Orbital and Adnexal Sarcoidosis

Abstract

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.

Orbital inflammatory disease management

“…In larger case series, Mavrikakis et al, Demirci et al, and Prabhakaran et al reported partial or complete response to systemic corticosteroids in at least 95% of their patients with orbital and adnexal sarcoid [161][162][163], and recurrence rates were noted to be less than 10% [161,163].…”

“…Ophthalmic sarcoidosis occurs frequently, and about 10% of these cases involve the orbit and ocular adnexa [160], with the lacrimal gland and orbit being the most frequently involved [161].…”

“…Authors have discussed periocular steroid injections [161][162][163] and debulking surgery with concurrent systemic steroid therapy and found both to be effective for local control [161]. Minimal literature exists on the use of biologic and antibody therapy in sarcoid confined to the orbit.…”

The role of inflammation and antiinflammation therapies in keratoconjunctivitis sicca

“…31 It as been documented that tear fi lm defi ciencies are common in sarcoidosis, even though clinical symptoms are present. In the same study, it was emphasized that defi ciency of mucin and lipid layers may also accompany aqueous layer defi ciency.…”

Sarcoidosis Presenting as Scleral Nodule

“…3 However, ocular manifestations of sarcoidosis may comprise a variety of clinical findings, including anterior, intermediate, or posterior uveitis, optic neuropathy, adnexal and orbital disease, scleral involvement, glaucoma, and cataracts. 4 The lacrimal gland is the most common adnexal site affected, 5 which can occur prior to other manifestations of the disease, 6 and may lead to subsequent sicca syndrome. Scleral involvement is infrequently reported in the literature, particularly in the absence of other ocular involvement.…”