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The ocular presentation of
systemic diseases
Data collected by : Ruwida Mohammed Saeed
The eyes are actually a part of the
human body sharing functions with
it , and affected by factors leading to
its deterioration …
It can be your mirror if you knew
how to use
Here are some of the main
complaints that are
commonly faced
Eye
presentation
Amaurosis
Fugax
Uveitis
Cataracts
Abnormal Eye
Movements
Pupillary
Abnormalities
Retinal vessel
disease
Corneal
effects
Eyelids
Amaurosis Fugax = is a
painless transient monocular
visual loss
Amaurosis
Fugax
Embolic
Vasospastic/
Haemodynamic
Neurologic
Ocular
Idiopathic
Amaurosis
Fugax
Embolic
Vasospastic/
Hemodynamic
Ocular
Idiopathic
Optic neuritis
• Rapidly developing impairment of vision in 1
eye or, less commonly, both eyes
• Dyschromatopsia ,may be more prominent than
the decreased vision
• Retro-orbital or ocular pain exacerbated by eye
movement
• Uhthoff phenomenon, in which vision loss is
exacerbated by heat or exercise
• Pulfrich phenomenon, in which objects moving
in a straight line appear to have a curved
trajectory: Presumably caused by asymmetrical
conduction between the optic nerves
Multiple sclerosis
is the most common
manifestation (usually unilateral,
but may be bilateral) and the
presenting feature in about 25% of
MS patients
About 60% of patients in the 20-
40 years age group who present
with ON will subsequently develop
evidence of systemic
demyelinisation !
Amaurosis
Fugax
Embolic
Vasospastic/
Haemodynamic
Neurologic
Ocular
Idiopathic
Amaurosis
Fugax
Embolic
Vasospastic/
Hemodynamic
Neurologic
Ocular
Idiopathic
Temporal Arteritis
Temporal arteritis
• is a systemic inflammatory vasculitis of
unknown etiology that affects medium-
and large-sized arteries.
The clinical criteria is :
1. New headache (localized temporal)
2. Abnormalities of the temporal arteries
(tenderness or decreased temporal artery
pulse)
3. (ESR) of 50 mm/h
4. Positive results of a temporal artery
biopsy
5. visual impairment may occur in as many
as 60% of patients
• The most common cause of
vision loss is anterior ischemic
optic neuropathy (AION). This
results from ischemia of the optic
nerve head
• optic neuropathy, central retinal
artery occlusion, branch retinal
artery occlusion
• diplopia, ptosis,nystagmus,
internuclear ophthalmoplegia
(INO),and pupillary
abnormalities. Branch retinal vein occlusion in a patient
with giant cell arteritis
Eyelids presentation
Dermatochalsis
Proptosis
Ptosis
Proptosis
• Infectious
• Orbital cellulitis . Mucormycosis ,
Concurrent sinus disease
• Inflammatory
• Thyroidopathy
• Vasculitis
• Wegener granulomatosis
• Churg-Strauss syndrome
• Neoplastic
• Lymphoma
• Leukemia
• Meningioma
• Glioma
• Ossifying fibroma
• Metastatic (breast in women, lung and
prostate in men, gastrointestinal, kidney
Dermatochalsis
• Chronic dermatitis , as of the chronic
inflammation leads to recurrent edema and
redundancy of the eyelid skin.
• Thyroid eye disease frequently associated
with dermatochalsis and steatoblepharon
,infiltration of the orbital fat and extraocular
muscles with immunoglobulin complexes.
• Chronic renal insufficiency can be associated
with periorbital edema. When chronic
edema can result in stretching of the eyelid
skin and redundancy of the eyelid
1. Soft tissue involvement
• Periorbital and lid swelling
• Conjunctival hyperaemia
• Chemosis
• Superior limbic keratoconjunctivitis
2. Eyelid retraction
3. Proptosis
4. Optic neuropathy
5. Restrictive myopathy
THYROID EYE DISEASE
Soft tissue involvement
Periorbital and lid swelling
Chemosis
Conjunctival hyperaemia
Superior limbic
keratoconjunctivitis
Signs of eyelid retraction
Occurs in about 50%
Bilateral lid retraction
Bilateral proptosis Lid lag in downgaze
Proptosis
• Occurs in about 50%
• Uninfluenced by treatment of hyperthyroidism
Axial and permanent in about 70% May be associated with choroidal folds
• Occurs in about 40%
• Due to fibrotic contracture
Restrictive myopathy
Elevation defect - most common Abduction defect - less common
Depression defect - uncommon Adduction defect - rare
Ptosis
• Any condition causing the upper eyelid to swell may cause ptosis
• Reduced Sympathetic Activity (Horner’s syndrome )
• Partial Ptosis , Miosis , anhydrosis
 Brainstem stroke or tumor , 33% of patients with brainstem lesions demonstrated
Horner syndrome
 Tumors (eg, Pancoast) or infection of the lung apex
 Dissecting carotid aneurysm
 Demyelinating disease (eg, multiple sclerosis)
 Central venous catheterization , Chest tubes
 Lymphadenopathy (eg, Hodgkin disease, leukemia, tuberculosis, or mediastinal tumors)
 Lesions of the middle ear (eg, acute otitis media
• Muscular , nerve , neuromuscular
junction : myotonic dystrophy ,
polymyositis, dermatomyositis , myasthenia,
Lambert–Eaton syndrome
• Oculomotor nerve palsy
 giant cell arteritis, Infection, cavernous
sinus thrombosis ,
 diabetes mellitus
 Miller Fisher syndrome, associated with
antibodies to ganglioside GQ1b In Miller
Fisher syndrome, extraocular muscle
involvement is the rule but there are
reports of isolated ptosis.
