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Types of Uveitic Diseases

Acute Multifocal Placoid Pigment Epitheliopathy (AMPEE) causes posterior uveitis and generally affects young men and women. It begins with a viral infection that can cause symptoms which include: headaches, skin lesions, coughing, nausea, vomiting and joint pains. Approximately one week after these symptoms people report a sudden decrease in vision. There are usually no other eye symptoms. Both eyes are affected by AMPEE, however, one eye is usually worst than the other. Most often, symptoms resolve within several weeks and no treatment is required. The image below illustrates how the eye appears to eye doctors when they examine an eye with Acute Multifocal Placoid Pigment Epitheliopathy.

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Acute Retinal Necrosis (ARN) is a rapidly progressive and severe cause of posterior uveitis. Although it can affect men and women of all ages, it typically affects young adults. It generally occurs in people who are otherwise healthy.

ARN begins by affecting one eye of a patient and in 1/3 of patients the second eye becomes affected within 6 weeks. Patients often have a history of pain, redness and blurry vision. ARN may begin by causing anterior uveitis but ultimately creates unique lesions in the retina. Irreversible vision loss results from damage to areas of the retina responsible for central vision. As the inflammation progresses, it can lead to severe complications such as retinal detachment and optic nerve damage.

ARN is thought to be caused by herpes virus and may have a hereditary component. Patients benefit from antiviral therapy and may require surgery to repair retinal detachments. When untreated, ARN progresses rapidly over the course of 2-3 months.

Anterior uveitis commonly occurs in patients with arthritis. There are many different arthritis syndromes and the ones that most commonly cause uveitis are: Ankylosing Spondylitis, Reactive Arthritis and Juvenile Idiopathic Arthritis.

1. Ankylosing Spondylitis

Most people with Ankylosing Spondylitis are men between 20 to 30 years old. This is one of the most common causes of uveitis and it can occur in one or both eyes. Often one eye is affected before or has more severe symptoms than the other. It occurs suddenly and lasts for weeks to months. Its symptoms include sensitivity to light, red eye and reduced vision. The symptoms of uveitis may not occur at the same time as the joint disease that is caused by Ankylosing Spondylitis. In fact, uveitis may occur before any signs of joint disease at all. This form of arthritis most often leads to pain and prolonged stiffness in the back and hips. It is not known what exactly causes Ankylosing Spondylitis. However, it is believed to be genetically inherited. Most people have the HLA B27 gene. In addition to receiving medical care by an Ophthalmologist, a referral may be made to a Rheumatologist who examines the joints to monitor the arthritis.

2. Reactive Arthritis

This form of arthritis predominantly occurs in men but can occur in women as well. It leads to a variety of symptoms including skin rashes (generally on the soles of feet - see image below), burning, painful or frequent urination, pain and stiffness in joints, heel pain, mouth ulcers and uveitis. The uveitis can occur in both eyes and is severe. It causes redness, sensitivity to light and reduced vision. There may be puss released from the eyes as well. Reactive Arthritis can occur as a result of sexually transmitted infections. It can also occur from infections due to food poisoning or drinking unclean water which often leads to fever, diarrhea or abdominal pain. Some people are more likely than others to acquire Reactive Arthritis as a result of these infections because it is genetically inherited as well. The causes of Reactive Arthritis are, therefore, complex as it occurs due to a result of both infections and inheritance.

3 Juvenile Idiopathic Arthritis (JIA)

Juvenile Idiopathic Arthritis is a group of arthritis disorders that specifically affect children. JIA can occur anytime before the age of 16, but, most often occurs in girls between the ages of 2 and 4 years old. It causes pain and stiffness in joints around the body. Some people report a fever and abdominal pain as well. JIA is a common cause of uveitis in this age range. Those who develop uveitis generally only have mild pain in less than four joints of their body.

Uveitis can affect both eyes and is unique because it usually does not create symptoms. Sometimes there may be reduced vision and whiteness in the central portion of the eye that is normally black. The swelling within the eye lasts from months to years and does not get better on its own. It is particularly concerning because it can commonly lead to blindness. For this reason, it is important to regularly visit an Ophthalmologist for a thorough eye examination. Although it may not be apparent to you that the eye has uveitis, the Ophthalmologist can detect changes in the eye that indicate uveitis. The image below illustrates uveitis in a patient with reactive arthritis that can be seen through an eye examination.

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Behcet’s syndrome is a relatively rare cause of uveitis in North America. It is a common cause of uveitis in Turkish, Middle Eastern and Asian populations. Its cause is unknown. Behcet’s syndrome classically causes a combination of symptoms that consists of: sores in the mouth, sores on the sex organs and posterior uveitis. It is more common in men and occurs between the ages of 20 and 40 years. The uveitis occurs in both eyes. Patients often experience blurry vision, red eye, eye pain and sensitivity to light. New medications have been developed specifically for Behcet’s syndrome which can help to prevent complications. However, Behcet’s syndrome is very serious and aggressive e treatment is needed to prevent blindness. The image below illustrates how the eye appears to eye doctors when they examine an eye with Behcet's syndrome.

