Retinitis PigmentosaSymptoms, Doctors, Treatments, Advances & More
Learn About Retinitis Pigmentosa
Retinitis pigmentosa is an eye disease in which there is damage to the retina. The retina is the layer of tissue at the back of the inner eye. This layer converts light images to nerve signals and sends them to the brain.
RP; Vision loss - RP; Night vision loss - RP; Rod Cone dystrophy; Peripheral vision loss - RP; Night blindness
Common conditions include: X-Linked Retinitis Pigmentosa (XLRP)
Retinitis pigmentosa can run in families. The disorder can be caused by several genetic defects.
The cells controlling night vision (rods) are most likely to be affected. However, in some cases, retinal cone cells are damaged the most. The main sign of the disease is the presence of dark deposits in the retina.
The main risk factor is a family history of retinitis pigmentosa. It is a rare condition affecting about 1 in 4,000 people in the United States.
Symptoms often first appear in childhood. However, severe vision problems do not often develop before early adulthood.
- Decreased vision at night or in low light. Early signs may include having a harder time moving around in the dark.
- Loss of side (peripheral) vision, causing "tunnel vision."
- Loss of central vision (in advanced cases). This will affect the ability to read.
- Loss of color vision
- Sensitivity to bright light
There is no effective treatment for this condition. Wearing sunglasses to protect the retina from ultraviolet light may help preserve vision.
Some studies suggest that treatment with antioxidants (such as high doses of vitamin A palmitate) may slow the disease. However, taking high doses of vitamin A can cause serious liver problems. The benefit of treatment has to be weighed against risks to the liver.
Clinical trials are in progress to assess new treatments for retinitis pigmentosa, including the:
- Use of DHA, which is an omega-3 fatty acid.
- Treatments related to the possible genetic causes of this disorder
Other treatments, such as microchip implants into the retina that act like a microscopic video camera, are in the early stages of development. These treatments may be useful for treating blindness associated with RP and other serious eye conditions.
A vision specialist can help you adapt to vision loss. Make regular visits to an eye care specialist, who can detect cataracts or retinal swelling. Both of these problems can be treated.
UPMC Vision Institute
Jose' Sahel is an Ophthalmologist in Pittsburgh, Pennsylvania. Dr. Sahel is rated as an Elite provider by MediFind in the treatment of Retinitis Pigmentosa. His top areas of expertise are Retinopathy Pigmentary Mental Retardation, Cone-Rod Dystrophy, X-Linked Congenital Stationary Night Blindness, Retinitis Pigmentosa, and Vitrectomy. Dr. Sahel is currently accepting new patients.
The Johns Hopkins Hospital
Mandeep S. Singh, M.D., Ph.D., is associate professor of ophthalmology and genetic medicine in the Retina Division of the Wilmer Eye Institute and the recipient of the 2023 Andreas C. Dracopoulos Professorship in Ophthalmology. Dr. Singh is a retinal specialist. He is Co-Director of the Genetic Eye Disease (GEDi) Center, and is a principal investigator at the Center for Stem Cells and Ocular Regenerative Medicine (STORM). Dr. Singh completed his medical degree at the National University of Singapore. In 2009, he was elected to the Fellow of the Royal College of Surgeons of Edinburgh. He earned a PhD in ophthalmology from the University of Oxford, United Kingdom, in 2014. He completed fellowships in vitreoretinal diseases and surgery at the Oxford Eye Hospital and Moorfields Eye Hospital, both in the United Kingdom. His clinical expertise includes surgical, medical and genetic conditions of the retina and macula. Dr. Singh is specialty-trained in vitrectomy surgery for retinal detachment, macular holes, macular pucker, epiretinal membrane, proliferative vitreoretinopathy, complex retinal detachments including reoperations, vitreous hemorrhage removal, and other retinal surgeries. He also specializes in age-related macular degeneration, diabetic retinopathy, diabetic macular edema, retinal vein occlusion, and choroidal neovascularization. Dr. Singh sees patients and families with genetic retinal diseases such as retinitis pigmentosa, Usher syndrome, Stargardt disease and macular dystrophies. His research focus is retinal stem cell transplantation. Dr. Singh’s work has been recognized through the Eye Institute–Allergan Research Prize, the Merton College University of Oxford Graduate Prize Scholarship, the Oxford Ophthalmological Congress Founder’s Cup and Medal, the Ruskell Medal, the Johns Hopkins Clinician Scientist Award, and the Bert M. Glaser, MD Award for Innovative Research in Retina. He is a member of the American Academy of Ophthalmology and the Association for Research in Vision and Ophthalmology. He is also a member of the Club Jules Gonin and the Macula Society. CV https://www.hopkinsmedicine.org/-/media/wilmer/documents/cvs/Singh_CV.pdf. Dr. Singh is rated as an Elite provider by MediFind in the treatment of Retinitis Pigmentosa. His top areas of expertise are Retinitis Pigmentosa, Stargardt Macular Degeneration, Late-Onset Retinal Degeneration, Retinopathy Pigmentary Mental Retardation, and Vitrectomy.