Pupillary Abnormalities
• Horner's syndrome
• Third nerve palsy
• Holmes-Adie syndrome
• Argyl Robertson
• Pontine stroke
Third nerve palsy
• Infectious meningitis - Bacterial,
fungal/parasitic, viral
• Carcinomatous/lymphomatous/l
eukemic infiltration,
granulomatous inflammation
(sarcoidosis, lymphomatoid
granulomatosis, Wegener
granulomatosis)
• Pituitary adenoma, meningioma,
craniopharyngioma, metastatic
carcinoma
• is a neurological
disorder characterized
by dilated pupil that reacts slowly
to light but shows a more definite
response to accommodation
• the classic signs of Argyll
Robertson pupil, are rigidity to
light and contraction to
accommodation
• it has been seen with herpes
zoster, sarcoid, von Beonomo's
encephalitis, diabetes, Lyme
disease, Wernicke's
encephalopathy.
• Tertiary syphilis
Holmes-Adie syndrome Argyl robertson pupil
The corneal changes and the systemic
disease
• Ulceration
• Collagen vascular disease:
rheumatoid arthritis, lupus
erythematosus, polyarteritis,
Sjögren's disease, Wegener's
granulomatosis
• Sjögren's disease (keratitis sicca,
marginal melting)
• Alterations in contour /
keratoconus
• Atopic diseases
• Down's syndrome
• Marfan's syndrome
• DEPOSITS
• Gout , myeloma
• Wilson's disease (dark copper
pigment in Descemet's membrane)
The lens changes are presented as cataracts
CATARACT
Metabolic
Other Systemic
Diseases Drug
Induced
DM
G6PD
Muscular
Dystrophy
NF 2
Dermatitis
Steroids
Two types of cataracts result from diabetes
Earlier onset age-related
• diabetic cataract ,more common
type is which occurs in type I or II
diabetics.
• It is very similar to other age-
related cataracts, and may
present as a cortical, posterior
subcapsular
snowflake cataract
• Rare, bilateral, occurs rapidly, and
related to very high, uncontrolled
DM in young type II diabetics
Uveitis
Systemic Dis
& Syndromes
Infections Infestations
TB
Candida
Herpes Zoster
Uveitis
Systemic Dis
&
Syndromes
Sarcoid
BehcetsReiter's
Ankylosing
Spondylitis
Crohn's
Dis UC JCA
Uveitis
Systemic Dis
& Syndromes
Sarcoid
BehcetsReiter's
Ankylosing
Spondylitis
Crohn's
Dis
Ulcerative
colitis
JCA
Ulcerative colitis is characterized severe diarrhea and
cramps , Fever ,Leukocytosis , Abdominal distention
UC is associated with various extracolonic
manifestations, as follows:
• Uveitis
• Pyoderma gangrenosum
• Pleuritis
• Erythema nodosum
• Ankylosing spondylitis
• Spondyloarthropathies
Uveitis
Systemic Dis
&
Syndromes
Sarcoid
BehcetsReiter's
Ankylosing
Spondylitis
Crohn's
Dis UC JCA
Systemic Dis
& Syndromes
Sarcoid
BehcetsReiter's
Ankylosing
Spondylitis
Crohn's
Disease
UC
Rheumatoid
arthritis
Uveitis
Systemic Dis
& Syndromes
Sarcoid
Behcets
Reiter's
Ankylosing
Spondylitis
Crohn's
Dis UC JCA
Sacroilitis
33% of pts of AS present with acute anterior uveitis …
Systemic Dis
& Syndromes
Sarcoid
Behcets
Reiter's
Ankylosing
Spondylitis
Crohn's
Dis UC JCA
Systemic Dis
& Syndromes
Sarcoid
Behcets
Reiter's
Ankylosing
Spondylitis
Crohn's
Dis UC JCA
Ocular involvement in Behçet disease
• Ocular involvement is seen in about 70% of patients
• In most cases, the ocular symptoms follow the oral
and genital ulcers by 3-4 years, although ocular
disease is the initial manifestation in about 20% of
cases.
• The classic finding of Behçet disease, iridocyclitis
with hypopyon, is present in about one third of
cases.
