitali C, Bowman SJ, Mariette X, International Sjögren's Syndrome Criteria Working Group. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren's Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. Arthritis & rheumatology (Hoboken, N.J.). 2017;69(1):35-45. pubmed.ncbi.nlm.nih.gov/27785888
2. 分类标准（2012 ACR 干燥综合征分类标准：SICCA 队列的数据驱动与专家共识）Shiboski SC, Shiboski CH, Criswell L, Baer A, Challacombe S, Lanfranchi H, Schiødt M, Umehara H, Vivino F, Zhao Y, Dong Y, Greenspan D, Heidenreich AM, Helin P, Kirkham B, Kitagawa K, Larkin G, Li M, Lietman T, Lindegaard J, McNamara N, Sack K, Shirlaw P, Sugai S, Vollenweider C, Whitcher J, Wu A, Zhang S, Zhang W, Greenspan J, Daniels T, Sjögren's International Collaborative Clinical Alliance (SICCA) Research Groups. American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort. Arthritis care & research. 2012;64(4):475-487. pubmed.ncbi.nlm.nih.gov/22563590
3. 系统评价与荟萃分析（原发性干燥综合征的流行病学）Qin B, Wang J, Yang Z, Yang M, Ma N, Huang F, Zhong R. Epidemiology of primary Sjögren's syndrome: a systematic review and meta-analysis. Annals of the rheumatic diseases. 2015;74(11):1983-1989. pubmed.ncbi.nlm.nih.gov/24938285
4. 指南（EULAR 干燥综合征局部与系统治疗推荐）Ramos-Casals M, Brito-Zerón P, Bombardieri S, Bootsma H, De Vita S, Dörner T, Fisher BA, Gottenberg JE, Hernandez-Molina G, Kocher A, Kostov B, Kruize AA, Mandl T, Ng WF, Retamozo S, Seror R, Shoenfeld Y, Sisó-Almirall A, Tzioufas AG, Vitali C, Bowman S, Mariette X, EULAR-Sjögren Syndrome Task Force Group. EULAR recommendations for the management of Sjögren's syndrome with topical and systemic therapies. Annals of the rheumatic diseases. 2020;79(1):3-18. pubmed.ncbi.nlm.nih.gov/31672775
5. 系统评价（支持 EULAR 干燥综合征推荐的局部与系统用药证据）Brito-Zerón P, Retamozo S, Kostov B, Baldini C, Bootsma H, De Vita S, Dörner T, Gottenberg JE, Kruize AA, Mandl T, Ng WF, Seror R, Tzioufas AG, Vitali C, Bowman S, Mariette X, Ramos-Casals M. Efficacy and safety of topical and systemic medications: a systematic literature review informing the EULAR recommendations for the management of Sjögren's syndrome. RMD open. 2019;5(2):e001064. pubmed.ncbi.nlm.nih.gov/31749986
6. 诊断研究（362 例疑似病例中唇腺活检作为诊断标准的评估）Daniels TE. Labial salivary gland biopsy in Sjögren's syndrome. Assessment as a diagnostic criterion in 362 suspected cases. Arthritis and rheumatism. 1984;27(2):147-156. pubmed.ncbi.nlm.nih.gov/6696772
7. 系统评价（唾液腺超声在干燥综合征中的价值）Jousse-Joulin S, Milic V, Jonsson MV, Plagou A, Theander E, Luciano N, Rachele P, Baldini C, Bootsma H, Vissink A, Hocevar A, De Vita S, Tzioufas AG, Alavi Z, Bowman SJ, Devauchelle-Pensec V, US-pSS Study Group. Is salivary gland ultrasonography a useful tool in Sjögren's syndrome? A systematic review. Rheumatology (Oxford, England). 2016;55(5):789-800. pubmed.ncbi.nlm.nih.gov/26667216
