Acute Multifocal Placoid Pigment Epitheliopathy

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.

Image 1 Acute multifocal placoid pigment epitheliopathy

Acute Retinal Necrosis (ARN)

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.

Arthritis syndromes

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.

Image 1 Feet affected in reactive arthritis

Image 2 Reactive arthritis

Behcet's syndrome

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.

Image 1 Behcet s syndrome

Birdshot choroidopathy

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.

Image 1 Birdshot choroidopathy

Cancers

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.

Candida retinitis

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.