• Gonioscopy may reveal an occult hypopyon
characterised by hypopyon that it may change
position with head movement, and it may form
and disappear rapidly without sequelae.
• Recurrent attacks may result in posterior
synechiae, peripheral anterior synechiae, iris
atrophy, and secondary glaucoma.
A 19-year-old boy presenting with vitreous
haze, and retinal infiltrates due to Behçet’s
disease.
• The classic fundus finding is retinal vasculitis
• retinal hemorrhages, yellow-white exudates deep in the
retina, white focal retinal infiltrates, retinal edema, and
optic disc edema with hyperemia.
• Retinal neovascularization, secondary to either retinal
vein occlusion or chronic inflammation, may result in
retinal or vitreous hemorrhage.
• Neovascular glaucoma
• Vitreous cellular infiltration almost always is present
during the acute phase.
• Repeated episodes of posterior segment inflammation
cause sheathing of retinal vessels, chorioretinal scars, and
retinal and optic nerve atrophy.
Uveitis
Systemic Dis
& Syndromes
Sarcoid
BehcetsReiter's
Ankylosing
Spondylitis
Crohn's
Dis UC JCA
Unknown
Aetiology granuloma
SARCOIDOSIS
Systemic Involvement
• Lung lesions – 95%
• Thoracic lymph nodes – 50%
• Skin lesions – 30% 
• Eyes – 30%
SARCOIDOSIS
Ocular Involvement
• Conjunctival granuloma
• Lacrimal gland involvement/dry
eye
• Acute or chronic uveitis 
• KPs described as ‘mutton fat’
because they are large and greasy
• Chorioretinal granulonmas
• Vasculitis including occlusive causing:-
• Neovascularisation
• Infiltrate in vitreous (vitritis)
• Optic nerve head granuloma
The retina is affected by many diseases , but
thecommonest are diabetes , hypertension ,
Capillary damage
Pericyte necrosis
(capillaries and veins)
Endothelial damage
Capillary non-perfusion
Retinal ischaemia
Vasoactive factors
Hyperperfusion
Abnormal autoregulation
Hypertension
New vesselsGrowth factors
Hyperglycemia
Diabetic retinopathy
• Diabetic retinopathy is the leading cause of new blindness in persons aged
25-74 years in the United States . And classified as :
Background diabetic retinopathy
Nonproliferative diabetic retinopathy
proliferative diabetic retinopathy
Maculopathy
Background
Retinopathy
Dot
Haemorrhages
Hard Exudates
NORMAL eyesight
-present in almost all who have had type 1 diabetes for 20 years
-present in nearly 80 percent of those with type 2 disease for 20 years
Micro-
aneurysms
Pre-prolif
Retionopathy
Cotton Wool
Spots
Venous
Irregularity
NORMAL EYESIGHT
Pre-prolif
Retionopathy
REFER!
50% of patients progress to
proliferative retinopathy within 1-2 years
Proliferative
Retinopathy
Neo-
vascularisation
NVD
NVE
NORMAL
EYESIGHT,
but
threatened....
Maculopathy
Early and
Diffuse
Severe and
Ischaemic
EYESIGHT
AFFECTED
ANTERIOR SEGMENT COMPLICATIONS OF
DIABETES
Recurrent, non-healing corneal
epithelial defects
Neovascular glaucoma
Diabetes lowers the resistance of the
patient to pyogenic infections and
predisposes to recurrent styes
Transient Refractive Changes and diabetes
Accommodation Disorders.
Weakness of accommodation may result when the ciliary muscle
becomes weakened by peripheral neuritis involving its nerve supply.
Subjective Visual Disturbances.
Amaurosis may occur in severe cases of diabetes. Disturbances of the
visual cortex may lead to misty vision and to lashes of light which patients
may experience after an overdose of insulin. .
Hypertension
Grade I
Grade II
Grade III
Grade IV
Papill-oedema
HaemorrhagesCotton
Wool
Spots
A-V
Nipping
Silver
Wiring
Abnormal eye movement
Nystagmus
• Seesaw nystagmus
• Rostral midbrain lesions - Parasellar
lesions (eg, pituitary tumors)
Downbeat nystagmus Lesions of the vestibulocerebellum
and underlying medulla, including the following:
• Demyelination (eg, multiple sclerosis)
• Microvascular disease with vertebrobasilar insufficiency
• Brain stem encephalitis
• Tumors at the foramen magnum (eg, meningioma, cerebellar hemangioma)
• Nutritional (eg, Wernicke encephalopathy, parenteral feeding, magnesium deficiency)
Restricted eye movement
1. Cranial Nerve Disease (palsy/paresis, congenital/developmental anomaly,tumor,
vascular, inflammatory, trauma, immune, etc.)
2. Neuromuscular Junction (Myasthenia, etc)
3. Muscular Disease (congenital/developmental anomaly, tumor, vascular,
inflammatory, trauma, immune, etc.)
4. Orbital Disease (pulley, congenital/developmental, tumor, vascular,inflammatory,
trauma, immune, etc.)
Thank you

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