Panuveitis

Panuveitis can be associated with an underlying condition, or it may occur by itself without the cause being known (idiopathic). Possible causes may include an attack from the body's own immune system (autoimmunity); an infection or tumor within the eye or in another part of the body; injury to the eye; and toxins that may penetrate the eye. Specific conditions that have been associated with panuveitis include tuberculosis; Vogt- Koyanagi-Harada syndrome; sympathetic ophthalmia; Behcet's disease; and sarcoidosis. The signs and symptoms of the condition are due to inflammatory responses inside the eye.

Panuveitis is not thought to be an inherited condition. While familial cases of uveitis have been reported, it is very rare. The few cases that have been reported may represent the familial occurrence of underlying conditions that are associated with uveitis (e.g. sarcoidosis, ankylosing spondylitis). We are not aware of reports of familial cases of panuveitis specifically, and to our knowledge, no gene responsible for the condition has been identified.

Panuveitis is inflammation of all layers of the uvea of the eye, which includes the iris, ciliary body, and choroid. These make up the middle layer of the eye. The condition can also affect the lens, retina, optic nerve, and vitreous, causing reduced vision or blindness. Signs and symptoms may include eye redness and pain; blurring; light sensitivity; decreased vision; and seeing floaters. It can last for a short time or can occur chronically. Severe cases may recur many times. In many cases the specific cause is unknown, but in some cases it occurs in association with other eye conditions, or with another condition or infection that also affects other body parts. Early treatment is needed and may include eye drops to reduce inflammation and pain; and/or oral medications or injections in severe cases.

We are not aware of genetic markers associated with panuveitis specifically, but there is some information in the medical literature about genetic markers for other types of uveitis.

Genetic factors have reportedly been involved in the development of pediatric uveitis. It has been demonstrated that human leukocyte antigens HLA-A19, HLA-B22 and HLA-DR9 can increase susceptibility to juvenile idiopathic arthritis (JIA)-associated uveitis, whereas HLA-DR1 can be protective for uveitis development. Other HLA genes have also been associated with JIA-associated uveitis. Additionally, there has been evidence that the TRAF5 gene is involved in the development of acute anterior uveitis and pediatric uveitis.

Uveitis can also be associated with other conditions for which genetic markers have been identified (e.g., Blau syndrome, Behcet disease, autoimmune lymphoproliferative syndrome, and neovasular inflammatory vitreoretinopathy).

The outlook (prognosis) for people with panuveitis somewhat varies depending upon the cause and severity. However, uveitis is one of the major causes of blindness in the world, and visual morbidity in panuveitis is poor. Profound and irreversible vision loss can occur, especially when the condition is unrecognized, inadequately treated, or both.

Uveitis can rapidly damage the eye and cause long-term, vision-threatening complications. The most frequent complications include:

• Cataract
• Glaucoma
• Retinal detachment
• Neovascularization of the retina, optic nerve, or iris
• Cystoid macular edema (the most common cause of decreased vision in patients with uveitis)

Panuveitis may be treated in several ways, including injections around the eye, oral medications, and eye drops. Corticosteroids are the treatment of choice for most types of uveitis, including panuveitis. Depending on the severity of the disease, oral prednisolone is typically started in a larger dose, and then is tapered off a few weeks after starting therapy as inflammation improves. A maintenance dose is typically used thereafter.

Immunomodulating drugs (drugs that alter the body's immune response) are needed if the inflammation cannot be controlled with corticosteroids or if active inflammation interferes with activities of daily living. Cycloplegics (eye drops that temporarily paralyze the ciliary body) are used to help with pain due to ciliary spasms.

Forms of uveitis due to infectious agents are treated by specific antimicrobial therapy (antibiotics, antiparasitics or antivirals), with or without corticosteroids.