Management of chronic ocular sarcoidosis: challenges and solutions

Abstract

BackgroundSarcoidosis constitutes one of the leading causes of ocular inflammation. Chronic ocular sarcoidosis can affect any segment of the eye and its adnexa, producing a wide range of clinical manifestations and severity. If left untreated, permanent visual impairment or even blindness may ensue. Treatment approaches vary from topical therapy to systemic agents that induce immunosuppression to different levels according to disease severity.ObjectiveTo review the published literature on the management options for chronic ocular sarcoidosis and provide a comprehensive list of available treatment strategies, including the newer biologics.SummaryOcular disease remains a challenging aspect of sarcoidosis and may even be the presenting sign of the disease. Prompt and effective therapy may reverse visual damage and prevent permanent loss of vision. Because of the complexity of the disease, a multidisciplinary approach is often required, with a view to addressing both the ocular and other systemic manifestations of sarcoidosis. Recent data suggest that achieving overall optimal systemic control is of paramount importance in controlling eye inflammation as well. Cytotoxic immunosuppressive agents for refractory chronic ocular disease, as well as biologic anti-TNFα therapies, have advanced the management of chronic disease and should be considered corticosteroid-sparing strategies before the onset of significant steroid-induced morbidity.

Ocular sarcoidosis: clinical experience and recent pathogenetic and therapeutic advancements

“…Any segment of the eye and its adnexa may be affected, but uveitis is the most frequently diagnosed condition [ 11 – 13 , 39 ]. The prevalence of uveitis as the initial presenting complaint of SS ranges from 20–30% [ 14 , 16 ] to almost 80% [ 11 ], depending on the population studied and the length of ophthalmic follow-up. Conversely, 30–60% [ 13 ] and up to 80% [ 12 , 15 , 16 ] of patients with SS may develop ophthalmic disease at some point during the course of the disease.…”

“…5 ), plays a central role in the initiation and amplification of the inflammatory response. In addition to TNF-α inhibitors, a steadily growing list of other biologic agents, such as lymphocyte inhibitors and specific receptor antagonists, are being tested in patients with non-infectious uveitis, including sarcoidosis, both in observational case series and in non-randomized off-label studies [ 16 , 50 ].…”

“…Although all segments of the eye and its adnexa can be involved, the most common sarcoidosis-related ocular disease is uveitis that may result in permanent visual impairment if not timely diagnosed and properly treated [ 14 – 16 ]. However, across the entire spectrum of uveitides (caused by several systemic diseases), the proportion of patients with sarcoid uveitis is variably reported as 2.4% [ 17 ], 3% [ 18 ], 6.4% [ 19 ], and up to 15% [ 20 ], again reflecting ethnicity, geography, and diagnostic challenges.…”

<p>Refractory Sarcoidosis: A Review</p>

“…61 Subconjunctival injections of dexamethasone, triamcinolone acetate, or betamethasone may be used in case of persistence of uveitis despite topical CS therapy. 62 Posterior sub-tenon and peribulbar injections of triamcinolone acetate permit direct administration of steroids. Alternatively, intravitreal injections of steroids can be suggested to treat unilateral macular edema or when it resists systemic therapy and periocular injections.…”

Noninfectious uveitis in the Asia–Pacific region

“…Two recent pivotal multinational phase 3 trials established the effectiveness of adalimumab (a fully humanized monoclonal antibody) in treating noninfectious uveitis. In VISUAL 1 study, 18 subjects with active sarcoid uveitis responded to adalimumab, with a 50% reduction in experiencing treatment failure than the placebo group [3,84]. In VISUAL 2 trial, 229 inactive uveitis cases (including 32 cases with sarcoidosis) were investigated.…”