Roles of IgG4 and IgG4/IgG ratio to IgG4-related disease in patients with elevated serum IgG4 level.

OBJECTIVE

To evaluate the performance of elevated serum IgG4 and IgG4/IgG in IgG4-related disease (IgG4-RD) and other diseases.

METHODS

Seven hundred seventy-three patients with elevated serum IgG4 level (> 2.01 g/L) were reviewed in Zhongda Hospital of Southeast University from 1 July 2016 to 31 December 2021. Demographic, disease distribution and the role of elevated serum IgG4 and IgG4/IgG in IgG4-RD and other diseases were analysed. The alteration of IgG4 and IgG4/IgG in pre-therapy and post-treatment were also assessed in IgG4-RD.

RESULTS

Patients with elevated serum IgG4 were principally observed in older males. Chronic diseases of various organs (21.7%), rheumatic immune diseases (19.4%), bacterial infection disease (11.5%) and malignant tumor (5.2%) were the common diseases with elevated serum IgG4, but only 3.2% was IgG4-RD. The level of IgG4 and IgG4/IgG in IgG4-RD was significantly higher than that in various diseases except for eosinophilia group. Serum IgG4 and IgG4/IgG manifested a similar diagnostic capacity for IgG4-RD among this study cohort and the optimal cut-off values were 3.345 g/L and 0.295 respectively. The sensitivity and specificity were 96% and 71% for the optimal cut-off value of IgG4, and 80% and 88.8% for the optimal cut-off value of IgG4/IgG4. IgG4 and IgG4/IgG both were remarkably reduced in IgG4-RD after therapy compared with prior treatment (P < 0.05).

CONCLUSIONS

Elevated serum IgG4 was found in a variety of diseases, especially in chronic diseases of various organs. IgG4 and IgG4/IgG manifest a great value for IgG4-RD diagnosis, and are available for the treatment evaluation of IgG4-RD. Key Points • Elevated serum IgG4 level was not a specific marker to IgG4-related disease and can be observed in various diseases. • Patients with IgG4-related disease or eosinophilia manifest a higher level of serum IgG4 and IgG4/IgG. • Both of IgG4 and IgG4/IgG are available for the diagnosis and the clinical treatment evaluation of IgG4-related disease.