Uveitis

What is uveitis?

Uveitis refers to a group of inflammatory diseases that cause swelling of the middle layer of the eye and destroy eye tissue. These disorders can permanently damage the eye, leading to vision loss or, in rare cases, blindness.

The eye has three different layers of tissue. While uveitis refers to inflammation of the middle layer, or the uvea, inflammation can also affect other parts of the eye. The innermost layer is the retina, which senses light and helps send images to the brain. The outermost layer is made up of the sclera and cornea, the strong white wall of the eye.

The uvea contains many blood vessels, veins, arteries and capillaries that carry blood to and from the eye. When the eye becomes inflamed, different structures can be damaged, including the lens (cataract), retina or optic nerve (from increased pressure or inflammation). In addition, blood supply can interrupted. Together — if left untreated — these could lead to permanent damage to your child’s sight.

Types of uveitis

There are four different types of uveitis. Each is defined by the part of the eye in which it occurs.

  • Iritis, also called anterior uveitis, affects the front of the eye and causes inflammation in the watery liquid in the front of the eye and the iris. It is the most common type of uveitis. Iritis may develop suddenly and last six to eight weeks. However, in many cases, iritis can become a chronic condition or episodes may be recurrent.
  • Intermediate uveitis, sometimes called pars planitis, involves inflammation of the middle part of the eye, including the clear gelatin like substance (vitreous).
  • Choroiditis, also called posterior uveitis, is an inflammation in the back of the eye. It often develops slowly and can last for years.
  • When more than one layer of the eye is inflamed, it is called panuveitis.

Signs and symptoms

It is important to note that in some children with uveitis, there are no outward symptoms of the disorder.

In particular, patients with juvenile idiopathic arthritis (JIA) often have no symptoms and require routine screenings every 3 to 12 months to monitor for development of uveitis.

In other cases, signs and symptoms of uveitis can include:

  • Light sensitivity
  • Blurred vision
  • Eye pain
  • Redness of the eye
  • Floating spots in the field of vision

If your child’s eye becomes red or painful and does not clear up quickly, she should be examined by an ophthalmologist, a medical doctor who has special training to care for eyes. The ophthalmologist can determine if your child has uveitis or another condition.

Causes

Uveitis is caused by inflammatory responses inside the eye. The inflammation is the body’s natural response to germs, toxins, or tissue damage. It produces redness, swelling and heat, and destroys tissue as white blood cells try to contain the infection.

The inflammation present in uveitis may be caused by:

  • Infections, including bacterial infections (such as Lyme disease or tuberculosis), viral infections (such as herpes or Epstein–Barr virus) and in rare cases, fungal and parasitic infections
  • Related autoimmune disease in other parts of the body such as juvenile idiopathic arthritis, vasculitis or inflammatory bowel disease
  • Injury or trauma to the eye; in some cases one eye can get inflamed because of severe injury to the opposite eye (sympathetic uveitis)

In approximately half of cases, the cause of uveitis remains unknown.

Testing and diagnosis

A careful eye examination by an eye doctor is extremely important when symptoms occur. Inflammation inside the eye can permanently affect sight — and even cause blindness — if not treated.

Your child’s ophthalmologist will examine the inside of your child’s eye using a special lens or light, and may order additional tests to help make a diagnosis. These tests may include:

  • Blood tests
  • Skin tests
  • X-rays
  • Eye imaging, such as optical coherence tomography (OCT), a non-invasive imaging test that uses light waves take cross-section pictures of the retina

Since uveitis can be associated with several diseases, the eye doctor will want to know about your child’s overall health. The ophthalmologist may consult with your child’s primary care physician or other medical specialists.

Treatment

Uveitis is a serious eye condition that may scar your child’s eye. It needs to be treated as soon as possible.

Eye drops, especially steroids and pupil dilators, can help relax the eye, reduce inflammation and relieve pain. Some eye drops may increase your child’s sensitivity to light, so wearing sunglasses is recommended.

For more severe or persistent inflammation, oral medication, injections, or infusions may be necessary. Your child’s ophthalmologist will discuss the best treatment options for your child.

Because uveitis is often linked with inflammatory problems in other parts of the body — such as juvenile idiopathic arthritis and inflammatory bowel disease — your child may also be referred to additional specialists for evaluation and treatment.

If your child is referred outside of CHOP for a uveitis evaluation, please ask the eye doctor to complete the Uveitis Assessment Form and bring it with you to your child’s appointment at CHOP. You can also upload it using MyCHOP or fax it to 215-590-4750.

At The Children’s Hospital of Philadelphia, children with uveitis are cared for by both a rheumatologist and an ophthalmologist through the Uveitis Coordinated Care Clinic. The specialists work together to develop a coordinated treatment plan for your child that can help control your child’s eye inflammation sooner and result in better outcomes.

In some cases, uveitis can lead to other serious eye conditions, including glaucoma, cataracts, neovascularization (the growth of new, abnormal blood vessels) and damage to the retina. These complications may also need treatment, in the form of eye drops, conventional surgery or laser surgery.

Follow-up care

Children or adolescents who are treated for uveitis may be followed by an ophthalmologist, a rheumatologist, or both. When the inflammation of your child’s eye is active, she may be examined as often as every other week. Once the inflammation is under control, she may be asked to come in for follow-up appointments every three to six months to make sure there are no changes in her condition.

Follow-up care is important to proactively adjust your child’s treatment plan if needed.

Outlook

Uveitis is a serious medical condition. Left untreated, it can lead to vision loss, cataracts, glaucoma, macular edema and retinal complications. With early diagnosis and treatment — along with ongoing monitoring — your child will experience fewer complications and improved vision.