Sarcoid-like granulomatous orbitopathy—presentation, systemic involvement and clinical outcome

Abstract

Aim: To describe patients with sarcoid-like granulomatous orbitopathy (SLGO), the rate of known and subsequent systemic sarcoidosis, and the treatment and outcome for the condition. Patients and methods: Retrospective review of patients with SLGO presenting between 1990 and 2018, excluding solely lid or lacrimal drainage disease. Results: Sixty-one patients (45 female; 74%) were identified, 54 having biopsy-proven sarcoidosis (47 orbital, 24 prior extra-orbital), and half were Afro-Caribbean. The average age at presentation was 45.3 years (range 18–78 years), with the commonest symptoms being swelling, pain and diplopia; of clinical signs, most patients (87%) had eyelid swelling, reduced motility (25%), and disease-related visual impairment (10%). Localized dacryoadenitis was present in 49/61 (21/49 bilateral) patients, and more diffuse disease in 28/61 (8/28 bilateral). Systemic involvement was found in 23 (62%) of the 37 first presenting with orbital disease. Twenty-three (38%) patients were observed and two-thirds received oral corticosteroids, with 53/61 (87%) patients having subjective and objective clinical improvement. The average follow-up was 53.4 months (range 1–315 months) and clinical recurrence occurred in 12 (20%) patients at 26.5 months (range 1–115 months) after first diagnosis. Conclusions: Systemic sarcoidosis may be discovered in about two-thirds of patients presenting with SLGO (that tends to present with inflammatory features), but the treatment response appears similar in patients with known sarcoidosis and those with newly-diagnosed systemic disease after orbital presentation. With long-term follow-up, a third of patients have spontaneous regression of orbital disease, but 20% have recurrence after reducing or stopping systemic immunosuppression.