Conditions

What is sarcoidosis?

Sarcoidosis is a rare multisystem inflammatory disease that results in inflammatory nodules called granulomas in the affected organs , including the eye. The onset is typically in the second to fourth decade of life.

Signs and symptoms depend on the parts of the body affected by the disease. 

Patients with sarcoidosis may feel tired and fatigued, lose weight, or suffer with fevers and night sweats.

The lungs and lymph nodes in the chest are the sites most commonly involved, and patients can manifest cough or feel breathless.

However, some people do not have any symptoms and the disease is incidentally diagnosed after a routine chest X-ray.

Ocular involvement occurs in about 30–60% of the patients and usually manifests as bilateral uveitis, affecting the anterior and/or the posterior part of the eye. Ocular manifestations include:
In addition to ocular findings, other common manifestation of the disease include:

What causes sarcoidosis?

Sarcoidosis is an inflammatory condition presumably driven by an abnormal immune response that occurs in genetically predisposed individuals. It’s possible that environmental factors, including infections and chemicals may trigger the disorder. Signs and symptoms of the disease are due to the inflammation throughout the body and the changes in function of the affected organs.

Sarcoidosis cannot be passed on to other people or from parent to child, but genetic factors may increase the likelihood of a person developing the condition. Environmental factors may be important and occurrence of the disease in family members is more likely to be connected to exposure to these factors.

What are the symptoms of ocular disease?

Sarcoidosis is an inflammatory condition presumably driven by an abnormal immune response that occurs in genetically predisposed individuals.

It’s possible that environmental factors, including infections and chemicals may trigger the disorder. Signs and symptoms of the disease are due to the inflammation throughout the body and the changes in function of the affected organs.

Sarcoidosis cannot be passed on to other people or from parent to child, but genetic factors may increase the likelihood of a person developing the condition.

Environmental factors may be important and occurrence of the disease in family members is more likely to be connected to exposure to these factors.

What are the symptoms of ocular disease?

Symptoms include

Diagnosis

In the presence of ocular manifestations suggestive of sarcoidosis, blood tests are usually ordered to support the diagnosis and rule out other inflammatory diseases.

A marker called angiotensin-converting enzyme (ACE) is measured in the blood since it may sometimes be raised in patients with sarcoidosis. Other blood tests include markers of inflammation, kidney and liver function and calcium levels. Chest X-ray allows assessment of the lungs and involved chest lymph nodes. Other imaging scans, including CT scan or PET CT scan can be performed to look for other parts of the body affected by inflammation. However, the gold standard to make a definite diagnosis of the disease is a sample of tissue (biopsy), taken from one of the areas of inflammation, which will confirm the presence of the classical granuloma.

In patients with ocular manifestations, fluorescein and indocyanine angiography are usually performed to detect inflammation of the retinal vessels and inflammatory lesions in the choroid, to monitor the course of the disease and its complications and to evaluate the response to treatment.

Treatment

The intention of therapy is to control the inflammation and prevent the damage to the ocular tissues , and consequently the visual loss.

The treatments are not curative but aim at controlling the immune response which is the reason for the inflammation in the eye.

Options include the use of oral medications, usually starting with systemic steroids to induce rapid response.

In view of the nature of the disease, the addition of immunosuppressive drugs is important to allow long term control and minimize the side effects of systemic steroids. Local therapy is an option for controlling ocular disease and includes the use of steroid devices (Ozurdex and Iluvien).

Complications and Prognosis

If not well controlled, ocular sarcoidosis can result in long term damage because of the chronic inflammation leading to macular oedema and loss of retinal tissue.

Other common complications include glaucoma, cataract and epiretinal membrane. All these complications can affect the visual function.

Anterior uveitis with the presence of deposits on the back of the cornea (granulomatous keratic precipitates) and nodules of the iris.
Optos colour image showing retinal vasculitis.
Optos colour image showing multifocal peripheral small punched-out chorioretinal scars.

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