IRVAN syndrome

Alternate names

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

Definition

IRVAN syndrome is an acronym for "idiopathic retinal vasculitis-aneurysms-neuroretinitis syndrome," a condition that primarily affects the eyes.

Cause

The underlying cause of IRVAN syndrome is currently unknown.

Inheritance

Most cases occur sporadically in people with no family history of the condition.

Onset

Although this condition can occur in people of all ages, it is most commonly diagnosed in the third or fourth decade of life.

Signs and symptoms

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.

Clinical presentation

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.

30%-79% of people have these symptoms

• Ocular hypertension(High eye pressure)
• Reduced visual acuity(Decreased clarity of vision)
• Vitreous floaters(Eye floaters)

5%-29% of people have these symptoms

• Blurred vision
• Glaucoma
• Macular edema
• Optic atrophy
• Photophobia(Extreme sensitivity of the eyes to light)
• Retinal exudate
• Tractional retinal detachment

Diagnosis

The disease has variable clinical presentation, and clinical diagnosis is aided by the help of ophthalmic imaging, such as fundus fluorescein angiography (FFA), preferably by wide-field system and optical coherence tomography (OCT).<ref>Bajgai, P., Katoch, D., Dogra, M. R., & Singh, R. (2017). Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome: clinical perspectives. Clinical ophthalmology (Auckland, N.Z.), 11, 1805–1817. https://doi.org/10.2147/OPTH.S128506</ref>.

Treatment

• 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.
• Early intervention in these patients in the form of prompt laser treatment without waiting for neovascularization to develop is important.
• Anti-VEGF and infliximab have been used as adjunctive treatment in IRVAN with laser treatment to reduce disease progression. <ref>Bajgai, P., Katoch, D., Dogra, M. R., & Singh, R. (2017). Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome: clinical perspectives. Clinical ophthalmology (Auckland, N.Z.), 11, 1805–1817. https://doi.org/10.2147/OPTH.S128506</ref>.

References

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NIH genetic and rare disease info

• NIH info on IRVAN syndrome

IRVAN syndrome is a rare disease.

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