Conditions

What is serpiginous choroiditis?

Serpiginous choroiditis (SC) is a rare, bilateral, chronic, recurrent inflammatory disorder, affecting the posterior part of the eye. The disease is more frequent in middle aged healthy men. SC is limited to the eye and no systemic disease associations have been reported so far.

The disease classically manifests with yellowish lesions involving the deep retina and choroid, typically located in the area around the optic nerve (peripapillary region).

Active lesions spread centrifugally from the optic nerve towards the periphery of the eye in a serpentine manner, producing the typical ‘serpiginoid’ appearance that gave the disease its name.

Inflammation can involve both the anterior part of the eye and the vitreous (the jelly-like structure which fills the posterior cavity of the eye) manifesting as vitritis, both usually mild.

Healed lesions result in loss of tissue (chorioretinal atrophy) and hyperpigmentation.
The disease is usually bilateral.

However, multiple lesions in different stages of activity can be detected in both eyes.

Consecutive recurrences cause further tissue atrophy irregularly spreading at the back of the eye.

What causes serpiginous choroiditis?

Although of unknown etiology, SC is an inflammatory condition limited to the eye driven by an abnormal immune response that occurs in genetically predisposed individuals.

What are the symptoms?

Symptoms include a progressive, painless, decrease in vision, manifesting as

Diagnosis

There are no diagnostic laboratory tests and the clinical presentation of SC is usually sufficient to confirm the diagnosis.

Dye tests, including fundus fluorescein angiography and indocyanine green angiography, and fundus autofluorescence, a non-invasive ocular imaging technique, are carried out to confirm the diagnosis, detect the inflammatory activity, monitor the course of the disease and its response to treatment.

Retinal function can be measured by special tests, including visual fields and electrodiagnostic tests.

Treatment

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

The treatments are not curative but aim at controlling the immune response which is the reason for the inflammatory reaction 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 the steroids.

Prognosis

The clinical course of the disease is prolonged and insidious with multiple recurrences. If not well controlled, SC can result in long term damage because of the loss of chorioretinal tissue. In case of foveal involvement visual outcome is generally significantly affected.

Abnormal vessels growing under the retina (choroidal neovascularization) is a common complication that can affect the quality of vision.