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Birdshot choroidopathy is a rare disease that affects middle-aged men and women. Its causes are not known, however, researchers suspect that it may be genetic. It causes posterior uveitis. Symptoms experienced include decreased vision, floaters, pain and night blindness. In rare cases it may not cause any symptoms. Birdshot retinochoroidopathy can lasts for years and often results in vision loss. It is extremely important to regularly see an ophthalmologist and to follow treatment guidelines. Treatment with an oral immunosuppressant may be needed. The image below illustrates how the eye appears to eye doctors when they examine an eye with Birdshot choroidopathy.

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Cancers can cause uveitis-like symptoms. Although they are not true causes of uveitis, the symptoms that some individuals experience are similar to uveitis and can often be mistaken to be anterior or posterior uveitis. Cancers that commonly lead to uveitis-like symptoms are malignant melanoma, leukemia and most commonly-various forms of lymphoma. Each of these disorders is unique, has distinct symptoms and can occur in different age groups. Retinoblastoma, for instance, occurs in children under the age of 3. Lymphoma commonly occurs after the age of 60 years old. Patients receive medical care from a team of specialists including an ophthalmologist and an oncologist.

Canadida is a type of fungus that is naturally found on human skin. It can enter into the bloodstream if there is a break in the skin. In most healthy individuals, this not does cause uveitis. However, in patients with a weakened immune system, exposure of this fungus to the bloodstream can cause serious symptoms including posterior uveitis. This is a particularly important cause of uveitis in hospitalized individuals with catheters because the catheter may cause breaks into the skin. With an already weakened immune system, this poses a great threat for acquiring candida infection. Another common risk factor is intravenous drug usage that may be practiced through improperly sterilized needles. Other reasons for a weakened immune response include medications, such as chemotherapy and long-term antibiotic usage. This form of uveitis can occur at any age, occurs suddenly and usually affects in both eyes. Patients experience decreased vision and floaters. The outcomes and treatment of this infection depend upon the amount of fungus in the blood.

Cytomegalovirus (CMV) is a virus commonly found in the environment around the world. Most human beings are exposed to the virus at some point in their life. In healthy individuals, it only causes mild flu-like symptoms. However, it can cause severe diseases, including posterior uveitis, in newborns, infants and people with a weakened immune system. Other parts of the body that be affected by CMV include skin, lungs, intestines and brain.

The immune system can weaken due to a number of reasons such as diseases or medications. Some diseases such as AIDS and cancer exhaust the body’s resources that are needed to fight off infections. Medications (for example: chemotherapy) can also have this same effect. In addition, patients who have received transplants (for example: kidney transplant) also have weakened immune systems. CMV can cause uveitis in these patients. Eyes often appear white and the patient’s only symptoms may be floaters. Patients rarely report pain, sensitivity to light and redness. The end-stage of this infection can lead to vision loss. Historically there were no treatment options for this infection. Fortunately, today antiviral medications exist that can fight CMV. These medications reduce symptoms and prolong vision in most cases.

Newborns and infants may also be infected by CMV. They often experience a combination of symptoms including: reduced blood cells, fever, pneumonia, red or purple spots on the body and enlarged portions of the abdomen. They may also experience other changes in the eye, such as cataract formation. Most newborns acquire the infection from their mothers. Infected mothers are capable of transmitting the infection to their fetus through the placenta during pregnancy. After pregnancy, newborns may acquire the disease through a number of other routes such as breast milk, exposure to infected saliva or exposure to the virus during birth in the birth canal. This infection can lead to loss of vision. Treatment in newborns and infants may be possible but requires the expert opinion of an ophthalmologist.

About 2% of uveitis is caused by Fuchs’ Heterochromic Iridocyclitis. Its exact cause is unknown but studies indicate that it may be genetic. It leads to anterior uveitis. Fuchs heterochromic iridocyclitis occurs in men and women who are in their 30s and 40s. Most often it affects only one eye. It can cause redness, sensitivity to light, floaters and blurry vision. There is often no pain. In fact, sometimes there may not be any symptoms at all and the uveitis will only be seen during a routine eye examination. A unique feature of this disorder is that there may be a colour change in the eye. This form of uveitis lasts for months to years. The two most common complications of Fuchs heterochromic iridocyclitis are cataract and glaucoma. Depending on the severity of the condition, Ophthalmologist may or may not recommend treatment. The image at the top (left) illustrates colour change in the eye. The remaining images illustrate how the eye appears to an eye doctor who is examining an eye with Fuchs heterochromic iridocyclitis.

