AB1244 PROGNOSTIC FACTORS IN IGG4-RELATED DISEASE: A LONG-TERM MONOCENTRIC CHINESE COHORT STUDY
Authors: Z. Ji, L. Ma, L. Zhang, L. Ma, D. Liu, Y. Sun, X. Kong, X. Dai, R. Chen, H. Chen, L. Jiang
Journal: Annals of the Rheumatic Diseases (2020)

Background: Through initial response to treatment with GC, the patients with IgG4-related disease (IgG4-RD) exhibited high relapse rate after reduction or withdrawal of GC treatment, indicated the unsatisfactory prognosis for IgG4-RD. It is of clinical significance to develop new informative risk factors for refractory and relapsed disease. Objectives: To evaluate the prognosis of IgG4-RD and identify predictive factors for treatment resistance and disease relapse in a Chinese cohort. Methods: 102 patients newly diagnosed with IgG4-RD were followed for 6-111 months. Clinical data were compared between patients whose disease went into remission and those who suffered refractory or relapsed disease. Predictive factors for refractory and relapsed disease were calculated by univariate analysis. Results: Among the 78 patients who received medical treatment with regular follow-up, 55 (59.8%) patients sustained clinical remission, and 23 (25%) patients suffered refractory or relapsed disease. The mortality and incidence of malignancy were both 4.35% during follow-up. Serum TNF-α ≥ 13 pg/ml, sIL-2R ≥ 1010 pg/ml, TC &lt; 3.55 mmol/L, LDL &lt; 2.0 mmol/L, IgG &gt; 20.2 g/L, GC withdrawal, and treatment without immunosuppressor (IM) during the maintenance period (OR 3.23) were predictive factors for refractory and relapsed IgG4-RD. The combination of GC and IM treatment was protective (OR 0.338) against refractory and relapsed IgG4-RD. Conclusion: Serum TNF-α, sIL-2R, LDL, TC, IgG, GC withdrawal, and treatment without IM during the maintenance period were predictive factors for refractory and relapsed IgG4-RD. Treatment with GC and IM may protect against refractory and relapsed IgG4-RD. References: [1]Takahashi H, Yamamoto M, Suzuki C, Naishiro Y, Shinomura Y, Imai K. The birthday of a new syndrome: IgG4-related diseases constitute a clinical entity. Autoimmun Rev. 2010, 9: 591-4. [2]Lian L, Wang C, Tian JL. IgG4-related retroperitoneal fibrosis: a newly characterized disease. Int J Rheum Dis. 2016, 19: 1049-55. [3]Li PH, Ko KL, Ho CT, Lau LL, Tsang RK, Cheung TT , et al . Immunoglobulin G4-related disease in Hong Kong: clinical features, treatment practices, and its association with multisystem disease. Hong Kong Med J. 2017, 23: 446-53. [4]Culver EL, Sadler R, Bateman AC, Makuch M, Cargill T, Ferry B , et al . Increases in IgE, Eosinophils, and Mast Cells Can be Used in Diagnosis and to Predict Relapse of IgG4-Related Disease. Clin Gastroenterol Hepatol. 2017, 15: 1444-52. [5]Inoue D, Yoshida K, Yoneda N, Ozaki K, Matsubara T, Nagai K , et al . IgG4-related disease: dataset of 235 consecutive patients. Medicine (Baltimore). 2015, 94: e680. [6]Brito-Zeron P, Kostov B, Bosch X, Acar-Denizli N, Ramos-Casals M, Stone JH. Therapeutic approach to IgG4-related disease: A systematic review. Medicine (Baltimore). 2016, 95: e4002. [7]Peng Y, Li JQ, Zhang PP, Zhang X, Peng LY, Chen H , et al . Clinical outcomes and predictive relapse factors of IgG4-related disease following treatment: a long-term cohort study. J Intern Med. 2019. Acknowledgments: This work was supported by the National Natural Science Foundation of China (NSFC 81601398; NSFC 81771730); the Animal Research Project of Shanghai Science and Technology Commission (grant number 17140902000); and Shanghai Pujiang Rheumatologists Training Program (SPROG201801) Disclosure of Interests: None declared