Clinical rheumatology · 2025
IgG4-related disease with comorbid autoimmune diseases: a retrospective study from a large cohort.
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Yang Xinli, Nie Yuxie, Li Jingna, Peng Yu, Zhang Jialei, Zhang Nianyi, Wang Yifei, Jiang Jialing, Luo Qinhuan, Wang Mu, Peng Linyi, Fei Yunyun, Li Mengtao, Zhou Jiaxin, Zhang Wen
Аннотация
OBJECTIVES: This study aimed to explore the clinical characteristics, treatment, and prognosis of IgG4-related disease (IgG4-RD) patients with comorbid autoimmune diseases (AIDs).
METHOD: A total of 879 IgG4-RD patients treatment-naïve at baseline from Peking Union Medical College Hospital were retrospectively included in this study. IgG4-RD patients with comorbid AIDs were classified into the AID-positive (AID-Pos) group, while those without AIDs were classified into the AID-negative (AID-Neg) group. Additionally, AIDs were classified as systemic or organ-specific subgroups. The clinical features, laboratory findings, treatment regimens, and long-term prognosis were compared among the AID-Pos and AID-Neg groups, and the characteristics of AIDs were also explored.
RESULTS: AID prevalence in IgG4-RD was 9.67% (85/879), with systemic (n = 47; 55.3%) and organ-specific (n = 38; 44.7%) subtypes. Common AIDs included vitiligo (n = 15; 17.6%), systemic vasculitis (n = 12; 14.1%), psoriasis (n = 12; 14.1%), Hashimoto's thyroiditis (n = 11; 12.9%), and rheumatoid arthritis (n = 10; 11.8%). Most preceded/synchronized with IgG4-RD; 88.2% had a single AID. All groups showed a median of 3 involved organs, but AID-Neg had more multi-organ involvement. AID-Pos had lower IgG4 levels, while systemic AIDs showed higher ESR and IgG1. Treatment and prognosis did not differ between groups.
CONCLUSIONS: IgG4-RD patients can complicate with other AIDs. Compared with the AID-Neg group, IgG4-RD patients with comorbid AIDs contained a lower proportion of patients with multi-organ involvement, and their patients have lower serum IgG4 levels. The treatment and prognosis were similar between the two groups. Key Points • IgG4-related disease (IgG4-RD) can complicate with autoimmune diseases (AIDs). The relationship between IgG4-RD and AID is yet to be fully elucidated. • 9.67% of IgG4-RD patients (85/879) had comorbid AIDs. AID-positive patients showed lower IgG4, and patients with systemic AIDs showed higher ESR and IgG1. • IgG4-RD and AIDs are not entirely contrary in the diagnosis of IgG4-RD.
METHOD: A total of 879 IgG4-RD patients treatment-naïve at baseline from Peking Union Medical College Hospital were retrospectively included in this study. IgG4-RD patients with comorbid AIDs were classified into the AID-positive (AID-Pos) group, while those without AIDs were classified into the AID-negative (AID-Neg) group. Additionally, AIDs were classified as systemic or organ-specific subgroups. The clinical features, laboratory findings, treatment regimens, and long-term prognosis were compared among the AID-Pos and AID-Neg groups, and the characteristics of AIDs were also explored.
RESULTS: AID prevalence in IgG4-RD was 9.67% (85/879), with systemic (n = 47; 55.3%) and organ-specific (n = 38; 44.7%) subtypes. Common AIDs included vitiligo (n = 15; 17.6%), systemic vasculitis (n = 12; 14.1%), psoriasis (n = 12; 14.1%), Hashimoto's thyroiditis (n = 11; 12.9%), and rheumatoid arthritis (n = 10; 11.8%). Most preceded/synchronized with IgG4-RD; 88.2% had a single AID. All groups showed a median of 3 involved organs, but AID-Neg had more multi-organ involvement. AID-Pos had lower IgG4 levels, while systemic AIDs showed higher ESR and IgG1. Treatment and prognosis did not differ between groups.
CONCLUSIONS: IgG4-RD patients can complicate with other AIDs. Compared with the AID-Neg group, IgG4-RD patients with comorbid AIDs contained a lower proportion of patients with multi-organ involvement, and their patients have lower serum IgG4 levels. The treatment and prognosis were similar between the two groups. Key Points • IgG4-related disease (IgG4-RD) can complicate with autoimmune diseases (AIDs). The relationship between IgG4-RD and AID is yet to be fully elucidated. • 9.67% of IgG4-RD patients (85/879) had comorbid AIDs. AID-positive patients showed lower IgG4, and patients with systemic AIDs showed higher ESR and IgG1. • IgG4-RD and AIDs are not entirely contrary in the diagnosis of IgG4-RD.