《IgG4相关性疾病诊治中国专家共识》解读

周佳鑫; 张文

doi:10.3969/j.issn.1001-5256.2021.09.012

《IgG4相关性疾病诊治中国专家共识》解读

DOI: 10.3969/j.issn.1001-5256.2021.09.012

周佳鑫,
张文^,

中国医学科学院北京协和医院风湿免疫科，国家皮肤和免疫性疾病临床研究中心，教育部重点实验室，疑难重症和罕见病国家重点实验室，北京 100730

基金项目:

首都卫生发展科研专项基金 (2020-2-4017);

中国医学科学院中央级公益性科研院所基本科研业务费专项资金资助 (2019XK320038);

国家自然基金面上项目 (82071839)

利益冲突声明：所有作者均声明不存在利益冲突。

作者贡献声明：周佳鑫参与起草及修改文章关键内容；张文负责确定文章的思路及论文框架设计。

详细信息

通信作者:
张文，zhangwen91@sina.com

中图分类号: R593.2
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- 被引次数: 0
出版历程
- 收稿日期: 2021-08-16
- 录用日期: 2021-08-16
- 出版日期: 2021-09-20

Interpretation of Chinese expert consensus on the diagnosis and treatment of IgG4-related diseases

Jiaxin ZHOU,
Wen ZHANG^,

National Clinical Research Center for Dermatologic and Immunologic Diseases, Key Laboratory of Ministry of Education, National Key Laboratory of Difficult Severe Diseases and Rare Diseases, Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China

Research funding:

The Capital's Funds for Health Improvement and Research (2020-2-4017);

the Non-profit Central Research Institute Fund of Chinese Academy of Medical Sciences (2019XK320038);

the National Natural Science Foundation of China (82071839)

摘要

摘要: IgG4相关性疾病(IgG4-RD)是一种以受累脏器慢性炎症伴纤维化为特征表现的免疫介导的疾病。近年来，我国学者对该病的认识逐渐深入，积累了较多的诊治经验，并于2020年在中国罕见病联盟和中华医学会风湿病学分会的平台上，由专家组制定了首个《IgG4相关性疾病诊治的中国专家共识》。该共识较全面概括了IgG4相关性疾病的诊治原则，为临床医生对该病的诊治提供了较好的依据。现就该指南的内容进行解读。
- 免疫球蛋白G /
- 诊断 /
- 治疗学 /
- 共识
Abstract: IgG4-related disease (IgG4-RD) is an immune-mediated disease characterized by chronic inflammation and fibrosis of affected organs. With a deeper understanding of this disease among Chinese scholars and the rich experience in diagnosis and treatment in recent years, an expert group was organized by China Alliance for Rare Diseases and Chinese Rheumatology Association in 2020 and developed the first Chinese expert consensus on the diagnosis and treatment of IgG4-related diseases. This consensus comprehensively summarizes the principles of the diagnosis and treatment of IgG4-RD and thus provides a basis for its diagnosis and treatment by clinicians.
- Immunoglobulin G /
- Diagnosis /
- Therapeutics /
- Consensus

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参考文献(42)

