Steroids remain first-line treatment for pediatric IgG4-RD, but combination therapies show promise

Systemic steroids remain the primary first-line treatment for pediatric IgG4-related disease, but combination therapies—including immunosuppressants, biologics like rituximab, and surgery for localized cases—are often necessary for effective disease management, according to a study.

Researchers analyzed 81 studies, encompassing 114 pediatric cases, to assess treatment patterns and outcomes. They found that 57% of patients had multi-organ involvement, often necessitating multiple therapies. Steroids were the most used treatment, prescribed to about 75% of patients either alone or in combination. Although most responded well to therapy, approximately 30% experienced relapses, frequently during steroid tapering.

Immunosuppressants, particularly azathioprine and mycophenolate mofetil, were commonly used as steroid-sparing agents. Rituximab was the primary biologic for severe or refractory cases, while surgery was employed for localized disease. Less common but effective treatments included adalimumab and ruxolitinib.

The study also found that older children, particularly those over 10, were more likely to receive aggressive treatments such as surgery and rituximab and had higher relapse rates. Female patients also exhibited a greater likelihood of relapse.

Reference
Sapountzi E, Kotanidou EP, Tsinopoulou VR, et al. The Management of IgG4-Related Disease in Children: A Systematic Review. Children (Basel). 2025;12(2):213. doi: 10.3390/children12020213. PMID: 40003315; PMCID: PMC11854391.