Long-Term Follow-Up in IgG4-Related Ophthalmic Disease: Serum IgG4 Levels and Their Clinical Relevance

Abstract

(1) Background: To analyze the association between long-term changes in serum IgG4 levels and the clinical course of patients with IgG4-related ophthalmic disease (IgG4-ROD). (2) Methods: Retrospective analysis of 25 patients with IgG4-ROD. (3) Results: Mean age at diagnosis was 60.68 years. Fifty-six percent of patients had bilateral ocular involvement and 32% had systemic associations. The ocular structures involved were the lacrimal gland (76%), orbital soft tissue (36%), extraocular muscle (20%) and infraorbital nerve (20%). According to last follow-up, 9 (36%) patients had normalized IgG4 levels, and 16 (64%) patients had elevated IgG4 levels. Patients with normalized IgG4 levels had better response to initial steroid treatment and attained a significantly lower IgG4 level after treatment (p = 0.002). The highest IgG4 levels were at baseline and disease recurrence, and lowest after initial treatment. At final follow-up, IgG4 levels differed in patients with remission (mean 326.25 mg/dL) and stable disease (mean 699.55 mg/dL). Subgroup analysis was performed in patients with remission, categorized according to whether IgG4 levels were normalized (9 patients) or elevated (10 patients) on last follow up. The elevated group had a higher percentage of bilateral disease, lacrimal gland involvement and recurrence. (4) Conclusions: IgG4-ROD patients with a greater response to initial steroid therapy were more inclined to have normalized IgG4 levels in the long term. Some patients remained in remission despite persistently elevated IgG4 levels, and had regular follow-up without treatment.

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

“…Chou et al [32] believed that serum IgG4 was a response to inflammatory stimulus, more likely a consequence than a cause of the disease, and patients with IgG4-ROD had a favorable outcome, regardless of their serologic status in the long-term. Serum IgG4 was not found to be a factor affecting LGBLEL recurrence in this study, which was inconsistent with previous results indicating that IgG4 was an influencing factor for IgG4-ROD recurrence.…”

The Challenges and Therapeutic Prospects in Eye Disease

“…Serial changes in serum IgG4 levels is a useful adjunct diagnostic method in the assessment of disease activity in patients with IgG4-ROD [ 4 ]. Chou et al further investigated the relationship between the long-term changes in serum IgG4 levels and the clinical course of patients with IgG4-ROD [ 5 ]. They found 64% patients with IgG4-ROD had persistently high serum IgG4 levels during long-term follow-up.…”

“…Importantly, their study disclosed 40% of patients with IgG4-ROD remained in remission despite persistently elevated serum IgG4 levels. Chou et al suggest these patients can be followed-up without treatment unless disease relapse occurs [ 5 ].…”