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Disease Profile

IRVAN syndrome

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.

<1 / 1 000 000

US Estimated

Europe Estimated

Age of onset






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


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.


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.


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


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.


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.


Not applicable


Other names (AKA)

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


Congenital and Genetic Diseases; Eye diseases; Nervous System Diseases


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]


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.

Medical Terms Other Names
Learn More:
30%-79% of people have these symptoms
Ocular hypertension
High eye pressure
Reduced visual acuity
Decreased clarity of vision
Vitreous floaters
Eye floaters
Spots in front of eyes

[ more ]

5%-29% of people have these symptoms
Blurred vision
Macular edema
Optic atrophy
Extreme sensitivity of the eyes to light
Light hypersensitivity

[ more ]

Retinal exudate
Tractional retinal detachment


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

    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.


      1. 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.
      2. 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/.