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A large multicenter study found that surgical debulking and steroid tolerability significantly improved remission in IgG4-related ophthalmic disease, while high serum IgG4 levels and diabetes were linked to increased relapse risk, highlighting the need for long-term management strategies.

Awareness of IgG4-related ophthalmic disease (IgG4-ROD) is growing, but lacking is comprehensive, large-scale studies that integrate clinical, pathological, and treatment-related data. Most existing research is based on data from a limited number of hospitals and does not offer a nationwide perspective.

This led Jae Sang Ko, MD, PhD, and colleagues to conduct a nationwide multicenter study in Korea to investigate the clinical characteristics, sex- and laterally-specific variations, patterns of orbital and systemic involvement, and factors influencing treatment outcomes, including remission and relapse.

The findings reported in Ophthalmology highlighted several crucial aspects of IgG4-ROD, which specifically affects the orbital and ocular adnexal structures.

The multicenter, retrospective case series study included 341 patients with IgG4-ROD from 24 hospitals. The researchers reviewed the medical records of all the patients with IgG4-ROD, which were consistent with all comprehensive diagnostic criteria. Additionally demographic, clinical, histopathological, and laboratory data were collected.

Of the 341 patients enrolled, 155 had a definite diagnosis of IgG4-ROD. Extra-lacrimal gland involvement was seen in 53 (34.2%) patients and associated with older age, male sex, unilaterality, and higher serum IgG4 levels. A significant (67.5%) proportion of patients exhibited extraorbital manifestations who underwent adequate systemic evaluation. The most frequently affected extraorbital organs were the lungs (27.3%), salivary glands (20.8%), and paranasal sinuses (18.2%). Extraorbital involvement did not show significant differences in clinical characteristics.

Regarding treatment outcomes, 130 patients with IgG4-ROD and followed for more than 6 months were included in the analysis. Remission was achieved in 71.5% of patients; however, a significant portion experienced relapses, highlighting the importance of adequate immunomodulatory therapy and careful long-term monitoring for effective disease management. Therefore, the development of novel biomarkers and therapeutic agents based on a better understanding of disease pathogenesis is needed, the researchers explained.

They noted that one important finding was that surgical debunking was strongly associated with positive treatment outcomes, specifically related to achieving remission and steroid-free remission (HR, 6.95; 95% CI, 2.86-16.86 and HR, 28.88; 95% CI, 9.78-85.33, respectively).

The highest serum IgG4 tertile was negatively associated with steroid-free remission. Relapse occurred in 28.6% of patients who experienced remission, and diabetes was an associated risk factor (HR, 4.24; 95% CI, 1.49-12.07).

“These findings suggest that surgical debulking and patient tolerability to steroid therapy may be critical factors in determining the treatment outcomes for IgG4-ROD,” the authors concluded.