Risk factors for relapse of IgG4-related disease: a systematic review and meta‑analysis

Patients with IgG4-related disease (IgG4-RD) suffer high relapse during long-term treatment, but risk factors associated with relapse are not well established. This study aims to review the potential risk factors and integrate relapse rates of observational studies for IgG4-RD.

Relevant articles published up to January 28, 2025, were comprehensively and systematically identified from PubMed, EMBASE, and Web of Science databases. A systematic review and meta-analysis was performed to estimate pooled odds ratios (ORs), hazard ratios (HRs), and 95% confidence interval (CI).

Twenty-four studies comprising 3797 patients were included. Our findings reveal that a history of allergies (OR: 2.97, 95%CI: 2.07-4.25, P < 0.001), multi-organ involvement (HR: 1.60, 95% CI: 1.23-2.09, P = 0.0005), and decreased complement levels (HR: 2.27, 95% CI: 1.04-4.93, P = 0.04) are significantly associated with relapse. Furthermore, the combination of corticosteroids and immunosuppressants significantly reduces relapse rates (HR: 0.25, 95% CI: 0.10-0.62, P = 0.003). The pooled 12-, 24-, and 36-month relapse rates were 17% (95% CI: 0.10-0.24), 26% (95% CI: 0.22-0.31) and 33% (95% CI: 0.26-0.39), respectively.

This meta-analysis indicated that a history of allergy, multi-organ involvement, and decreased complement levels were risk factors for IgG4-RD relapse. Besides, corticosteroid combined with immunosuppressant therapy could decrease relapse rates. Key Points • A history of allergy, multi-organ involvement, and decreased complement levels were risk factors for IgG4-RD relapse. • The combination of corticosteroids and immunosuppressants could reduces relapse rates of IgG4-RD. • The 12-, 24- and 36-month relapse rates of IgG4-RD were 17%, 26% and 33%, respectively.