[1]	KHOSROSHAHI A, WALLACE ZS, CROWE JL, et al. International consensus guidance statement on the management and treatment of IgG4-related disease[J]. Arthritis Rheumatol, 2015, 67(7): 1688-1699. DOI: 10.1002/art.39132.
[2]	ZHANG W, DONG LL, ZHU J, et al. Chinese expert consensus on the diagnosis and treatment of IgG4 related diseases[J]. Chin J Intern Med, 2021, 60(3): 192-206. DOI: 10.3760/cma.j.cn112138-20200803-00726. 张文, 董凌莉, 朱剑, 等. IgG4相关性疾病诊治中国专家共识[J]. 中华内科杂志, 2021, 60(3): 192-206. DOI: 10.3760/cma.j.cn112138-20200803-00726.
[3]	STONE JH, KHOSROSHAHI A, DESHPANDE V, et al. Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations[J]. Arthritis Rheum, 2012, 64(10): 3061-3067. DOI: 10.1002/art.34593.
[4]	KAMISAWA T, ZEN Y, PILLAI S, et al. IgG4-related disease[J]. Lancet, 2015, 385(9976): 1460-1471. DOI: 10.1016/S0140-6736(14)60720-0.
[5]	LIU Y, XUE M, WANG Z, et al. Salivary gland involvement disparities in clinical characteristics of IgG4-related disease: A retrospective study of 428 patients[J]. Rheumatology (Oxford), 2020, 59(3): 634-640. DOI: 10.1093/rheumatology/kez280.
[6]	LI W, CHEN Y, SUN ZP, et al. Clinicopathological characteristics of immunoglobulin G4-related sialadenitis[J]. Arthritis Res Ther, 2015, 17(1): 186. DOI: 10.1186/s13075-015-0698-y.
[7]	UMEHARA H, OKAZAKI K, MASAKI Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011[J]. Mod Rheumatol, 2012, 22(1): 21-30. DOI: 10.1007/s10165-011-0571-z.
[8]	WALLACE ZS, NADEN RP, CHARI S, et al. The 2019 American College of Rheumatology/European league against rheumatism classification criteria for IgG4-related disease[J]. Arthritis Rheumatol, 2020, 72(1): 7-19. DOI: 10.1002/art.41120.
[9]	HAMANO H, KAWA S, HORIUCHI A, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis[J]. N Engl J Med, 2001, 344(10): 732-738. DOI: 10.1056/NEJM200103083441005.
[10]	CARRUTHERS MN, KHOSROSHAHI A, AUGUSTIN T, et al. The diagnostic utility of serum IgG4 concentrations in IgG4-related disease[J]. Ann Rheum Dis, 2015, 74(1): 14-18. DOI: 10.1136/annrheumdis-2013-204907.
[11]	WANG Y, GUAN Z, GAO D, et al. The value of (18)F-FDG PET/CT in the distinction between retroperitoneal fibrosis and its malignant mimics[J]. Semin Arthritis Rheum, 2018, 47(4): 593-600. DOI: 10.1016/j.semarthrit.2017.07.011.
[12]	ZHANG J, CHEN H, MA Y, et al. Characterizing IgG4-related disease with ¹⁸F-FDG PET/CT: A prospective cohort study[J]. Eur J Nucl Med Mol Imaging, 2014, 41(8): 1624-1634. DOI: 10.1007/s00259-014-2729-3.
[13]	ZHAO Z, WANG Y, GUAN Z, et al. Utility of FDG-PET/CT in the diagnosis of IgG4-related diseases[J]. Clin Exp Rheumatol, 2016, 34(1): 119-125. http://www.ncbi.nlm.nih.gov/pubmed/26842851
[14]	DESHPANDE V, ZEN Y, CHAN JK, et al. Consensus statement on the pathology of IgG4-related disease[J]. Mod Pathol, 2012, 25(9): 1181-1192. DOI: 10.1038/modpathol.2012.72.
[15]	ARORA K, RIVERA M, TING DT, et al. The histological diagnosis of IgG4-related disease on small biopsies: Challenges and pitfalls[J]. Histopathology, 2019, 74(5): 688-698. DOI: 10.1111/his.13787.
