To evaluate clinical features, management, and prognosis of biopsy-proven orbital and adnexal sarcoidosis.

Observational, retrospective case series.

Clinical features and treatment methods were evaluated retrospectively in 30 consecutive patients managed in 1 center. Data from 11 patients without systemic disease at presentation were analyzed for their impact on occurrence of systemic sarcoidosis using univariate and multivariate regression models.

Orbital and adnexal involvement included the lacrimal gland in 63% (19/30), the eyelid in 17% (5/30), the orbit in 13% (4/30), and the lacrimal sac in 7% (2/30). Thirty-seven percent (11/30) had known systemic disease. Of 63% (19/30) of cases with only orbital disease, systemic disease initially was found in 27% (8/30), subsequently in 7% (2/30), and never in 30% (9/30). On computed tomography scans, the lesions were well circumscribed in 85% (25/30) and diffuse in 15% (5/30). Management included systemic steroids in 50% (15/30) of cases with symptomatic disease, followed by excision in 27% (8/30) of cases and observation in 23% (7/30) of cases with asymptomatic disease. After a mean follow-up of 44 months, all cases showed regression or remained stable, but 7% (1/30) of cases treated with systemic steroids demonstrated recurrence. Using Kaplan-Meier estimates, systemic sarcoidosis developed in 8% at 5 years. In univariate or multivariate analysis, no clinical feature was found to be significantly predictive of systemic sarcoidosis.

In our series, in patients with orbital and adnexal sarcoidosis alone, systemic disease developed in 8% by 5 years. Systemic steroids were effective for patients with symptomatic disease.