8. 随机对照试验（毛果芸香碱治疗干燥综合征口干与眼干：373 例）Vivino FB, Al-Hashimi I, Khan Z, LeVeque FG, Salisbury PL, Tran-Johnson TK, Muscoplat CC, Trivedi M, Goldlust B, Gallagher SC. Pilocarpine tablets for the treatment of dry mouth and dry eye symptoms in patients with Sjögren syndrome: a randomized, placebo-controlled, fixed-dose, multicenter trial. P92-01 Study Group. Archives of internal medicine. 1999;159(2):174-181. pubmed.ncbi.nlm.nih.gov/9927101
9. 随机对照试验（西维美林治疗干燥综合征口干：75 例）Fife RS, Chase WF, Dore RK, Wiesenhutter CW, Lockhart PB, Tindall E, Suen JY. Cevimeline for the treatment of xerostomia in patients with Sjögren syndrome: a randomized trial. Archives of internal medicine. 2002;162(11):1293-1300. pubmed.ncbi.nlm.nih.gov/12038948
10. Cochrane 系统评价（局部环孢素 A 治疗干眼病）Priyadarshini SR, Sadhu S, Tzang CC, Niedert C, Holt CA, Han G, de Paiva CS, Pflugfelder SC, Akpek EK, Li T. Topical cyclosporine A therapy for dry eye disease. The Cochrane database of systematic reviews. 2026;7():CD010051. pubmed.ncbi.nlm.nih.gov/42464966
11. 随机对照试验（羟氯喹改善原发性干燥综合征症状：JOQUER，主要终点阴性）Gottenberg JE, Ravaud P, Puéchal X, Le Guern V, Sibilia J, Goeb V, Larroche C, Dubost JJ, Rist S, Saraux A, Devauchelle-Pensec V, Morel J, Hayem G, Hatron P, Perdriger A, Sene D, Zarnitsky C, Batouche D, Furlan V, Benessiano J, Perrodeau E, Seror R, Mariette X. Effects of hydroxychloroquine on symptomatic improvement in primary Sjögren syndrome: the JOQUER randomized clinical trial. JAMA. 2014;312(3):249-258. pubmed.ncbi.nlm.nih.gov/25027140
12. 系统评价（原发性干燥综合征的治疗：37 项试验）Ramos-Casals M, Tzioufas AG, Stone JH, Sisó A, Bosch X. Treatment of primary Sjögren syndrome: a systematic review. JAMA. 2010;304(4):452-460. pubmed.ncbi.nlm.nih.gov/20664046
13. 随机双盲安慰剂对照试验（利妥昔单抗治疗原发性干燥综合征）Meijer JM, Meiners PM, Vissink A, Spijkervet FK, Abdulahad W, Kamminga N, Brouwer E, Kallenberg CG, Bootsma H. Effectiveness of rituximab treatment in primary Sjögren's syndrome: a randomized, double-blind, placebo-controlled trial. Arthritis and rheumatism. 2010;62(4):960-968. pubmed.ncbi.nlm.nih.gov/20131246
14. 随机对照试验（利妥昔单抗改善原发性干燥综合征乏力与口干：TRACTISS，主要终点阴性）Bowman SJ, Everett CC, O'Dwyer JL, Emery P, Pitzalis C, Ng WF, Pease CT, Price EJ, Sutcliffe N, Gendi NST, Hall FC, Ruddock SP, Fernandez C, Reynolds C, Hulme CT, Davies KA, Edwards CJ, Lanyon PC, Moots RJ, Roussou E, Giles IP, Sharples LD, Bombardieri M. Randomized Controlled Trial of Rituximab and Cost-Effectiveness Analysis in Treating Fatigue and Oral Dryness in Primary Sjögren's Syndrome. Arthritis & rheumatology (Hoboken, N.J.). 2017;69(7):1440-1450. pubmed.ncbi.nlm.nih.gov/28296257
15. 荟萃分析（利妥昔单抗治疗原发性干燥综合征的疗效与安全性）Zheng X, Di J, Chen X, Li F, Liu Y, Cao J, Ning X, Wei L, Zheng G. Meta-analysis of the efficacy and safety of rituximab in the treatment of primary Sjögren's syndrome. Frontiers in immunology. 2025;16():1561214. pubmed.ncbi.nlm.nih.gov/40630946
