RETINITIS PIGMENTOSA

AND CHLOROQUINE
RETINOPATHY
NANDANA JIDESH
ROLL NO: 66
RETINITIS
PIGMENTOSA
Retinitis pigmentosa, primary pigmentary retinal dystrophy is
a heriditary dystrophy is a heriditary disorder predominantly
affecting the photoreceptors
INHERITANCE
 1. Sporadic disorder, isolated without family history due to mutation of multiple
gene including rhodopsin gene
 Inherited disorder as
 Autosomal recessive (AR), most common (25%),intermediate severity
 Autosomal dominant (AD), next common (25%),least severe
 X-linked (XL), least common (10%), most severe.
 Digenic inheritance is very rare
PATHOGENESIS
 Majority of conditions are characterised by death of old
photoreceptors
 Molecular mechanism is not clear
 Ample evidence indicates that apoptosis is the final pathway of
cell death
 Cone photoreceptors ultimately die from a disease that begins
with rod cell disease
CLINICO INVESTIGATIVE
FEATURES
 Type 1 RP – early and preferential loss of rod sensitivity
 Early signs- diminished night vision
 Progress slowly
 Leads to region specific vision loss

 Type 2 RP – progressive and combined loss of rods and cones sensitivity

 Adult onset
 Later onset of diminished night vision
VISUAL SYMPTOMS
 Night blindness
 Characteristic and earliest feature
 Due to degeneration of rods

 Dark adaptation
 Light threshold of peripheral retina is increased
 Dark adaptation not affected until very late

 Tubular vision – loss of peripheral vision with preservation of central vision

 Central vision lost after many years
 Photopsia – flashes of light
FUNDUS CHANGES
 Retinal pigmentary changes- perivascular
and jet black spots
 Retinal arterioles are narrowed
 Thinning and atrophy of retinal pigment
epithelium
 Optic disc becomes pale and waxy
 Consecutive optic atrophy
 Other changes
 colloid bodies
 Choroidal sclerosis
 Cystoid macular edema
VISUAL FIELD CHANGES
 Annular or ring shaped scotoma – corresponds to
degenerated equatorial zone of retina
 As disease progresses scotoma increases
anteriorly and posteriorly and ultimately only
central field is left
 Eventually this is also lost and patient becomes
blind
ASSOCIATIONS
 Ocular association
 Myopia
 Primary open angle glaucoma
 Keratoconus
 Posterior subcapsular cataract

 Syatemic associations
 Laurence- Moon-Biedl syndrome- retinitis pigmentosa, obesity, hypogenitalism, polydactyly, mental
retardation
 Cockayne’s syndrome
 Hallgren’s syndrome
ATYPICAL FORMS
 Retinitis pigemtosa sine pigmento- all clinical features except there are no visible pigemtary
changes in fundus
 Sectorial retinitis pigmentosa- involvement of only one sector of retina
 Preicentric retinitis pigmentosa- all clinical features except pigmentary changes are confined
to an area immediately around macula
 Retinitis pigmentosa albescens- presence of innumerable white dots scattered over fundus
without pigmentary changes
TREATMENT
 Measures to stop progression
 Vasodilators
 Placental extracts
 Light exclusion therapy
 Vit A

 Glasses for correction of any refractive errors

 Systemic acetazolamide – for cystoid macular edema
 Stem cell therapy
 Gene therapy trials
 Retinal prosthesis
 Low vision aids
 Rehabilitation
 Prophylaxis
CHLOROQUINE
RETINOPATHY
It is an antimalarial drug

Used in the treatment of lupus erythematosus and arthritis

The prolonged use of chloroquine may cause keratopathy,

myopathy and retinopathy.
FUNDUS EXAMINATION
 A mild pigmentary disturbance
leads to the characteristic
“bulls eye” lesion in the macular
area.
 There is widespread retinal atrophy
with clumps of pigment and
attenuated retinal vessels seen in
the later stage.
Hydroxychloroquine, which has a lower risk of ocular
toxicity than chloroquine, is perceived to be a safer drug

•The maximum dose allowed for chloroquine is 6 mg/kg in

24 hours while for hydroxychloroquine it is 4.5 mg/kg.

•Careful perimetry, preferably static, with determination

of threshold sensitivities within 5° of the fixation point with
red stimuli is a reliable method of detecting the early signs
of chloroquine retinal toxicity.
THANK YOU