[16]	CAMPBELL SN, RUBIO E, LOSCHNER AL. Clinical review of pulmonary manifestations of IgG4-related disease[J]. Ann Am Thorac Soc, 2014, 11(9): 1466-1475. DOI: 10.1513/AnnalsATS.201403-128FR.
[17]	WANG ZQ, LIU YY, ZHANG X, et al. Analysis of the clinical features and misdiagnosis reasons of 17 patients misdiagnosed with IgG4-related disease[J]. J Peking Univ(Health Sci), 2019, 51(6): 1025-1031. DOI: 10.19723/j.issn.1671-167X.2019.06.008. 王子乔, 刘燕鹰, 张霞, 等. 17例误诊为IgG4相关疾病患者的临床特点及误诊原因分析[J]. 北京大学学报(医学版), 2019, 51(6): 1025-1031. DOI: 10.19723/j.issn.1671-167X.2019.06.008.
[18]	HONG X, LI W, XIE XY, et al. Differential diagnosis of IgG4-related sialadenitis, primary Sjögren syndrome, and chronic obstructive submandibular sialadenitis[J]. Br J Oral Maxillofac Surg, 2017, 55(2): 179-184. DOI: 10.1016/j.bjoms.2016.10.021.
[19]	BAI Z, CHEN Y, CHEN X, et al. A medical mirroring: Chronic myelomonocytic leukemia mimicking immunoglobulin G4-related disease[J]. Am J Transl Res, 2019, 11(7): 4561-4567. http://www.researchgate.net/publication/335099953_A_medical_mirroring_chronic_myelomonocytic_leukemia_mimicking_immunoglobulin_G4-related_disease
[20]	WALLACE ZS, KHOSROSHAHI A, CARRUTHERS MD, et al. An international multispecialty validation study of the IgG4-related disease responder index[J]. Arthritis Care Res (Hoboken), 2018, 70(11): 1671-1678. DOI: 10.1002/acr.23543.
[21]	ZHANG W, STONE J. Management of IgG4 related disease[J]. The Lancet Rheumatol, 2019, 1(1): e55-e65. DOI: https://doi.org/ 10.1016/S2665-9913(19)30017-7.
[22]	MIKI M, FUJIMORI N, OONO T, et al. Relapse patterns and predictors of IgG4-related diseases involved with autoimmune pancreatitis: A single-center retrospective study of 115 patients[J]. J Dig Dis, 2019, 20(3): 152-158. DOI: 10.1111/1751-2980.12708.
[23]	BACKHUS J, SEUFFERLEIN T, PERKHOFER L, et al. IgG4-related diseases in the gastrointestinal tract: Clinical presentation, diagnosis and treatment challenges[J]. Digestion, 2019, 100(1): 1-14. DOI: 10.1159/000492814.
[24]	OKAZAKI K, UCHIDA K. Current perspectives on autoimmune pancreatitis and IgG4-related disease[J]. Proc Jpn Acad Ser B Phys Biol Sci, 2018, 94(10): 412-427. DOI: 10.2183/pjab.94.027.
[25]	OKAZAKI K, CHARI ST, FRULLONI L, et al. International consensus for the treatment of autoimmune pancreatitis[J]. Pancreatology, 2017, 17(1): 1-6. DOI: 10.1016/j.pan.2016.12.003.
[26]	KAMISAWA T, NAKAZAWA T, TAZUMA S, et al. Clinical practice guidelines for IgG4-related sclerosing cholangitis[J]. J Hepatobiliary Pancreat Sci, 2019, 26(1): 9-42. DOI: 10.1002/jhbp.596.
[27]	EBBO M, DANIEL L, PAVIC M, et al. IgG4-related systemic disease: Features and treatment response in a French cohort: Results of a multicenter registry[J]. Medicine (Baltimore), 2012, 91(1): 49-56. DOI: 10.1097/MD.0b013e3182433d77.
[28]	RAINA A, YADAV D, KRASINSKAS AM, et al. Evaluation and management of autoimmune pancreatitis: Experience at a large US center[J]. Am J Gastroenterol, 2009, 104(9): 2295-2306. DOI: 10.1038/ajg.2009.325.
