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To present the clinical features and management in a series of patients with orbital and adnexal sarcoidosis. Methods: 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. Results: 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 anteroinferior 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. Conclusions: 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.

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