IRVAN syndrome
Alternate names[edit | edit source]
Idiopathic retinal vasculitis-aneurysms-neuroretinitis syndrome; Idiopathic retinal-aneurysms-neuroretinitis syndrome
Definition[edit | edit source]
IRVAN syndrome is an acronym for "idiopathic retinal vasculitis-aneurysms-neuroretinitis syndrome," a condition that primarily affects the eyes.
Cause[edit | edit source]
The underlying cause of IRVAN syndrome is currently unknown.
Inheritance[edit | edit source]
Most cases occur sporadically in people with no family history of the condition.
Onset[edit | edit source]
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[edit | edit source]
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[edit | edit source]
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[edit | edit source]
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).[1].
Treatment[edit | edit source]
- 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. [2].
References[edit | edit source]
- ↑ 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
- ↑ 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
NIH genetic and rare disease info[edit source]
IRVAN syndrome is a rare disease.
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