BACKGROUND

Immunoglobulin G4-related disease (IgG4-RD) is a rare, chronic inflammatory condition characterized by fibrosis and tendency for multi-organ involvement. This study aims to analyze the clinical characteristics associated with multi-organ versus single-organ involvement in IgG4-RD, thereby enhancing clinicians' understanding of the differences between these two patient groups and ultimately improving patient prognosis.

METHODS

We performed a retrospective analysis of clinical data from 82 patients diagnosed with IgG4-RD admitted to Yichang Central People's Hospital between January 2019 and December 2024.

RESULTS

Among the 82 patients diagnosed with IgG4-RD, 47 patients (57.32%) exhibited involvement of multiple organs. The incidence of multi-organ involvement was significantly higher in male patients than female patients [63.49% vs. 36.84%, odds ratio (OR): 2.98, 95% confidence intervals (CI): 1.03-8.64, <0.05]. The misdiagnosis rate in the multi-organ involvement group was significantly higher than that in the single-organ involvement group (29.79% vs. 8.57%, OR: 4.525, 95% CI: 1.19-17.26, <0.05). In patients with involvement of the pancreas (72.50% vs. 42.86%, OR: 3.515, 95% CI: 1.39-8.86, <0.05), or lymph nodes (83.72% vs. 28.21%, OR: 13.091, 95% CI: 4.50-38.11, <0.05), the incidence of additional organ involvement was significantly higher than those with involvement of other organs. The eosinophil percentage [median difference (Hodges-Lehmann): 1.60%, 95% CI: 0.40-2.80, <0.05], absolute eosinophil count [median difference (Hodges-Lehmann): 0.10×10/L , 95% CI: 0.30-0.16, <0.05], serum immunoglobulin G (IgG) levels [median difference (Hodges-Lehmann): 4.10 g/L, 95% CI: 0.10-7.80, <0.05], and erythrocyte sedimentation rate (ESR) [median difference (Hodges-Lehmann): 30.50 mm/h, 95% CI: 13.00-48.00, <0.05] were significantly higher in the multi-organ involvement group compared to the single-organ involvement group. There was a positive correlation between the number of involved organs and ESR (=0.404, 95% CI: 0.166-0.597, =0.001), eosinophil percentage (=0.287, 95% CI: 0.068-0.480, =0.009), absolute eosinophil count (=0.293, 95% CI: 0.075-0.485, =0.007), serum IgG levels (=0.370, 95% CI: 0.130-0.570, =0.003), and serum IgG4 levels (=0.370, 95% CI: 0.130-0.570, =0.003).

CONCLUSION

The clinical features associated with multi-organ involvement in IgG4-RD are characterized by significant diversity and complexity. Clinicians must enhance their understanding of the characteristics associated with multi-organ involvement to more effectively improve patient prognosis.