Sarcoidosis-Related Uveitis: A Review

Abstract

Sarcoidosis is an inflammatory disease that involves the eyes in 10-55% of cases, sometimes without systemic involvement. All eye structures can be affected, but uveitis is the most common ocular manifestation and causes vision loss. The typical ophthalmological appearance of these uveitis is granulomatous (in cases with anterior involvement), which are usually bilateral and with synechiae. Posterior involvement includes vitritis, vasculitis and choroidal lesions. Tuberculosis is a classic differential diagnosis to be wary of, especially in people who have spent time in endemic areas. The diagnosis is based on histology with the presence of non-caseating epithelioid granulomas. However, due to the technical difficulty and yield of biopsies, the diagnosis of ocular sarcoidosis is often based on clinico-radiological features. The international criteria for the diagnosis of ocular sarcoidosis have recently been revised. Corticosteroids remain the first-line treatment for sarcoidosis, but up to 30% of patients require high doses, justifying the use of corticosteroid-sparing treatments. In these cases, immunosuppressive treatments such as methotrexate may be introduced. More recent biotherapies such as anti-TNF are also very effective (as they are in other non-infectious uveitis etiologies).

Keywords: granuloma; ocular sarcoidosis; sarcoidosis; uveitis.

Figure 1

Acute granulomatous anterior uveitis with active retrodescemetic precipitates (white arrows).

Figure 2

Central and peripheral bilateral multifocal choroiditis.

Figure 3

Cystoid macular edema in spectral domain by optical coherence tomography.

Figure 4

Revised diagnostic criteria for ocular sarcoidosis (OS) as recommended by the “International Workshop on Ocular Sarcoidosis (IWOS)”, adapted from [51]. Abbreviations: BHL: bilateral hilar lymphadenopathy; ACE: angiotensin converting enzyme; OS: ocular sarcoidosis.

Figure 5

Diagnostic algorithm for suspected ocular sarcoidosis adapted from [74,123]. Abbreviations: ACE: angiotensin-converting enzyme; BAL: bronchoalveolar lavage; EBUS: endoscopic ultrasound-guided fine-needle aspiration of intrathoracic nodes; IGRA: interferon-γ release assay; MSGB: minor salivary-gland biopsy; OS: ocular sarcoidosis; 18F-FDG PET: 18-fluorodeoxyglucose positron emission tomography.

Figure 6

Therapeutic management in ocular sarcoidosis adapted from [123]. Abbreviations: ON: optic neuritis; ME: macular edema; ORV: occlusive retinal vasculitis; MTX: methotrexate; AZA: azathioprine; MMF: mycophenolate mofetil; LFN: leflunomide; RTX: rituximab; TCZ: tocilizumab; JAKi: Janus kinase inhibitor; IV: intravenous.