[Chinese guidelines for the treatment and evaluation of IgG4-related disease (2026 edition)]

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated, chronic fibroinflammatory disease characterized by multisystem involvement. As it is relatively rare and has been recognized for a short time, standardized treatment and disease assessment remain clinical challenges. In recent years, substantial progress has been made both internationally and domestically in the diagnosis, treatment, and management of IgG4-RD, with more evidence-based studies now available in this field. However, there is no Chinese guideline focusing on the treatment and evaluation of systemic involvement in IgG4-RD. To address this gap, the Chinese Rheumatology Association and the IgG4-Related Disease Group, Committee of Rheumatology and Immunology, Cross-Strait Medicine Exchange Association jointly organized experts from multiple disciplines, including rheumatology, gastroenterology, ophthalmology, stomatology, pulmonology, nephrology and urology, to systematically review and evaluate the latest domestic and international literature. In combination with clinical practice in China, and following the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) evidence grading system, the experts addressed 9 clinical questions of concern prioritized by frontline clinicians in China and formulated 18 evidence-based recommendations. The guideline aims to improve the standardized treatment and evaluation of IgG4-RD, promote daily clinical care, and ultimately improve patient outcomes.