IRVAN syndrome

Title

Other Names:

Idiopathic retinal vasculitis-aneurysms-neuroretinitis syndrome; Idiopathic retinal-aneurysms-neuroretinitis syndrome

Categories:

Congenital and Genetic Diseases; Eye diseases; Nervous System Diseases

Summary Summary

IRVAN syndrome is an acronym for "idiopathic retinal vasculitis-aneurysms-neuroretinitis syndrome," a condition that primarily affects the eyes. Although this condition can occur in people of all ages, it is most commonly diagnosed in the third or fourth decade of life.^[1]^[2] The severity of the condition varies from person to person with some affected people experiencing a mild form that resolves on its own and others experiencing a severe form that may progress to vitreous hemorrhage (leakage of blood into the eye), vision loss and/or glaucoma. The underlying cause of IRVAN syndrome is currently unknown. Most cases occur sporadically in people with no family history of the condition. Treatment varies based on the severity of the condition and the associated signs and symptoms. In severe cases, surgery or medications such as corticosteroids may be recommended.^[1]

Last updated: 1/21/2016

Symptoms Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms	Other Names	Learn More: HPO ID
30%-79% of people have these symptoms
Ocular hypertension	High eye pressure	0007906
Reduced visual acuity	Decreased clarity of vision	0007663
Vitreous floaters	Eye floaters Spots in front of eyes [ more ]	0100832
5%-29% of people have these symptoms
Blurred vision		0000622
Glaucoma		0000501
Macular edema		0040049
Optic atrophy		0000648
Photophobia	Extreme sensitivity of the eyes to light Light hypersensitivity [ more ]	0000613
Retinal exudate		0001147
Tractional retinal detachment		0007917

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Last updated: 2/1/2021

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Organizations 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 Providing General Support

American Foundation for the Blind
1401 South Clark Street
Suite 730
Arlington, VA 22202
Toll-free: 800-232-5463
Telephone: 212-502-7600
E-mail: info@aph.org
Website: https://www.afb.org/

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Learn More 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.

In-Depth Information

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.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss IRVAN syndrome. Click on the link to view a sample search on this topic.

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

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References References

Pichi F, Ciardella AP. Imaging in the diagnosis and management of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN). Int Ophthalmol Clin. Fall 2012; 52(4):275-282. https://www.ncbi.nlm.nih.gov/pubmed/22954951.
Krishnan R, Shah P, Thomas D. Subacute idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) in a child and review of paediatric cases of IRVAN revealing preserved capillary perfusion as a more common feature. Eye (Lond). January 2015; 29(1):145-147. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4289823/.

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