Orbital and adnexal involvement in sarcoidosis: analysis of clinical features and systemic disease in 30 cases.

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

DESIGN: Observational, retrospective case series.

METHODS: 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.

RESULTS: 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.

CONCLUSIONS: 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.