Ocular Sarcoidosis – A Comprehensive View and Newfound Hope

Ocular sarcoidosis is a manifestation of sarcoidosis, which is an inflammation of the body tissues that affects lungs, lymph nodes and other organs. Ocular sarcoidosis develops in around 30% of the sarcoidosis patients, although in Japan the percentage is much higher. This inflammation can be chronic, and if left untreated it leads to a permanent impairment of the visual system and even to a complete vision loss.

Sarcoidosis granulomas formed in the eyes, are not unlike granulomas that form in the rest of the areas affected by sarcoidosis. They form when the immune system tries to wall off an infection or a foreign substance, and they appear as solitary, yellow nodules.

Ocular sarcoidosis manifests mainly as uveitis (swelling and irritation of the middle layer of the eye called uvea) but it can also affect other parts of eyes. Anterior or posterior granulomatous uveitis is most common, and may be accompanied by conjunctival and scleral lesions.

Types of uveitis found in ocular sarcoidosis patients include:

• Anterior uveitis may cause pain and excessive sensitivity to light, but some patients don’t experience any symptoms. Chronic anterior uveitis left untreated may be the cause of glaucoma, band keratopathy (corneal disease) and cataract.
• Intermediate uveitis is another type of uveitis indicative of sarcoidosis. It is characterized by inflammation in the vitreous (thick, transparent substance that fills the center of the eye), pars plana (layer of the uvea), or peripheral retina.
• Posterior uveitis affects the back of the uvea called the choroid. This causes an inflammation called choroiditis, which may spread and affect other areas of the eye.

All types of uveitis may lead to a disease by the name of cystoid macular edema (swelling of the macula, which is a small, highly sensitive central area of the retina).

Ocular sarcoidosis may also manifest as other eye conditions like: optic neuropathy, which is a rare complication of sarcoidosis that leads to vision loss; and orbital disease, which mainly affects lacrimal glands, and may lead to eye entrapment and diplopia. Lacrimal gland disease is a common symptom and may appear before all the other signs of sarcoidosis.

When sarcoidosis is suspected, the patient should receive a detailed examination of the eyes, especially if the eye problems are noted.

Ocular sarcoidosis may be treated in different ways including topical therapy, immunosuppressive therapy and methotrexate (a commonly used steroid-sparing agent). Topical corticosteroids are used if the inflammation is mild, while in more severe cases steroids may be injected, for a rapid improvement. After the use of corticosteroids, reoccurrence of the disease is possible, while immunosuppressive therapy has more long term effects.

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