16. 多中心随机对照试验（艾拉莫德治疗原发性干燥综合征）Chen X, Zhang T, Meng L, Xu C, Jiang L, Wang G, Hou T, Wang H, Han Y, Guan Y, Wang Y, Xue J. Efficacy and safety of iguratimod in patients with primary Sjögren's syndrome: a multicentre randomised controlled trial. RMD open. 2025;11(4):e006180. pubmed.ncbi.nlm.nih.gov/41423293
17. 系统评价与荟萃分析（原发性干燥综合征与非霍奇金淋巴瘤的关联：15 项队列）Ansari N, Salesi M. The association between primary Sjogren's syndrome and non-Hodgkin's lymphoma: a systematic review and meta-analysis of cohort studies. Clinical rheumatology. 2024;43(7):2177-2186. pubmed.ncbi.nlm.nih.gov/38722505
18. 队列研究（原发性干燥综合征的淋巴瘤与其他恶性肿瘤发生及淋巴瘤预测因素）Theander E, Henriksson G, Ljungberg O, Mandl T, Manthorpe R, Jacobsson LT. Lymphoma and other malignancies in primary Sjögren's syndrome: a cohort study on cancer incidence and lymphoma predictors. Annals of the rheumatic diseases. 2006;65(6):796-803. pubmed.ncbi.nlm.nih.gov/16284097
19. 全国登记研究（自身免疫相关先天性心脏传导阻滞的死亡、并发症与复发率：新生儿狼疮登记）Buyon JP, Hiebert R, Copel J, Craft J, Friedman D, Katholi M, Lee LA, Provost TT, Reichlin M, Rider L, Rupel A, Saleeb S, Weston WL, Skovron ML. Autoimmune-associated congenital heart block: demographics, mortality, morbidity and recurrence rates obtained from a national neonatal lupus registry. Journal of the American College of Cardiology. 1998;31(7):1658-1666. pubmed.ncbi.nlm.nih.gov/9626848
20. 队列研究（母亲使用羟氯喹与抗 SSA/Ro 抗体相关新生儿狼疮心脏表现复发风险下降相关）Izmirly PM, Costedoat-Chalumeau N, Pisoni CN, Khamashta MA, Kim MY, Saxena A, Friedman D, Llanos C, Piette JC, Buyon JP. Maternal use of hydroxychloroquine is associated with a reduced risk of recurrent anti-SSA/Ro-antibody-associated cardiac manifestations of neonatal lupus. Circulation. 2012;126(1):76-82. pubmed.ncbi.nlm.nih.gov/22626746
21. 横断面研究（原发性干燥综合征乏力的患病率、严重程度与预测因素）Segal B, Thomas W, Rogers T, Leon JM, Hughes P, Patel D, Patel K, Novitzke J, Rohrer M, Gopalakrishnan R, Myers S, Nazmul-Hossain A, Emamian E, Huang A, Rhodus N, Moser K. Prevalence, severity, and predictors of fatigue in subjects with primary Sjögren's syndrome. Arthritis and rheumatism. 2008;59(12):1780-1787. pubmed.ncbi.nlm.nih.gov/19035421
22. 系统评价与荟萃分析（老年人中口干的全球患病率）Kamnoedboon P, Spyraki F, Thomson WM, Srinivasan M. A Systematic Review and Meta-Analysis of the Global Prevalence of Dry Mouth in Older Adults. Gerodontology. 2026;43(3):383-402. pubmed.ncbi.nlm.nih.gov/42144904
23. 病例系列研究（免疫检查点抑制剂治疗相关的干燥综合征）Warner BM, Baer AN, Lipson EJ, Allen C, Hinrichs C, Rajan A, Pelayo E, Beach M, Gulley JL, Madan RA, Feliciano J, Grisius M, Long L, Powers A, Kleiner DE, Cappelli L, Alevizos I. Sicca Syndrome Associated with Immune Checkpoint Inhibitor Therapy. The oncologist. 2019;24(9):1259-1269. pubmed.ncbi.nlm.nih.gov/30996010
24. 队列研究（中国原发性干燥综合征患者的恶性肿瘤发生率）Zhang W, Feng S, Yan S, Zhao Y, Li M, Sun J, Zhang FC, Cui Q, Dong Y. Incidence of malignancy in primary Sjogren's syndrome in a Chinese cohort. Rheumatology (Oxford, England). 2010;49(3):571-577. pubmed.ncbi.nlm.nih.gov/20040528