The Johns Hopkins Hospital
Peter A. Campochiaro, M.D. is the George S. and Dolores Doré Eccles Professor of Ophthalmology and Neuroscience at the Wilmer Eye Institute, Johns Hopkins University School of Medicine. He is a clinician-scientist who directs a research laboratory and conducts clinical trials. His laboratory research is directed at understanding the pathogenesis of ocular neovascularization and excessive retinal vascular permeability, and the mechanism of cone cell death in inherited retinal degenerations. He helped to determine the importance of vascular endothelial growth factor (VEGF), hypoxia-inducible factor-1, and Tie2 in retinal and choroidal vascular diseases. The clinical trial group under Dr. Campochairo provided the first demonstration of the benefits of suppression of VEGF in diabetic macular edema and retinal vein occlusion. He has developed strategies for sustained suppression of VEGF that are currently being tested in clinical trials. Dr. Campochiaro trained at the University of Notre Dame, Johns Hopkins School of Medicine, and the University of Virginia. He did a vitreoretinal fellowship and research fellowships at Johns Hopkins and joined the faculty of the University of Virginia in 1984. He became professor of Ophthalmology and Neuroscience at the Wilmer Eye Institute, Johns Hopkins in 1991. Dr. Campochiaro is rated as an Elite provider by MediFind in the treatment of Retinitis Pigmentosa. His top areas of expertise are Retinal Vein Occlusion, Late-Onset Retinal Degeneration, Age-Related Macular Degeneration (ARMD), Trabeculectomy, and Vitrectomy.
The disorder will continue to progress slowly. Complete blindness is uncommon.
Peripheral and central loss of vision will occur over time.
People with retinitis pigmentosa often develop cataracts at an early age. They may also develop swelling of the retina (macular edema). Cataracts can be removed if they contribute to vision loss.
Contact your health care provider if you have problems with night vision or you develop other symptoms of this disorder.
Genetic counseling and testing may help determine whether your children are at risk for this disease.
Summary: This study explores whether adding early nurse-led and psychological support after the diagnosis of retinitis pigmentosa (RP) can improve patient experience and emotional well-being. RP is a rare, progressive eye disease often diagnosed after a long and difficult process, and receiving the diagnosis can be emotionally distressing. Eighty newly diagnosed adults will be randomly assigned to either u...
Summary: Inherited retinal diseases (IRDs) are a group of degenerative disorders that cause progressive vision loss. Retinitis pigmentosa (RP) is the most common form, with a global prevalence of approximately 1 in 4,500. About 20-30% of these cases are syndromic, most notably Usher syndrome (USH), which combines hearing loss with visual impairment. Usher syndrome type 1 (USH1), the most severe form, prese...
Published Date: January 29, 2024
Published By: Audrey Tai, DO, MS, Athena Eye Care, Mission Viejo, CA. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
American Academy of Ophthalmology website. New treatments for retinitis pigmentosa. www.aao.org/eye-health/tips-prevention/gene-therapy-new-retinitis-pigmentosa-lca-luxturna. Updated August 16, 2021. Accessed February 20, 2024.
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Gregory-Evans K, Yang P, Pennesi ME. Retinitis pigmentosa and allied disorders. In: Sadda SR, Sarraf D, Freund KB, et al , eds. Ryan's Retina. 7th ed. Philadelphia, PA: Elsevier; 2023:chap 43.
Olitisky SE, Marsh JD. Disorders of the retina and vitreous. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 648.