Clinical Analysis of Thirty-one Cases of IgG4-related Disease

doi:10.13701/j.cnki.kqyxyj.2021.02.018

Abstract

Abstract: Objective: To improve the understanding, diagnosis, and treatment of IgG4-RD. Methods: The clinical, imaging, serological, pathological, and therapeutic features of 31 patients with IgG4-RD diagnosed in the first affiliated Hospital of Xinjiang Medical University were analyzed, and IgG4-RD responder index was used to evaluate disease activity and damage. Results: Among the 31 patients, there were 18 males and 13 females, with an average age of (59.1±9.12) years. The median time from onset to diagnosis was 10 months (range 1-120 months). Six cases were misdiagnosed at the first diagnosis, with a misdiagnosis rate of 19.35%. 32.26% of the patients were complicated with allergic diseases. Imaging revealed that all patients had organ occupying, and all patients had 2 or more organs involved, mainly salivary gland (77.42%), followed by lymph nodes, lacrimal gland, bile duct, and so on. The IgG4-RD responder index was 7.13±3.25, and it was positively correlated with the involved organs (r=0.15, P<0.0001). Serum IgG4 increased in 93.55% of these patients, and it was positively correlated with serum IgG (r=0.54, P<0.01). Histopathology showed infiltration of lymphatic plasma cells, obvious proliferation of lymphoid follicles and irregular fibrosis between tissues. Among the 25 patients who took glucocorticoid drugs, one patient was treated with rituximab because of ineffective treatment, and two patients were treated with immunosuppressants. The follow-up time was (25.89±14.02) months. Twelve patients had good results after treatment, the level of IgG4 decreased. Twelve patients had recurrence during treatment, with a recurrence rate of 48%. One case died of heart attack during treatment. Conclusion: IgG4-RD is a multi-system fibro-inflammatory disease, which is common in middle-aged and elderly men. Enlargement of salivary glands, lymph nodes, lacrimal glands, and internal organs is a common clinical manifestation. Glucocorticoids and biological agents are effective in the treatment, but there is a recurrence.

Key words: IgG4-related diseases, clinical characteristics, diagnosis, treatment

PU Jiao, HU Lulu, NIU Yaqi, JIA Mengying, ZENG Xuemin, HE Wei, GONG Zhongcheng. Clinical Analysis of Thirty-one Cases of IgG4-related Disease[J]. Journal of Oral Science Research, 2021, 37(2): 173-178.

