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.

Highlights

  • IgG4-related disease (IgG4-RD) is an immune-mediated inflammatory disorder with fibrosis that can affect multiple organs, and the affected organs can have tumor-like lesions and even failure [1]

  • We found that the levels of serum IgE, IgG, and eosinophils were significantly different between the long-duration group and short-duration group, which means that patients with a long duration of symptoms have higher baseline serum IgE, IgG, and eosinophil levels

  • Our study provides novel information about risk factors for IgG4ROD relapse and found that the duration of symptoms of IgG4-ROD has a significant positive correlation with relapse of the disease

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Summary

Introduction

IgG4-related disease (IgG4-RD) is an immune-mediated inflammatory disorder with fibrosis that can affect multiple organs, and the affected organs can have tumor-like lesions and even failure [1]. IgG-RD that affects orbital tissue is known as IgG4related ophthalmic disease (IgG4-ROD). The statement called for all active IgG4-RD and IgG4-ROD patients to receive treatment, and the first-line drug for remission induction is glucocorticoids. How to maintain treatment to avoid relapse is still an urgent problem to be solved, and a better understanding of risk factors for relapse plays an important role in disease management and relapse monitoring. Due to the heterogeneity and rarity of the disease, there are still few studies on the influencing factors of IgG-ROD relapse, especially in patients who have undergone surgical treatment

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