Prognostic factors in IgG4-related disease: a long-term monocentric Chinese cohort study
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ORIGINAL ARTICLE
Prognostic factors in IgG4-related disease: a long-term monocentric Chinese cohort study Ji Zongfei 1,2 & Ma Lingying 1,2 & Zhang Lijuan 1,2 & Sun Ying 1,2 & Chen Rongyi 1,2 & Liu Dongmei 1,2 & Kong Xiufang 1,2 & Dai Xiaomin 1,2 & Ma Lili 1,2 & Chen Huiyong 1,2 & Jiang Lindi 1,2 Received: 17 September 2020 / Revised: 17 September 2020 / Accepted: 26 October 2020 # International League of Associations for Rheumatology (ILAR) 2020
Abstract Objectives Patients with IgG4-related disease (IgG4-RD) suffer high relapse rates during long-term treatment, but factors that predict relapse outcomes are not well established. In the present study, we aimed to identify predictive factors for treatment resistance and disease relapse in a Chinese IgG4-RD cohort. Methods This study enrolled 102 patients newly diagnosed with IgG4-RD. Disease prognosis was determined by evaluating disease activity and dosage of glucocorticoids. Predictive factors for refractory and relapsed disease were identified by univariate analysis and Cox regression. Results Among the 102 patients, 78 cases received medical treatment with regular follow-up (21 [6-111] months). During the follow-up period, 55 (70.5%) patients sustained clinical remission, and 23 (29.5%) patients suffered refractory or relapsed disease. The relapse rate of the patients with IgG4-RD was significantly higher among patients who stopped taking medicine than among those who continued treatment with glucocorticoids (GC) + immunosuppressor (IM). Serum TNF-α ≥ 13 pg/mL, sIL-2R ≥ 1010 U/mL, total cholesterol < 3.55 mmol/L, low-density lipoprotein < 2.0 mmol/L, IgG ≥ 20.2 g/L, and drug withdrawal were predictive factors for refractory and relapsed IgG4-RD. Multivariate Cox regression revealed that both sIL2R and TNF-α were independent risk factors for refractory and relapsed disease. The combination of GC and IM treatment was an independent protective factor against refractory and relapsed IgG4-RD. Conclusions High serum levels of sIL-2R and TNF-α may be informative risk factors for refractory and relapsed IgG4-RD. Our data suggest that a combination treatment of GC along with IM may be protective against refractory and relapsed IgG4-RD. Key Points • High sIL-2R and TNF-α levels are informative risk factors for refractory and relapsed IgG4-related disease. • Combination treatment of GC with IM protects against refractory and relapsed IgG4-related disease
Keywords IgG4-related disease . Prognosis . Prognostic factors . Relapse . Treatment resistance
Introduction Supplementary Information The online version contains supplementary material available at https://doi.org/10.1007/s10067-02005484-8. * Jiang Lindi [email protected] 1
Department of Rheumatology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai 200032, China
2
Center of Clinical Epidemiology and Evidence-based Medicine, Fudan University, Shanghai, China
IgG4-related disease (IgG4-RD) is a systematic, inflammatory, autoimmune disease characterized by elevated levels of serum IgG4 and IgG4+ p
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