A small number of people with gastrointestinal diseases develop anterior or intermediate uveitis. Bowel diseases associated with uveitis are: Ulcerative colitis, Crohn's disease and Whipple's disease. This form of uveitis affects both eyes and occurs in men and women. If individuals with uveitis experience gastrointestinal symptoms it is important for the ophthalmologist to be informed. Medical care is delivered by a team of physicians that includes an ophthalmologist and gastroenterologist.

1. Whipple's disease

Whipple’s disease is a rare disease that affects many parts of the body. It is caused by bacteria. Common symptoms are: blurry vision, vision loss, poor digestion of food, pain and stiffness in joints, abdominal pain, nausea, fever and diarrhea. In addition to the general medications that are used to treat uveitis, ophthalmologists may prescribe an antibiotic as well.

2. Ulcerative colitis (UC)

UC is a lifelong disease that affects the colon. It commonly causes bleeding in bowel movements, abdominal cramps, weight loss and fever. It may also affect many other organs in the body. The cause of UC is unknown.

3. Crohn's disease

Crohn's disease commonly causes extensive diarrhea and abdominal cramps. Its causes are unknown and it can lasts for years. Patients may experience changes in the skin, joints, mouth and liver.

Lens-induced uveitis is a form of anterior uveitis that occurs when pieces of the eye’s lens break apart and cause irritation in the eye. The lens can break and form these pieces after trauma or surgery to the eye. This can occur at any age and impacts both men and women. It usually affects only one eye. Symptoms include: redness, sensitivity to light, pain and floaters. Medications are usually used to treat it. Sometimes surgery is also required to remove the pieces of the lens that are causing uveitis. With treatment, lens-induced uveitis is treatable.

Pars planitis is a common cause of intermediate uveitis in teenagers and young adults. Its cause is unknown. It affects both males and females. Patients generally experience floaters and hazy visions. However, there may not be any symptoms at all. Uveitis occurs in both eyes but one eye usually has fewer symptoms. Pars planitis can last from months to years. Ophthalmologists may choose to treat this with medicine. In mild cases, it is left untreated as the side-effects of medications may outweigh the benefits. Patients can generally recover if treatment is started and followed.

Presumed Ocular Histoplasmosis Syndrome (POHS) is thought to be associated with an infection by a type of fungus called Histoplasma capsulatum. Histoplasma capsulatum is often found in decaying bird or bat droppings and is most commonly found along the St. Laurence river in eastern Canada. In these areas, a major portion of the population has had exposure to this fungus. Studies have shown that large numbers of people have had exposure to this organism along river belts. Individuals involved in activities such as spelunking or those who have exposure to buildings inhabited by birds or bats are particularly at risk of acquiring an infection by Histoplasma capsulatum.

Infection begins when the fungus is inhaled. At this stage, it usually does not produce any serious symptoms. Occasionally it may produce flu-like symptoms. Nonetheless, the infection is transmitted throughout the body and in some cases it travels to the eyes where it can cause posterior uveitis. If this occurs, the infection in the eyes is called Presumed Ocular Histoplasmosis Syndrome (POHS). Patients with POHS often complain of distorted vision or sudden and painless loss of vision. However, there may not be any symptoms at all which can lead to a delay in visiting the ophthalmologist.

Most patients with POHS are in early to mid-adulthood and POHS is an important cause of vision loss in young people. Laboratory tests are generally not helpful in diagnosing this disease and the ophthalmologist may recommend treatment.

The image below illustrates how the eye appears to an eye doctor who is examining an eye with Presumed Ocular Histoplasmosis Syndrome.

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Sarcoidosis can cause uveitis in any portion of the eye. It is a disease that affects many different organs in the body but it is most commonly a disease of the lung. Its exact cause is not known. People often report fatigue, weight loss, fever, chest pain and difficulty breathing. It can also affect the skin, joints and abdomen. In the eye it leads to blurry vision, red eye, sensitivity to light, pain and floaters. Most patients are between the ages of 20 and 40 years and it affects women slightly more commonly than men. Studies show that people of African descent have more common and severe forms of this type of uveitis. A combination of medicine and surgery is sometimes needed for treatment. The outcomes differ from person to person, however, often uveitis often lasts for months and leads to other eye complications. The image below illustrates how an eye with sarcoidosis appears to an eye doctor during examination.

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Serpiginous choroidopathy is a poorly understood cause of posterior uveitis that affects the choroid. Although its exact mechanism of damage within the eye is not known, it is thought to be caused by inflammation and degeneration within the eye. Most affected patients are otherwise healthy middle-aged adults. They generally experience blurry vision and central area of vision loss. Serpiginous choroidopathy affects both eyes.

