Abstract
Purpose :
Existing studies on IgG4-related orbital disease (IgG4-ROD) epidemiology are largely limited to Japanese populations and literature supporting specific IgG4-ROD treatment regimens is sparse. Its diagnosis is also challenging due to conditions that can present similarly (orbital lymphoma, sarcoidosis). We performed a retrospective, observational clinical study to examine the associations between the demographic characteristics, diagnostic findings, and treatment outcomes of patients with IgG4-ROD.
Methods :
A review of IgG4-ROD patients at the University of Florida spanning 1973-2023 identified 14 cases out of 95 potential patients ascertained through relevant ICD codes for orbital myositis, orbital inflammation, and eye swelling/mass. Collected data included demographic factors (age, gender, history of smoking, malignancy, infection), diagnostic (biopsy, radiological, serological) findings and treatment outcomes (remission status, adverse effects). Descriptive statistics were conducted using REDCap; further statistical analysis to be conducted by a statistician.
Results :
The average age of patients was 43 (SD 15.71) and 78.6% n:11 were female. 93% n:13 had no history of malignancy, 71% n:10 never smoked and 29% n:4 had a history of chronic infection (sinusitis). All patients had unilateral onset of symptoms. Common symptoms included periorbital swelling (50%, n:7), diplopia (36%, n:5), and eye pain (29%, n:4). 29% n:4 had extraorbital manifestations (sinus/salivary gland). Imaging findings revealed extraocular muscle (85% n:11) and lacrimal gland (23% n:3) enlargement. Pathological findings of 4 patients were variable (only 1 patient with IgG4-positive cells). 9 patients had a baseline serum IgG4. Primary treatment was oral glucocorticoids with 29% n:4 on chronic steroid use for 3-6 months. 14% n:2 were treated with disease-modifying antirheumatic drugs (DMARDs), rituximab or methotrexate. 54% n:7 relapsed, including 1 on DMARDs, with 39% n:5 achieving remission.
Conclusions :
This study provides demographic information, diagnostic findings, and treatment outcomes for IgG4-ROD patients seen at one institution over 50 years. Ambiguous findings such as the baseline serum IgG4 level, lack of positive biopsy results, and underutilization of steroid-sparing agents emphasize the challenge of diagnosing IgG4-ROD. Further statistical analyses are ongoing.
This abstract was presented at the 2024 ARVO Annual Meeting, held in Seattle, WA, May 5-9, 2024.