Efficacy and Safety of Glucocorticoid Monotherapy Versus the Combination of Glucocorticoid and Immunosuppressive Agents for Immunoglobulin G4-Related Disease: A Systematic Review and Meta-Analysis

We conducted the current systematic review and meta-analysis to evaluate the efficacy and safety of the combination of glucocorticoid and immunosuppressive agents (IM) compared to glucocorticoid (GC) monotherapy for the treatment of immunoglobulin G4-related disease (IgG4-RD). PubMed, Web of Science, Scopus, OVID, and the Cochrane Library were searched for related articles. Meta-analysis was conducted with outcomes including relapse rate, remission, and adverse events. We calculated the odds ratio (ORs) and 95% confidence interval (CI) with the meta-analysis model. Ten studies involving 906 patients were included in the systematic review; of them, seven studies were included in the meta-analysis. The effect size showed that the GC group was associated with a higher relapse rate (OR = 2.97, 95% CI [1.91, 4.62], p < 0.0001) and a less complete remission rate (OR = 0.27, 95% CI [0.16, 0.47], p < 0.0001) than the combination of GC and IM group. While there was no significant difference between the two compared groups in terms of adverse events (OR = 0.73, 95% CI [0.44, 1.21], p = 0.22). No significant heterogeneity was detected regarding all outcomes (p > 0.1, I2 < 50%). Treatment of IgG4-RD patients with a combination of GC and IM was associated with higher remission rates, lower relapse rates, and comparable safety profiles. Larger RCTs should be conducted and focused on exploring the genetic and geographic differences between different cohorts.