Sarcoid Orbital Myopathy: Clinical Presentation and Outcomes.

PURPOSE

To describe the clinical manifestations, systemic associations, radiological features, and treatment outcome for patients presenting with sarcoid-like granulomatous disease involving the extraocular muscles.

STUDY DESIGN

Retrospective noncomparative case series and literature review.

METHODS

Patients presenting with presumed sarcoid orbital myopathy between 1990 and 2016 were classified either as having known systemic sarcoidosis at time of orbital presentation (Group I) or as having newly diagnosed sarcoidosis (Group II).

RESULTS

Twenty patients (15 females; 75%) were identified, 8 having a known systemic sarcoidosis and 12 with a new diagnosis. The average age at presentation was 53.7 years (median 54.5; range 31-78), with the commonest symptoms being swelling, pain, and diplopia. The superior rectus/levator complex was most commonly affected (50%), followed by lateral rectus (40%) and the superior oblique only rarely (2 in Group II), but with no evident bias between the groups. Most patients had additional involvement of lacrimal gland (70%) and orbital fat (95%), and systemic disease was present in 18 of 20 patients. Four patients were on oral corticosteroids prior to orbital presentation (2 in each group) and 16 received oral prednisolone after orbital diagnosis-14 commencing steroids and 2 continued previous steroids. Clinical improvement was noted in 13 of 20 (65%) patients, the results being similar in the 2 groups (p = 1.0). The average follow up was 56 months (median 41; range 1-315), and disease recurrence occurred in 6 patients (30%) at an average interval of 12.5 months after diagnosis.

CONCLUSIONS

This study presents the long-term results of patients with sarcoid-related orbital myopathy and systemic disease may be found in over 80% of newly diagnosed cases. The superior rectus/levator complex and lateral rectus are most commonly involved-possibly due to spill-over inflammation from granulomatous dacryoadenitis. Approximately two-thirds of cases respond well to systemic corticosteroids, while one-third develop recurrent disease.Sarcoid orbital myopathy is rare and mainly affects the superior rectus/levator complex and lateral rectus, possibly due to neighboring dacryoadenitis. Most settle with systemic corticosteroids, a quarter need second-line immunosuppressants, and one-third will have relapse.