Risk factors for extraophthalmic involvement and treatment outcomes in patients with IgG4-related ophthalmic disease

Background/aim To analyse the clinical risk factors for extraophthalmic involvement and treatment outcomes in patients with IgG4-related ophthalmic disease (IgG4-ROD) in Korea. Methods We retrospectively reviewed medical records of 42 patients diagnosed with IgG4-ROD confirmed by a surgical biopsy during the 7-year period, between January 2009 and March 2015, at three tertiary referral centres in Korea. Data regarding patients' demographics, clinical characteristics and radiological findings were obtained. The relapse rate was compared between systemic steroids only and combined immunosuppressant therapy. Results The mean patient age was 55.2 years (male:female ratio, 1:1). Based on the primary orbital structure affected, the IgG4-RODs were classified as dacryoadenitis (52.4%), orbital soft tissue inflammation (21.4%), trigeminal nerve involvement (11.9%) and myositis (9.5%). Extraophthalmic involvement (n=21) was associated with bilateral involvement (p=0.004), longer symptom duration (p=0.033) and a higher serum IgG4 level (p=0.034). Initial treatment regimens included attentive observation (n=7), prednisolone alone (n=22) and prednisolone plus immunosuppressive agent (n=13). During follow-up (mean, 24 months), 13 patients (37.1%) experienced relapse. In the extraophthalmic involvement group, steroid therapy alone resulted in a significantly higher relapse rate than combined immunosuppressant treatment (p=0.028). Conclusion Bilateral involvement, longer symptom duration and higher IgG4 levels were significant risk factors for extraophthalmic involvement in Korean patients with IgG4-ROD. In patients with an extraophthalmic involvement, prednisolone combined with an immunosuppressive agent was more effective for preventing recurrence than prednisolone alone.