Faq Uvea Service

Which part of the eye is the Uvea?

The Uvea is the middle layer of the eyeball. The eye is made of three layers. The outermost is the sclera and cornea which keep the contents intact and maintain its shape. The inner layer is the retina which is highly sophisticated neural tissue and forms the image that is sent to the brain via the optic nerve. The middle layer is the uvea and is responsible for the ‘housekeeping’ of the eye. It carries the blood vessels, nerves, pigment cells and immune cells. It extends from the front to the back of the eye and at different parts is called differently. In the front is the Iris and anterior part of the ciliary body (anterior uvea), in the middle is the posterior part of the ciliary body (pars plana) (intermediate uvea) and posteriorly it is called the choroid.

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What is Uveitis?

Uveitis is inflammation of any part of the uvea and is classified by the part of uvea that is inflamed.

Anterior Uveitis affects the front of the eye. It is often called iritis because it mainly affects the area around the eye’s iris. Anterior uveitis is the most common kind of uveitis making up 40-70% of all uveitis. It is usually acute (i.e. comes on suddenly and lasts for less than six weeks) and is associated with pain, light sensitivity and redness.
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Anterior uveitis with large collection of cells and a distorted pupil.
Intermediate Uveitis is inflammation of the ciliary body, the front end of the retina, and the vitreous. Intermediate uveitis is the least common type of uveitis, making up only 7-15% of cases. It is also known as cyclitis, pars planitis or vitritis. In most of the cases, the cause is unknown. Symptoms include floaters and blurry vision. People with intermediate uveitis are more likely to have chronic inflammation. Chronic uveitis is defined as uveitis lasting longer than six weeks.

Posterior Uveitis is inflammation of the choroid and retina.

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Scars in the choroid as a result of healed posterior uveitis

Panuveitis is inflammation of all the parts of the uvea and is a serious disorder.

What are the symptoms of Uveitis?

The symptoms depend on the location of the uveitis. Anterior uveitis usually presents as a painful, red eye with mild blurring of vision. (Exceptions include Juvenile rheumatoid arthritis associated anterior uveitis). In intermediate and posterior uveitis, the eye is not so painful but the patient sees multiple small floaters (like a dust storm) with or without blurring or severe loss of vision.

What Causes Uveitis?

Uveitis is inflammation of the uvea. Inflammation is the body’s response to injury, or trauma. To treat uveitis, doctors look for the cause of the trauma to the eye however in the majority of cases a cause (as many as a third or half of all uveitis) cannot be found. It is then believed that there may be an abnormal triggering of the body’s immune system causing the uveitis (autoimmune diseases).

Amongst the known causes uveitis can be caused by many different kinds of trauma, including a virus, bacteria infections, or parasites. Genes can play an important role in uveitis. Also, diseases that damage the body’s immune system, such as AIDS, can lower the body’s ability to protect itself from infections that can cause uveitis.

Causes of Anterior Uveitis
In more than a third of all cases of anterior uveitis, the exact cause is unknown. Causes may include:

Ankylosing Spondylitis is a type of arthritis that mainly affects the spine. This can cause recurrent arthritis and prompt referral to orthopedic specialist can prevent permanent changes.

Reiter’s Syndrome is an inflammatory arthritis common in young men – often caused by Chlamydia infection.

Psoriatic Arthropathy is a form of arthritis that nearly a quarter of people with psoriasis may develop. It causes pain, swelling and stiffness in joints. Psoriasis is an immune-related disease of the skin and joints.

Inflammatory Bowel Disease causes inflammation, ulcers and scarring to the wall of the intestine but can affect other parts of the body as well. Juvenile Rheumatoid Arthritis (JRA) is a kind of arthritis (inflammation of the joints) in children. Uveitis is the most common eye problem that can develop in children with JRA. Children with JRA may get uveitis before JRA is even diagnosed, or it can occur many years later. It may remain asymptomatic and all such children should be screened for uveitis 6 monthly.

Herpes is a common viral infection that causes oral or genital herpes. It mostly affects only one eye, but it can cause recurrent uveitis.

Sarcoidosis is a kind of inflammation that can affect many parts of the body. Its cause is unknown.

Fuch’s Heterochromic Iridocyclitis is a chronic kind of iritis. Its cause is unknown.

Lupus is a chronic inflammatory disease that affects different parts of the body, including the skin, joints, blood, and kidneys and is related to the body’s immune system.

Intraocular lens surgically implanted to replace a cataract lens. Uveitis can be caused by trauma related to cataract surgery. In cataract surgery, the eye’s natural lens is removed and replaced with an artificial (plastic) lens. The presence of this artificial lens can cause anterior uveitis.