Eye examination reveals numerous placoid lesions in the choroid that are unique to serpiginous choroidopathy. At times, additional tests are needed in order to visualize inner parts of the eye. Patients are generally treated with high doses of oral steroids.

Sympathetic ophthalmia can cause anterior or posterior uveitis. It occurs as a result of intentional or unintentional trauma that penetrates the eyes, for example eye surgery or injury. Typically the uveitis sets in 4-8 weeks after trauma to the eyes and 90% of cases occur within a year. Today, Sympathetic Ophthalmia is extremely rare and is particularly less common since the advent of modern surgical techniques in Ophthalmology. Sympathetic Opthalmia impacts both eyes and patients experience softness, redness and irritation in their eyes. Treatment may be recommended by the ophthalmologist.

Syphilis is an infection that can be transmitted through sexual contact. It can also be passed on to a fetus from an infected mother. It begins with painless ulcer in the genital region, rectum or mouth. 4 to 10 weeks later, syphilis can lead to a number of other symptoms in various organs, including uveitis in the eyes.

Syphilis can cause anterior or intermediate uveitis. It can occur in either one or two eyes. Eyes can become affected at any age in both men and women. Ophthalmologists may administer laboratory tests to determine whether the uveitis is a result of the syphilis. If detected and treated early, the uveitis can resolve. Along with the usual medications, antibiotics called penicillin is used for treatment. If left untreated, uveitis will worsen and can lead to complications.

Toxocariasis is an infection caused by a roundworm of cats and dogs called Toxocara canis (dogs) or Toxocara cati (cats). Although this parasite also causes a whole-body infection called Visceral Larva Migrans (VLM), posterior uveitis can occur without VLM. VLM causes a variety of symptoms including fatigue, weight loss and fever.

Those infected are generally under 30 years old but most are under 12 years. Uveitis results when this parasite travels into the rear portion of the eye. People experience uveitis symptoms in one eye and report decreased vision, sensitivity to light, irritation, red eye and a white appearance of the pupil. A potential complication of this infection is vision loss.

This form of uveitis may not require specific treatment. However, in some cases your ophthalmologist may recommend treatment against Toxocariasis and to reduce the swelling that it causes. The most common cancer of the eye in children called retinoblastoma has some of the same features of uveitis caused by toxocariasis. Although it may be possible to distinguish the two, in some cases this may not be possible and your ophthalmologist may recommend additional precautions to prevent the possible cancer from spreading.

The most important measure that one can take to avoid this form of uveitis is to eliminate toxocariasis from cats and dogs. Veterinarians (animal doctors) are trained to remove this roundworm from cats and dogs of this. It is also important to ensure proper hand hygiene following contact with pets.

Tuberculosis (TB) is an infection that may be potentially serious and fatal. It is caused by a small organism called Mycobacterium Tuberculosis which is transmitted through the air. In most situations, TB begins with a lung infection. It causes fever, chills, weight loss, coughing that lasts at least 3 weeks, chest pain when coughing or breathing and blood upon coughing. In a small portion of these patients, TB can also affect organs other than the lungs, including the eyes, and can lead to anterior or posterior uveitis.

Uveitis caused by TB can affect one or both eyes and may occur at any age. It most often leads to blurry vision. In some situations it can also cause pain and red eye. In rare situations, uveitis may occur without any symptoms in the lungs. Risk factors for developing TB include:

- close contact with someone who has TB infection

- international travel

- weak immune system

- young or old age

- AIDS

Ophthalmologists may choose to administer a number of tests in collaboration with other medical specialists. These can include a chest x-ray and skin test. In addition to the general medications used to treat uveitis, there are a number of medications that are specifically used for TB infections only. Although TB is a rare cause of uveitis, it is often treatable and this makes it very important to detect. If left untreated, it may progress to involve other structures within the eye and can lead to serious eye damage. When treated, the outcome can be quite positive.

Vogt-koyanagi-harada syndrome is a rare cause of uveitis in adults. It usually affects natives in the Canadian population. It affects both men and women and usually causes posterior uveitis. It can last for weeks to months to years. Patients with Vogt-koyanagi-harada often experience sensitivity to light, tearing, and blurred vision. In most people the second eye is affected within a few weeks of the first eye. It typically affects other organs in the body as well. However, not every patient will experience the same symptoms. These symptoms can include any of: headache, pain around the eyes, stiff neck, vertigo, fever, difficulty hearing, pain or discomfort when hearing, patchy loss of color on the skin and decreased or absence of colour in head hair, eyebrows or eyelashes. Vogt-koyanagi-harada syndrome’s causes are not fully understood. Ophthalmologists may recommend treatment to control this disease. Usually corticosteroid pills are an important part of the treatment. The top left image illustrates patchy loss of colour in the eyebrow. The remaining images illustrates how the eye appears to an eye doctor during an eye examination.

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