Ann Lab Med.  2023 Sep;43(5):461-469. 10.3343/alm.2023.43.5.461.

Clinical Significance of Serum IgG4 in the Diagnosis and Treatment Response of IgG4-Related Disease in Adults of Southwest China: A Retrospective Study

Affiliations
  • 1Department of Laboratory Medicine/Clinical Research Center of Laboratory Medicine, West China Hospital, Sichuan University, Chengdu, China
  • 2West China Second University Hospital, West China Women’s and Children’s Hospital, Key Laboratory of Birth Defects and Related Diseases of Women and Children, Sichuan University, Chengdu, China

Abstract

Background
There is no standard cut-off value of serum IgG4 concentration and serum IgG4/total IgG ratio for the diagnosis of IgG4-related disease (IgG4-RD) or as a marker of treatment responses. We aimed to explore this issue through a retrospective cohort analysis of adults in southwest China.
Methods
The diagnostic performance of serum IgG4 concentration and IgG4/IgG ratio for IgG4-RD was evaluated in a retrospective analysis of 177 adults newly diagnosed as having IgG4-RD and 877 adults without IgG4-RD. Dynamic analysis was performed to evaluate the significance of serum IgG4 concentration on IgG4-RD treatment responses.
Results
The serum IgG4 concentration differed according to sex. The optimal cut-off values of serum IgG4 concentration and IgG4/IgG ratio for IgG4-RD diagnosis were 1.92 g/L and 0.12 in males and 1.83 g/L and 0.11 in females, respectively. For patients with serum IgG4 concentration >2.01 g/L, the cut-off values in the total population were >3.00 g/L and 0.19, respectively. The median serum IgG4 concentration decreased over time, and the decrease rate increased over time. The serum IgG4 concentration significantly decreased at >1 week post-treatment (P=0.004), and the median decrease rate was close to 50% at >4 weeks post-treatment.
Conclusions
Serum IgG4 can be a good indicator for IgG4-RD diagnosis; however, different diagnostic cut-off values should be determined according to sex. The decreasing rate is more conducive than the serum IgG4 concentration to monitor treatment efficacy. The IgG4/IgG ratio did not improve the diagnostic efficacy for IgG4-RD.

Keyword

Immunoglobulin G4; IgG4-Related Disease; Diagnosis; Treatment; China

Figure

  • Fig. 1 Violin plot showing the serum IgG4 concentrations of IgG4-related disease patients according to the number of involved organs. The tops and bottoms of the violin plots represent the minimum and maximum, respectively. The bold dotted line in the middle represents the median, and the dotted lines on either side represent the interquartile range covering 50% of the values. **P<0.01. Abbreviation: IgG4-RD, IgG4-related disease.

  • Fig. 2 Dynamic analysis of serum IgG4 concentration (A-C) and the decrease rate of serum IgG4 concentration post-treatment (D) in IgG4-RD patients treated with glucocorticoids. *P<0.05, **P<0.01, and ***P<0.001. 0: pre-treatment; [0, 1]: 0 weeks


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