Posner-Schlossman Syndrome (PSS) is an uncommon inflammatory eye condition that usually affects one eye at a time. It typically affects young to middle-aged adults who develop high pressure inside the eye and mild inflammation.

Rheumatoid Arthritis is a chronic inflammation of the joints that can cause permanent joint damage and disability.

Syphilis is a sexually transmitted disease. It can be cured, but if left untreated, the late stages of the disease can cause heart problems, abnormalities, mental illness, blindness and death. Although on the decline over the past decade, it has seen more cases in recent years.

Tuberculosis (TB) is a disease caused by bacteria that usually attacks the lungs. It is spread through the air from one person to another and is rampant in our country.

Lyme Disease is a disease caused by bacteria that are transmitted to people from the bite of a deer tick. It often causes a “bull’s-eye” rash around the bite, with symptoms such as fever, tiredness, headache, muscle and joint aches.

Causes of Intermediate Uveitis
In more than two thirds of all cases of intermediate uveitis, the exact cause is unknown. The remaining third of all cases are thought to be caused by conditions such as:

Sarcoidosis is a kind of inflammation that can affect many parts of the body. Its cause is unknown.

MS (multiple Sclerosis) an inflammatory disease that affects the brain and spinal cord

Lyme Disease, a disease caused by bacteria that are transmitted to people from the bite of a deer tick. It often causes a “bull’s-eye” rash around the bite, with symptoms such as fever, tiredness, headache, muscle and joint aches.

Tuberculosis and Syphilis can cause all types of uveitis. In our country they need to be looked for in all patients with uveitis.

Causes of Posterior Uveitis
Following is a list of common causes of posterior uveitis. In more than one in ten cases of uveitis, however, the exact cause is unknown.

Toxoplasmosis is an infection caused by a parasite. It affects up to one fifth of all adults in America. People may become infected from eating raw or undercooked meats, drinking unpasteurized milk, cleaning cat litter boxes, or working in gardens or playing in sandboxes that contain cat feces.

Serpiginous Choroidopathy is related to uveitis – a rare condition that affects the part of the uvea called the choroid. It can affect the vitreous body and cause lesions on the retina that result in vision loss.

Tuberculosis can cause inflammations of the blood vessels, choroiditis and abscesses.

Cytomegalovirus Retinitis (CMV Retinitis) is a serious eye infection of the retina. It is a threat to people with weak immune systems, such as people with HIV, people undergoing chemotherapy, and people who have had organ transplants. Without treatment, CMV retinitis can destroy the retina and damage the optic nerve, resulting in blindness.

Lupus is a chronic inflammatory disease that affects different parts of the body, including the skin, joints, blood, and kidneys and is related to the body’s immune system.

Birdshot Retinochoroidopathy may be an autoimmune disease. People with this condition may experience blurry vision, sensitivity to light, floaters, and night blindness. In most people, it affects both eyes. It causes white or yellow lesions scattered around the retina that can damage the retina and affect the vitreous.

Sarcoidosis is a kind of inflammation that can affect many parts of the body. Its cause is unknown.

Acute Retinal Necrosis is a condition that often causes retinal detachment and vision loss. Signs may include red eyes, eye pain and hazy/blurry vision.

Behcet’s Disease is a rare, chronic inflammatory disease. The cause of Behcet’s disease is unknown. If often begins when individuals are in their 20s or 30s, although it can happen at any age. Uveitis is very common with people suffering from Behcet’s, which can cause acute uveitis that happens soon after onset of Behcet’s disease.

Syphilis is a sexually transmitted disease. It can be cured, but if left untreated, the late stages of the disease can cause heart problems, abnormalities, mental illness, blindness and death. Although on the decline over the past decade, it has seen more cases in recent years.

APMPPE is an eye condition that can cause temporary loss of vision related to lesions that appear in part of the retina. The lesions usually heal after a few weeks.

Sometimes inflammation can affect the entire uvea. This inflammation is called panuveitis. People with panuveitis may be more likely to experience vision loss from the condition. Symptoms include floaters, blurred or loss of vision.

Causes of Panuveitis
As with other kinds of uveitis, the cause of panuveitis is often unknown. Other possible causes include:

Sarcoidosis is a kind of inflammation that can affect many parts of the body. Its cause is unknown.

