Purpose: Immunoglobulin G4-related disease (IgG4-RD) share clinical features with primary Sjögren's syndrome (pSS). This study aimed to identify altered serological parameters and potential biomarkers of IgG4-RD and pSS.

Methods: Forty IgG4-RD patients, 40 pSS patients, and 40 healthy controls (HC) were enrolled in this study. Routine serological parameters and clinical manifestations were assessed. IgG subclasses (IgGSc) were detected using a Siemens BN P, and lymphocyte subsets were analyzed using flow cytometry. Cytokines assays were performed using cytometric bead array.

Results: Compared to pSS, IgG4-RD patients had higher IgG4 ( <0.001) and lower IgG1 ( =0.014). The natural killer (NK) cells ( = 0.004), CD4+ T cells ( = 0.028), and TBNK cells ( = 0.040) were increased in IgG4-RD compared to pSS. IgG4 used to differentiate IgG4-RD from pSS produced an area under the curve (AUC) of up to 0.952. In addition, we compared serum parameters, immune cells, and cytokines of IgG4-RDwith mouth dryness or eye dryness with those of pSS with the same symptoms, and similar serological changes were observed. IgG4-RD patients with mouth dryness had higher IgG4 ( <0.001) and Th cells ( = 0.016) but lower IgG1 ( = 0.009) compared to pSS with dry mouth. IgG4-RD patients with eye dryness had higher levels of IgG4 ( <0.001), Treg cells ( = 0.037), and NK cells ( = 0.017) than pSS patients with eye dryness. Moreover, IgG4-RD patients with mouth and eye dryness had higher levels of B ( = 0.006), Th ( = 0.026), Th2 ( = 0.007), and Treg cells ( = 0.028) than IgG4-RD patients without mouth and eye dryness.

Conclusion: Immune system disorder is an outstanding feature of IgG4-RD, and its feature differ from pSS. Assessment of immune status is important in the diagnosis and differential diagnosis of IgG4-RD.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11770921PMC
http://dx.doi.org/10.2147/JIR.S471266DOI Listing

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