[29]	WU Q, CHANG J, CHEN H, et al. Efficacy between high and medium doses of glucocorticoid therapy in remission induction of IgG4-related diseases: A preliminary randomized controlled trial[J]. Int J Rheum Dis, 2017, 20(5): 639-646. DOI: 10.1111/1756-185X.13088.
[30]	WALLACE ZS, MATTOO H, MAHAJAN VS, et al. Predictors of disease relapse in IgG4-related disease following rituximab[J]. Rheumatology (Oxford), 2016, 55(6): 1000-1008. DOI: 10.1093/rheumatology/kev438.
[31]	MASAMUNE A, NISHIMORI I, KIKUTA K, et al. Randomised controlled trial of long-term maintenance corticosteroid therapy in patients with autoimmune pancreatitis[J]. Gut, 2017, 66(3): 487-494. DOI: 10.1136/gutjnl-2016-312049.
[32]	OMAR D, CHEN Y, CONG Y, et al. Glucocorticoids and steroid sparing medications monotherapies or in combination for IgG4-RD: A systematic review and network meta-analysis[J]. Rheumatology (Oxford), 2020, 59(4): 718-726. DOI: 10.1093/rheumatology/kez380.
[33]	WANG Y, LI K, GAO D, et al. Combination therapy of leflunomide and glucocorticoids for the maintenance of remission in patients with IgG4-related disease: A retrospective study and literature review[J]. Intern Med J, 2017, 47(6): 680-689. DOI: 10.1111/imj.13430.
[34]	YUNYUN F, YU C, PANPAN Z, et al. Efficacy of Cyclophosphamide treatment for immunoglobulin G4-related disease with addition of glucocorticoids[J]. Sci Rep, 2017, 7(1): 6195. DOI: 10.1038/s41598-017-06520-5.
[35]	ZHANG P, GONG Y, LIU Z, et al. Efficacy and safety of iguratimod plus corticosteroid as bridge therapy in treating mild IgG4-related diseases: A prospective clinical trial[J]. Int J Rheum Dis, 2019, 22(8): 1479-1488. DOI: 10.1111/1756-185X.13633.
[36]	HONG X, ZHANG YY, LI W, et al. Treatment of immunoglobulin G4-related sialadenitis: Outcomes of glucocorticoid therapy combined with steroid-sparing agents[J]. Arthritis Res Ther, 2018, 20(1): 12. DOI: 10.1186/s13075-017-1507-6.
[37]	CARRUTHERS MN, TOPAZIAN MD, KHOSROSHAHI A, et al. Rituximab for IgG4-related disease: A prospective, open-label trial[J]. Ann Rheum Dis, 2015, 74(6): 1171-1177. DOI: 10.1136/annrheumdis-2014-206605.
[38]	XIN L, MENG QQ, HU LH, et al. Prediction and management for relapse of type 1 autoimmune pancreatitis after initial steroid treatment: A long-term follow-up from China[J]. Pancreas, 2018, 47(9): 1110-1114. DOI: 10.1097/MPA.0000000000001147.
[39]	LIU Y, ZENG Q, ZHU L, et al. Relapse predictors and serologically unstable condition of IgG4-related disease: A large Chinese cohort[J]. Rheumatology (Oxford), 2020, 59(8): 2115-2123. DOI: 10.1093/rheumatology/kez669.
[40]	PENG Y, LI JQ, ZHANG PP, et al. Clinical outcomes and predictive relapse factors of IgG4-related disease following treatment: A long-term cohort study[J]. J Intern Med, 2019, 286(5): 542-552. DOI: 10.1111/joim.12942.
[41]	HART PA, KAMISAWA T, BRUGGE WR, et al. Long-term outcomes of autoimmune pancreatitis: A multicentre, international analysis[J]. Gut, 2013, 62(12): 1771-1776. DOI: 10.1136/gutjnl-2012-303617.
[42]	PENG L, ZHANG P, LI J, et al. IgG4-related aortitis/periaortitis and periarteritis: A distinct spectrum of IgG4-related disease[J]. Arthritis Res Ther, 2020, 22(1): 103. DOI: 10.1186/s13075-020-02197-w.