Tuberculosis (TB) is a disease caused by bacteria that usually attacks the lungs. It is spread through the air from one person to another. Uveitis may be present in patients who are not harboring active systemic tuberculosis. The uveitis can occur both as an infection of the eye or as an allergic manifestation to the bacterium.

Behcet’s Disease is a rare, chronic inflammatory disease. The cause of Behcet’s disease is unknown. If often begins when individuals are in their 20s or 30s, although it can happen at any age. Uveitis is very common with people suffering from Behcet’s, which can cause acute uveitis that happens soon after onset of Behcet’s disease.

Lupus is a chronic inflammatory disease that affects different parts of the body, including the skin, joints, blood, and kidneys and is related to the body’s immune system.

Syphilis is a sexually transmitted disease. It can be cured, but if left untreated, the late stages of the disease can cause heart problems, abnormalities, mental illness, blindness and death. Although on the decline over the past decade, it has seen more cases in recent years.

Vogt-Koyanagi-Harada Syndrome is an inflammatory condition that involves the eyes and meninges (coverings of the brain). The condition is most common in Asians and American Indians. It often causes uveitis in both eyes and retinal detachments, and can result in serious vision loss and blindness.

Fungal Retinitis fungal infections can be a problem for people with weakened immune systems, including very premature babies and people with HIV or AIDS.

How is Uveitis Treated?

Uveitis is treated by several means:

One is to identify and treat the underlying cause if it can be found. This is usually in infective conditions such as tuberculosis, syphilis, herpes etc.

The other part of the treatment is directed at controlling the inflammation which by itself can damage the eye. These drugs are anti-inflammatory such as steroids and NSAID (non-steroidal anti inflammatory drugs such as Salicylates, Ibuprofen, Flurbiprofen, Naproxen).

Some patients need medication to suppress the immune systems that are called immunosuppressives. These include steroids and other non steroidal immunosuppressives (given below). The non steroidal immunosuppressives are used when long term immunosuppression is desired as steroids can have unacceptable side effects when taken over a prolonged period.

Antimetabolites

  • Azathioprine (Imuran)
  • Methotrexate (Rheumatrex)
  • Mycophenolate Mofetil (Cellcept)

T Cell Inhibitors

  • Cyclosporine (Sandimmune, Neoral)
  • Tacrolimus (Prograf)

Alkylating Agents

  • Chlorambucil (Leukeran)
  • Cyclophophamide (Cytoxan)

Biologics

  • Infliximab (Remicade)
  • Etanercept (Enbrel)
  • Interferon (Avonex)
  • Daclizumab (Zenapax)
  • Alefacept (Amevive)

Efalizumab (Raptiva)

What are the side effects of steroids?

Hence the most commonly used treatment for all types of uveitis is steroids either in the form of eye drops, injections outside the eye, injections within the eye and as oral tablets. In some situations very high dose steroids are given intravenously.

Side effects of steroid eye drops are seen with prolonged use and include cataract, glaucoma, ptosis, increased infections, flaring up of viral infections. Most side effects of oral steroids are from short-term use; however, long-term use can lead to additional side effects.

Short-Term Side Effects of Systemic Steroids
Most people receive systemic steroids for only a few days at a time, and experience only temporary side effects. These may include an increase in appetite, difficulty sleeping (insomnia), changes in mood and behavior, flushing (redness) of the face, and short-term weight gain due to increased water retention. These side effects usually resolve after a few days once the steroids have been stopped.
People with underlying medical conditions might also notice other side effects. Those with diabetes mellitus may see an increase in their blood sugar readings; those with high blood pressure may see their blood pressure readings rise. People with glaucoma could have an increase of the pressures within their eyes; people with congestive heart failure may retain water and have worsening of this condition. For this reason, a person with any chronic underlying disease should be closely followed by their physician while taking systemic steroids.
Long-Term Side Effects of Systemic Steroids
When systemic steroids are used for long periods of time, or when steroids are taken on multiple occasions, more serious side effects may occur. It is for these reasons that the dose and duration of systemic steroids should be minimized whenever possible. Many of the side effects are reversible if the steroids are stopped, while other side effects may be permanent.

Side effects of long-term steroid use include:

  • Glaucoma
  • Cataracts
  • High-blood pressure
  • Heart disease
  • Diabetes mellitus
  • Obesity
  • Acid reflux/GERD
  • Osteoporosis
  • Myopathy
  • Increase in certain types of infections
  • Cushing syndrome

People taking long-term systemic steroids should be closely monitored for the above diseases, and should take medicines to prevent osteoporosis. These medicines may include supplemental calcium and vitamin D, along with medicines to prevent bone loss called bisphosphonates.