Orbital and Adnexal Sarcoidosis

Abstract

To present the clinical features and management in a series of patients with orbital and adnexal sarcoidosis. This multicenter retrospective study included patients with biopsy-proven noncaseating granuloma involving the orbit or adnexa and evidence of systemic sarcoidosis. Clinical records were reviewed for initial examination findings, radiological findings, treatment modalities, and outcome. The study included 26 patients (19 female, 7 male; mean age, 52 years). The most common feature at the first examination was a palpable periocular mass followed by discomfort, proptosis, ptosis, dry eye, diplopia, and decreased vision. The disease affected the lacrimal gland (42.3%), orbit (38.5%), eyelid (11.5%), and lacrimal sac (7.7%). Among orbital lesions, the antero-inferior quadrant was preferentially involved. Treatment modalities included steroids, surgical debulking, and methotrexate. During a mean follow-up of 18.75 months, 84.6% of patients showed a complete response to the treatment, but 19.2% of patients developed further signs of sarcoidosis. Orbital soft tissue involvement is more common in patients older than 50 years and in women. The anterior inferior quadrants of the orbits appear to be preferentially affected. Although a good response to treatment with oral steroids is seen, long-term follow-up is recommended because active systemic disease can develop months to years later.