Rare Endocrinology News

Disease Profile

Panuveitis

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

All ages

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ICD-10

H44.1

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Inflammation of the whole uveal tract; Total uveitis; Diffuse uveitis

Categories

Eye diseases

Summary

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.[1] The condition can also affect the lens, retina, optic nerve, and vitreous, causing reduced vision or blindness.[2] Signs and symptoms may include eye redness and pain; blurring; light sensitivity; decreased vision; and seeing floaters.[1] It can last for a short time or can occur chronically. Severe cases may recur many times.[2] 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.[1][2] Early treatment is needed and may include eye drops to reduce inflammation and pain; and/or oral medications or injections in severe cases.[1]

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.[1][2]

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. The signs and symptoms of the condition are due to inflammatory responses inside the eye.[1][2][3]

Treatment

Panuveitis may be treated in several ways, including injections around the eye, oral medications, and eye drops.[2][3] 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.[3]

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.[3][5] Cycloplegics (eye drops that temporarily paralyze the ciliary body) are used to help with pain due to ciliary spasms.[3]

Forms of uveitis due to infectious agents are treated by specific antimicrobial therapy (antibiotics, antiparasitics or antivirals), with or without corticosteroids.[3]

FDA-Approved Treatments

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

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Social Networking Websites

      Learn more

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      Where to Start

      • The American Academy of Ophthalmology Web site has an information page on Panuveitis. Their Web site is dedicated to educating people about eye diseases and conditions and the preservation of eye health.
      • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
      • The Merck Manual provides information on this condition for patients and caregivers.
      • The National Eye Institute (NEI) was established by Congress in 1968 to protect and prolong the vision of the American people. Click on the link to view information on this topic.

        In-Depth Information

        • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
        • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
        • PubMed is a searchable database of medical literature and lists journal articles that discuss Panuveitis. Click on the link to view a sample search on this topic.

          References

          1. Kierstan Boyd. What is uveitis?. American Academy of Ophthalmology. March 1, 2014; https://www.geteyesmart.org/eyesmart/diseases/uveitis/index.cfm.
          2. Facts About Uveitis. National Eye Institute. August, 2011; https://nei.nih.gov/health/uveitis/uveitis.
          3. Reema Bansal, Vishali Gupta, and Amod Gupta. Current approach in the diagnosis and management of panuveitis. Indian Journal of Ophthalmology. Jan-Feb, 2010; 58(1):45-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2841373/.
          4. Deuter CM1, Stübiger N, Siepmann K, Derse M, Zierhut M. [Familial uveitis. Forms and incidence in patients at the University Eye Hospital Tubingen]. Ophthalmologe. September, 2003; 100(9):713-719.
          5. James T Rosenbaum. Uveitis: Treatment. UpToDate. Waltham, MA: UpToDate; October, 2016;
          6. Emmett T. Cunningham, Jr. Uveitis. Merck Manual. October, 2014; https://www.merckmanuals.com/professional/eye-disorders/uveitis-and-related-disorders/overview-of-uveitis.

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