The Role of Symptom Duration and Serologic Factors in the Relapse of IgG4-Related Ophthalmic Disease following Surgery: A Retrospective Cohort Study

Abstract

IgG4-related disease (IgG4-RD) affects multiple organs and is characterized by immune-mediated inflammation and fibrosis; IgG-RD affecting orbital tissue is known as IgG4-related ophthalmic disease (IgG4-ROD). This research is aimed at exploring whether symptom duration and common serologic factors, such as IgG, IgE, and eosinophils, are potential risk factors for IgG4-ROD patient relapse after surgery and identifying possible causes of the positive correlation between symptom duration and relapse. This retrospective cohort study included 40 IgG4-ROD patients after surgery. Auxiliary inspection results were obtained before surgery and during follow-up, and relapse risk factors were identified based on previous studies. We used the Spearman rank correlation test to reveal the relationship between symptom duration and relapse time and identified the optimal cutoff value for symptom duration by X-tile. Then, we divided the patients into the long-duration and short-duration groups. Kaplan–Meier survival analyses and log-rank tests were performed to identify the relationship between symptom duration and relapse using X-tile software. Finally, we studied the relationship between previously studied relapse risk factors and symptom duration. The survival curves of the long-duration and short-duration groups were obviously different, and the baseline serum IgG, IgE, and eosinophil levels and asthma concomitant rate were significantly different between the long-duration and short-duration groups. Furthermore, the baseline serum IgG ( r = 0.485 , P = 0.002 ), IgE ( r = 0.350 , P = 0.037 ), and eosinophil ( r = 0.6535 , P < 0.0001 ) levels were positively correlated with symptom duration. Our study shows that IgG4-ROD symptom duration is significantly positively correlated with relapse rate and negatively correlated with relapse time. Symptom duration was positively correlated with serum baseline IgG4, IgE, and eosinophil levels and asthma history, which were potential risk factors for disease relapse. We recommended that IgG4-ROD patients with symptom durations greater than 96 months continue to receive maintenance steroid therapy longer than 1 year postsurgery to reduce the relapse rate.

Different serum levels of IgG and complements and recurrence rates in IgG4-positive and negative lacrimal gland benign lymphoepithelial lesion

“…Previous studies had shown that serum IgG4, RF, and male gender were risk factors for recurrence of IgG4-ROD treated with glucocorticoid therapy [28][29][30] . Liu et al [31] found that IgG4-ROD symptom duration was significantly positively correlated with relapse rate. Our study showed that gender, extraocular muscle thickening, nerve thickening, sinusitis, IgG4 and RF were not risk factors for recurrence of LGBLEL.…”

Efficacy and safety of intravenous glucocorticoid therapy for IgG4-related ophthalmic disease

“…Serological factors, including high serum IgG4 level, elevated serum IgE, and elevated eosinophils, are known to be associated with IgG4-ROD relapse [ 7 , 40 ]. In our study, serum IgG4 levels before initial steroid therapy were significantly higher in the IV group than in the oral group.…”

Risk factors for IgG4‐related disease relapse: A systematic review and meta‐analysis

“…Patients with high serum indexes are more sensitive to the immune response, which makes the disease more likely to relapse. Furthermore, Th2 cells can secrete specific cytokines, such as IL-13, IL-5 and IL-4, to cause allergy and immune abnormalities, 48 and the presence of allergies can increase the number of circulating Th2 memory cells, which increases atopy and enhances Th2 responses, such as increasing serum IgG, IgE and eosinophil levels, 49 in IgG4-RD. 48,50 High multiple organ involvement and proximal bile duct stenosis rates might reflect the severity and activity of IgG4-RD, which helps to predict the relapse of the disease.…”