From Symptoms to Specialist Care in IgG4-Related Ophthalmic Disease: A Retrospective Case Series

Objectives IgG4-related ophthalmic disease (IgG4-ROD) is a complex, immune-mediated condition with a broad spectrum of clinical presentations involving the lacrimal glands, orbital tissues, extraocular muscles, and cranial nerves.[1] It is part of the wider spectrum of IgG4-related disease (IgG4-RD), which is characterized by fibroinflammatory lesions, IgG4-positive plasma cell infiltration, storiform fibrosis, and, in many cases, elevated serum IgG4 levels.[1,2] Given the disease’s heterogeneity and its overlap with other inflammatory or neoplastic conditions, timely diagnosis and effective management can be challenging. Through a systems-level approach, we examine how patients move through diagnostic and referral pathways within the Canadian healthcare model. By mapping these pathways and identifying delays or gaps in coordination, we aim to inform improvements in care delivery, streamline referral processes, and support earlier diagnosis and intervention. This approach is critical to optimizing outcomes and ensuring equitable access to subspecialty care across a publicly funded system with finite resources. Methods In this retrospective, observational case series, we performed a chart review at the University of British Columbia of 180 IgG4-RD patients. Research Ethics Board approval was obtained. Demographic data, IgG4 serum levels, diagnostic and referral pathways, clinical manifestations and pathological findings were retrieved from patients diagnosed with IgG4-ROD between the Jan 1, 2010 - June 1, 2025, all managed under a unified diagnostic and pathological framework. Descriptive analysis was performed on Excel (Microsoft, Redmond, WA). Results Eight representative patients with IgG4-ROD were included in this analysis from a cohort of 51 identified cases. Four patients (50%) had acute inflammatory flare-ups preceding the onset of chronic symptoms later attributed to IgG4-ROD. The median time from chronic symptom onset to expert subspecialty assessment was 15.5 months (range 1-23 months). Patients had a median of 6.5 specialist encounters, 1 diagnostic test panel, 2 biopsies, and 2 diagnostic imaging studies before a definitive diagnosis was established. Conclusion This study represents the first health-systems analysis of patients with IgG4-ROD in a Canadian context. We observed prolonged intervals before expert care and a high number of specialist encounters along the diagnostic pathway. In a publicly funded system already burdened by long wait times, these findings highlight the need to streamline triage and referral pathways for IgG4-ROD to enable more efficient, coordinated care. References [1.] Ko J. Ophthalmology 2025;132:995-1004. [2.] Stone J. N Engl J Med 2012;366:539-51.