References

[1] Chen LYC, Mattman A, Seidman MA, et al. IgG4-related disease: what a hematologist needs to know [J]. Haematologica, 2019, 104(3):444-455.
[2] 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.
[3] 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.
[4] 钟宇,田芳,邹明宇,等.IgG4相关性疾病的影像学特征分析[J].中国医科大学学报,2019,48(10):939-943.
[5] 张盼盼,张文.IgG-4相关性疾病脏器损伤的临床特点、影像学及病理特征[J].中华内科杂志,2016,55(8):657-661.
[6] Wang L, Zhang P, Zhang X, et al. Sex disparities in clinical characteristics and prognosis of immunoglobulin G4-related disease: a prospective study of 403 patients [J]. Rheumatology (Oxford), 2019,58(5): 820-830.
[7] Sekiguchi H, Horie R, Kanai M, et al. IgG4-related disease: retrospective analysis of one hundred sixty-six patients [J]. Arthritis Rheumatol, 2016,68(9): 2290-2299.
[8] Campochiaro C, Ramirez GA, Bozzolo EP, et al. IgG4-related disease in Italy: clinical features and outcomes of a large cohort of patients [J]. Scand J Rheumatol, 2016,45(2):135-145.
[9] 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.
[10] Culver EL, Sadler R, Bateman AC, et al. Increases in IgE, eosinophils, and mast cells can be used in diagnosis and to predict relapse of IgG4-related disease [J]. Clin Gastroenterol Hepatol, 2017,15(9):1444-1452.
[11] Moteki H, Yasuo M, Hamano H, et al. IgG4-related chronic rhinosinusitis: a new clinical entity of nasal disease [J]. Acta Otolaryngol, 2011,131(5):518-526.
[12] Tanaka A, Moriyama M, Nakashima H, et al. Th2 and regulatory immune reactions contribute to IgG4 production and the initiation of Mikulicz disease [J]. Arthritis Rheum, 2012,64(1):254-263.
[13] Dong ZM, Lin E, Wechsler ME, et al. Pulmonary eosinophilic granulomatosis with polyangiitis has IgG4 plasma cells and immunoregulatory features [J]. Am J Pathol, 2020, 190(7):1438-1448.
[14] Zhang X, Zhang P, Peng L, et al. Clinical characteristics of a concurrent condition of IgG4-RD and Castleman's disease [J]. Clin Rheumatol, 2018, 37(12): 3387-3395.
[15] Li KM, Xu MH, Wu X, et al. The expression of IgG and IgG4 in orbital MALT lymphoma: the similarities and differences of IgG4-related diseases [J]. Onco Targets Ther, 2020, 13:5755-5761.
[16] Majumder S, Mohapatra S, Lennon RJ, et al. Rituximab maintenance therapy reduces rate of relapse of pancreaticobiliary immunoglobulin G4-related disease [J]. Clin Gastroenterol Hepatol, 2018,16(5):1947-1953.
[17] Schirmer JH, Hoyer BF. IgG4-related disease [J]. Dtsch Med Wochenschr, 2019,144(24):1726-1730.
[18] Kamisawa T, Zen Y, Pillai S, et al. IgG4-related disease [J]. Lancet, 2015, 385(9976):1460-1471.
[19] Terao T, Yamamoto K, Ikeuchi K, et al. Hyper-IL-6 syndrome mimicking IgG4-related disease [J]. Rinsho Ketsueki, 2019, 60(5):392-397.
[20] Tsuboi H, Honda F, Takahashi H, et al. Pathogenesis of IgG4-related disease. comparison with Sjögren's syndrome [J]. Mod Rheumatol, 2020, 30(1):7-16.
[21] 李晓杰,邵博,刘慧,等.免疫球蛋白G4相关性涎腺炎12例临床特点分析[J].中华风湿病学杂志,2019,23(9):599-604.
[22] Gupta A, Shareef M, Lade H, et al. Kimura's disease: A diagnostic and therapeutic challenge [J]. Indian J Otolaryngol Head Neck Surg, 2019, 71:855-859.
[23] Blaho M, Dítē P, Kunovský L, et al. Autoimmune pancreatitis-An ongoing challenge [J]. Adv Med Sci, 2020, 65(2):403-408.
[24] Sedassari BT, Dos Santos HT, Mariano FV, et al. Carcinoma expleomorphic adenoma of minor salivary glands with major epithelial-myoepithelial component: clinicopathologic and immunohistochemical study of 3 cases [J]. AnnDiagn Pathol, 2015, 19(3):164-168.
[25] 赵厚亮,王文涛,王伟,等.CT纹理分析在腮腺多形性腺瘤与腺淋巴瘤鉴别诊断中的价值[J].口腔医学研究,2018,34(10):1127-1131.
[26] Shirakashi M, Yoshifuji H, Kodama Y, et al. Factors in glucocorticoid regimens associated with treatment response and relapses of IgG4-related disease: a multicentre study [J]. Sci Rep, 2018, 8(1):10262.
[27] 滕菲,张文.生物制剂在IgG4相关性疾病中的临床应用:现状与前景[J].中华临床免疫和变态反应杂志,2019,13(6):473-476.
[28] Shimizu Y, Yamamoto M, Naishiro Y, et al. Necessity of early intervention for IgG4-related disease-delayed treatment induces fibrosis progression [J]. Rheumatology(Oxford), 2013, 52(4):679-683.