Improved clinical outcomes of tocilizumab versus cyclophosphamide for IgG4-related disease: insights from a prospective IgG4-related disease registry

Ji Zongfei; Zhang Lijuan; Sun Ying; Liu Dongmei; Wu Sifan; Kong Xiufang; Ma Lingying; Liu Yun; Ma Lili; Chen Huiyong; Jiang Lindi

doi:10.1177/20406223211028776

Improved clinical outcomes of tocilizumab versus cyclophosphamide for IgG4-related disease: insights from a prospective IgG4-related disease registry

Ther Adv Chronic Dis. 2021 Jun 30:12:20406223211028776. doi: 10.1177/20406223211028776. eCollection 2021.

Authors

Ji Zongfei¹, Zhang Lijuan², Sun Ying¹, Liu Dongmei¹, Wu Sifan¹, Kong Xiufang¹, Ma Lingying¹, Liu Yun¹, Ma Lili¹, Chen Huiyong¹, Jiang Lindi³

Affiliations

¹ Department of Rheumatology, Zhongshan Hospital, Fudan University, Shanghai, China Center of Clinical Epidemiology and Evidence-Based Medicine, Fudan University, Shanghai, China.
² Department of Rheumatology, Xiamen Branch, Zhongshan Hospital, Fudan University, Xiamen, Fujian Province, China.
³ Department of Rheumatology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032, China Center of Clinical Epidemiology and Evidence-Based Medicine, Fudan University, Shanghai, China.

Abstract

Objective: To compare the clinical outcomes of patients with active immunoglobulin G (IgG) 4 related disease (IgG4-RD) receiving tocilizumab versus those receiving cyclophosphamide (CYC).

Methods: This IgG4-RD registry study was a prospective cohort study conducted among patients with active IgG4-RD hospitalized at Zhongshan Hospital, Fudan University. Patients who were treated with tocilizumab or CYC along with glucocorticoids (GCs) were enrolled. All participants were followed up at the hospital clinic at 3 and 6 months after discharge. Primary clinical outcomes were measured via the IgG4-RD responder index (RI), complete response (CR), and partial response (PR), as well as side effects.

Results: From January 2015 to June 2020, 29 patients enrolled. Fourteen and 15 patients were treated with tocilizumab and CYC, respectively. At the 6-month follow-up, disease activity parameters including erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), IgG4, and IgG4-RD RI, decreased significantly in both groups. At 6 months, tocilizumab demonstrated its superiority, with 50% of patients achieving CR in the Tocilizumab group versus 20% in the CYC group. However, no statistical significance was identified (p = 0.128). The GC dosage at 6 months was significantly lower in the tocilizumab group than in the CYC group [10 (9.4-15) mg/d versus 15 (15-15) mg/d, p = 0.025]. In the CYC group, two patients experienced lumbar vertebral compression fractures related to GCs. Other patients in both groups showed mild adverse effects.

Conclusions: Tocilizumab could be a better steroid-sparing agent, with a comparable curative effect and tolerance, than CYC, in the treatment of IgG4-RD.

Keywords: IgG4-related disease; clinical outcome; cyclophosphamide; tocilizumab; treatment response.