Ocular sarcoidosis

doi:10.1016/j.lpm.2012.04.004

La Presse Médicale

Volume 41, Issue 6, Part 2, June 2012, Pages e349-e354

https://doi.org/10.1016/j.lpm.2012.04.004 Get rights and content

Summary

Sarcoidosis, a chronic multisystem disease, is a common cause of ocular inflammation. Even though clinical features are well-established, diagnosis requires histological confirmation, which remains difficult in patients with uveitis. Thus, the frequency of ocular sarcoidosis is overestimated. A set of criteria has been recently established in order to improve the diagnostic procedure. New imaging tools will enable the ophthalmologist to evaluate the level of ocular inflammation and to monitor its resolution after treatment initiation. Indocyanine green angiography and optical coherence tomography have dramatically improved our understanding of choroidal granulomas and macular edema. Treatment is based on topical and systemic corticosteroids in most of the cases, but immuno-suppressive agents may be necessary. The visual outcome remains favorable but severe complications, including glaucoma, cystoid macular edema and choroidal neovascularization, may need a prompt and aggressive management.

Section snippets

Epidemiology

The prevalence in the Western world is approximately 20 per 100,000 (although in some countries it is higher), and 25–50% of patients may have ocular involvement at some time during the course of the disease. This large range may be due to the fact that a proportion of these cases are not biopsy-proven [9], [10], [11]. In a study published in 2007, the clinical features of 81 consecutive patients with biopsy-proven sarcoidosis, referred to a tertiary eye care center over a 16-year period, were

Diagnostic features

Histopathologic proof remains the gold standard for the diagnosis of sarcoidosis. However, biopsy of ocular tissue is not easily available, especially in patients presenting with isolated ocular involvement. Moreover, despite promising data that were recently published, ocular fluids do not help in establishing the final diagnosis or even in providing a possible orientation [15].

Lacrymal gland infiltration has been noted in 30% of cases. It is often bilateral and may be associated with parotid

New diagnostic criteria for ocular sarcoidosis

The first international workshop on ocular sarcoidosis (IWOS) to attempt to establish international diagnostic criteria for ocular sarcoidosis was held on October 28–29, 2006, in Tokyo, Japan by members of an international study group consisting of uveitis specialists and pulmonologists from Asia, Africa, Europe, and America. The goal of this set of criteria is to enable the ophthalmologist to suspect the diagnosis without further invasive investigations. The criteria consist of seven clinical

Differential diagnosis

In the absence of strong evidence for ocular sarcoidosis, differential diagnosis must be considered and some important entities should be excluded, depending on the anatomic site of inflammation. The first major step is to consider an infectious condition. In the face of a bilateral ocular inflammation with granulomatous features, ocular tuberculosis is the main etiology to rule out. Past medical history, tuberculin skin test, chest X-ray and, more recently, the IGRA tests will be performed in

Complications

Anterior uveitis is usually chronic and may be diagnosed late in the course of the disease. The rate of complications depends on the diagnostic delay. Band keratopathy is a corneal opacity, which occurs in 5 to 10% of cases. Posterior synechiae may induce seclusion with iris bombé, pupillary block and a rapid increase of intraocular pressure requiring a surgical iridectomy. Secondary glaucoma is observed in up to one third of the patients. Cataract may happen with the same frequency. Degree and

Therapeutic strategy

The conventional therapy of extra- and intraocular sarcoidosis is based on topical and/or systemic corticosteroids. The literature on the medical treatment of intraocular inflammation (uveitis) is littered with case reports, uncontrolled studies, and small case series [23]. Topical steroids are only helpful in mild presentations of anterior uveitis. Mydriatic agents are useful to prevent synechiae formation. In most of the cases, ocular involvement remains isolated and systemic therapy becomes

Ocular prognosis

Sarcoid uveitis has usually a favorable outcome if detected early and treated adequately. Anterior cases are responding well to topical agents but may need long-term therapy. Posterior uveitis may be more difficult to control and induce sight-threatening complications such as degenerative macular edema and choroidal neovascularization. Severe visual loss may occur in up to 24% of cases with permanent blindness or visual handicap in 10% of cases.

Illustrations are available in Figure 1, Figure 2.

Disclosure of interest

the authors declare that they have no conflicts of interest concerning this article.

Glossary

BHL: bilateral hilar lymphadenopathy
IWOS: international workshop on ocular sarcoidosis
KPs: Keratic precipitates
NPV: negative predictive value
OCT: optical coherence tomography
OS: ocular sarcoidosis
PAS: peripheral anterior synechiae
PPV: positive predictive value
TM: Trabecular meshwork

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