Panuveitis

Other Names: Inflammation of the whole uveal tract; Total uveitis; Diffuse uveitis 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. It can last for a short time or can occur chronically. Severe cases may recur many times.

Cause

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.

Inheritance

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.

Symptoms

Signs and symptoms of panuveitis can develop suddenly and may affect one or both eyes. They may include blurred vision; seeing dark, floating spots ("floaters"); eye pain; redness of the eye; and sensitivity to light (photophobia). There may also be a whitish area (called a hypopyon) obscuring the lower part of the iris. Signs and symptoms may include eye redness and pain; blurring; light sensitivity; decreased vision; and seeing floaters.

Diagnosis

Diagnosis of panuveitis is established in the presence of the following clinical signs: Evidence of choroidal or retinal inflammation such as choroiditis (focal, multifocal or serpiginous), choroidal granuloma, retinochoroiditis, retinal vasculitis, subretinal abscess, necrotizing retinitis or neuroretinitis; with evidence of vitreous inflammation (vitreous cells or vitritis); and presence of signs of anterior uveitis (cells and flare in the anterior chamber, keratic precipitates or posterior synechiae).

Laboratory tests Whereas anterior segment inflammations involving the iris are routinely picked up by slit-lamp examination, ciliary body involvement can best be confirmed by ultrasound biomicroscopy (UBM). Baseline color fundus photography serves as an extremely good clinical reference when monitoring the fundus changes during follow-up periods.

Digital color fundus photography allows easy storage and retrieval of fundus images. A composite montage of the retina can be made to document better the lesions in the peripheral fundus Fundus fluorescein angiography (FA) is mandatory for retinal and choroidal lesions. Indocyanine green angiography (ICGA) is the technique of choice for imaging the choroid.

Ultrasound is a safe, noninvasive, dynamic tool for the evaluation of the posterior segment when direct visualization of the fundus is obscured due to severe inflammation or its complications.

Optical coherence tomography (OCT) is a noncontact and noninvasive imaging tool.

PCR has been used to diagnose uveitis, including viral uveitis, mycobacterial intraocular infections, infectious endophthalmitis, and protozoal eye diseases. PCR is a powerful molecular technique for evaluation of very small amounts of Deoxyribonucleic acid and Ribonucleic acid. It can be a simple, rapid, sensitive, and specific tool for the diagnosis of infection, autoimmunity, and masquerade syndromes in the eye.

Treatment

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. The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.

• Adalimumab (Brand name: Humira)received expanded approval for the treatment of non-infectious intermediate, posterior, and panuveitis to include pediatric patients 2 years of age and older. It was approved for use in adults with non-infectious intermediate, posterior, and panuveitis in June 2016.
• Dexamethasone Intravitreal Implant (Brand name: Ozurdex)For treating non-infectious ocular inflammation, or uveitis, affecting eye's posterior segment.

Prognosis

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).

NIH genetic and rare disease info

• NIH info on Panuveitis

Panuveitis is a rare disease.

Panuveitis Resources
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