Noninfective Chorioretinitis

Noninfective chorioretinitis is an inflammatory condition affecting the back of the eye that can seriously threaten your vision if left untreated. Unlike infections, this condition stems from the body’s own immune system or other non-infectious causes.

Table of contents

• What Is Noninfective Chorioretinitis?
• Parts of the Eye Affected
• Causes of Noninfective Chorioretinitis
• Signs and Symptoms
• How It Is Diagnosed
• Treatment Options
• Possible Complications

What Is Noninfective Chorioretinitis?

Noninfective chorioretinitis is a form of uveitis (inflammation inside the eye) that affects the back portion of the eye, specifically the choroid and retina. The term “uveitis” originally referred to inflammation of the uveal tract, which includes the iris, ciliary body, and choroid, but it can also involve nearby structures like the retina, blood vessels in the retina, the vitreous (the clear gel filling the eye), the optic nerve head, and the sclera (the white part of the eye)^[1].

Chorioretinitis is classified as posterior uveitis based on where the inflammation occurs in the eye. The choroid is a layer rich in blood vessels that sits between the retina and the sclera. Because the choroid provides vital blood supply and nutrients to the outer layers of the retina, inflammation in these areas can lead to serious vision problems^[1].

When the condition is specifically noninfective, it means the inflammation is not caused by bacteria, viruses, parasites, or fungi, but rather by other factors such as autoimmune diseases or unknown causes^[1].

• Choroid
• Retina
• Vitreous
• Optic nerve

Parts of the Eye Affected

Your choroid is a layer of eye tissue filled with blood vessels. It is located between your retina and sclera. The sclera is the strong tissue that you might call “the white of your eye.” You can lose vision if you have problems with the choroid because it provides your retina with essential nutrients^[2].

The retina is the light-sensitive layer at the back of your eye that is responsible for vision. When inflammation affects both the choroid and retina together, it creates the condition known as chorioretinitis^[2].

Causes of Noninfective Chorioretinitis

Unlike infectious chorioretinitis (which is caused by germs), noninfective chorioretinitis has different underlying causes. These can be grouped into those with connections to other body-wide conditions and those without^[1].

Noninfective causes linked to other diseases in the body include:

• Sarcoidosis – a disease that causes inflammation in various body tissues
• Behçet’s disease – a disorder that causes blood vessel inflammation throughout the body
• Rheumatoid arthritis – an autoimmune condition affecting joints and other organs

These are examples where the body’s immune system mistakenly attacks its own tissues, including those in the eye^[2][8].

Noninfective causes without connections to other body-wide conditions include:

• Multifocal choroiditis and panuveitis
• Punctate inner choroidopathy – a condition causing small spots of inflammation in the choroid
• Multiple evanescent white dots syndrome
• Unilateral acute idiopathic maculopathy
• Birdshot choroidopathy – a specific pattern of inflammation in the choroid
• Serpiginous choroidopathy – inflammation that spreads in a snake-like pattern

In these conditions, the inflammation occurs without a clear connection to other diseases in the body^[1].

Other causes may include:

• Eye injuries or trauma
• Some types of cancer, like lymphoma
• Some genetic conditions
• Conditions like Sympathetic ophthalmia and Vogt Koyanagi Harada syndrome, which involve inflammation as part of a more widespread eye condition

Sometimes the inflammation can occur for no apparent reason, and in these cases it is called idiopathic, meaning the cause is unknown^[1][2].

Signs and Symptoms

The symptoms of noninfective chorioretinitis can vary in severity. Common warning signs include^[2][7]:

• Seeing black spots, floaters, or flashes of light in your vision
• Red eyes
• Blurred vision that may get progressively worse
• Eye pain or discomfort
• Sensitivity to light (photophobia) – when bright light causes discomfort or pain
• Excessive eye watering or tearing
• Difficulty seeing colors clearly
• Impaired night vision
• Distortion of objects – things may appear wavy or bent

If you experience any of these symptoms, especially blurred vision, eye pain, or sensitivity to light, you should contact an eye care professional as soon as possible^[2].

How It Is Diagnosed

An eye care provider will perform several tests to diagnose noninfective chorioretinitis and rule out infectious causes^[2].

The main diagnostic steps include:

A dilated eye exam is the primary way to see the retina and choroid. Your provider will give you eye drops to make your pupils wider (dilate) so they can see all the way to the back of your eye. Sometimes, providers can see chorioretinal scars, which are pieces of tissue or patches of color at the back of your eye that indicate past or ongoing inflammation^[2].

Additional tests may include:

• Lab tests on material swabbed from your eye or blood tests. These tests help rule out infections and identify underlying conditions that might be causing the inflammation^[2].
• Optical coherence tomography (OCT) – an imaging test that uses reflected light to take detailed pictures of the back of your eye. This helps see the different layers of the retina and detect any swelling or damage^[2].
• Fluorescein angiography – a test where a special dye is injected into your arm, and pictures are taken as the dye travels through the blood vessels at the back of your eye. This helps your provider see the blood vessels more clearly^[2].
• Indocyanine green (ICG) angiography – similar to fluorescein angiography, but this imaging test specifically lets your provider see the blood vessels of the choroid, the layer under your retina^[2].

If you have another condition that might be causing the chorioretinitis, such as an autoimmune disease, your provider might order additional tests to investigate those conditions^[2].

Treatment Options

Eye doctors, called ophthalmologists, treat most cases of noninfective chorioretinitis with medications designed to reduce inflammation and prevent damage to your vision^[2][9].

The main treatment approaches include:

Corticosteroids are the primary treatment for reducing inflammation. These can be given as eye drops, pills, or injections directly into the eye. Steroids work by suppressing the immune system’s inflammatory response. However, you cannot take steroids for extended periods because of potential side effects^[2][9].

For longer-term treatment, your eye care provider may work with a rheumatologist (a doctor who specializes in immune system disorders) to prescribe steroid-sparing medications. These are drugs that suppress your immune system but allow you to reduce or stop steroid use. Examples include medications like azathioprine, cyclosporine, tacrolimus, and mycophenolic acid^[2][11].

In cases of severe noninfective chorioretinitis that do not respond well to standard treatments, biologic agents may be used. These are newer medications that target specific parts of the immune system. Examples include anti-TNF-alpha antibodies, anti-IL-6 antibodies, or rituximab^[11].

Additional supportive treatments may include:

• Dilating drops to prevent the iris from sticking to the lens underneath and to reduce discomfort^[7]
• Medications to manage any underlying autoimmune disease that may be contributing to the eye inflammation^[7]

The specific treatment plan will depend on the severity of your condition, how well you respond to treatment, and whether you have any other health conditions. The goal is to control inflammation, preserve your vision, and minimize side effects from the medications^[9].

Possible Complications

If left untreated, noninfective chorioretinitis can lead to serious complications. The most significant concern is vision loss, which can become permanent if the inflammation causes lasting damage to the retina or choroid^[2].

When the inflammation is severe enough to cause actual harm to your eye structures, the condition may progress to chorioretinopathy. The suffix “-pathy” refers to damage or disease of the tissue. At this stage, the inflammation has caused structural changes that may affect your vision permanently^[2].

To help reduce your chance of complications, it is important to have regular eye exams, especially if you experience eye pain or vision problems. If you have any autoimmune diseases, monitoring and properly treating those conditions can also help prevent eye inflammation. Early detection and prompt treatment are critical to preventing permanent vision loss^[7].