Retinitis Pigmentosa
ORPHA791
ICD-10 H35.5
UMLS C0035334
MedDRA 10038914
Status Under evaluation
What is retinitis pigmentosa
Retinitis pigmentosa is a group of hereditary diseases of the eye that lead to progressive loss of sight. In patients with retinitis pigmentosa, cells in the retina (the light-sensitive surface at the back of the eye) become damaged and eventually die.
Retinitis pigmentosa is a long-term debilitating disease because it causes the patient’s sight to get worse, eventually leading to blindness.
What is the estimated number of patients affected by the condition?
Retinitis pigmentosa affects less than 3.7 in 10,000 people in the European Union (EU). This is equivalent to a total of fewer than 190,000 people, and is below the ceiling for orphan designation, which is 5 people in 10,000.
What treatments are available?
No satisfactory methods are authorised in the EU for treating retinitis pigmentosa. Patients with the condition are given sunglasses to slow down the damage to the retina, genetic counselling (discussion of the risks of passing the condition on to children) and general support.