Systemic disease and the eye Flashcards
What is the most common of all systemic diseases in eye clinics?
diabetes mellitus
What forms of sight impairment registration is diabetes a major cause of?
Both blind (severely sight impaired) and partial sighted (sight-impaired) registration in people under 65
What is a key endocrine disease associated with eye disease?
Graves’ disease - form of hypERthyroidism
What is ophthalmic Graves’ disease?
Clinical changes of Graves’ disease in the eye/orbit but clinically and biochemically euthyroid
What (briefly) is the physiology underlying Graves’ disease of the eye?
Immunologically mediated disease in which circulating auto-antibodies attack structures of the orbit and extra-ocular muscles
What is the key effect of Graves’ disease on the eye?
Inflammation of orbital and periorbital tissues
What are 5 EYE symptoms that a patient with Graves’ disease may present with?
- Exophthalmos (aka proptosis)
- Grossly swollen extra-ocular muscles –> restricted eye movements
- Lid retraction
- Lid lag
- Blindness
What causes exophthalmos/proptosis in Graves’ disease?
inflamed tissue in orbit (extra-ocular fat or peri-orbital muscles) forces the globe forwards; the orbit is an enclosed space, so the only direction for expansion is forwards.
Which extra-ocular muscles are particular affected by gross swelling in Graves’ disease?
medial and inferior recti
What is a consequence of the grossly swollen extra-ocular muscles?
Restricted eye movements
What is lid retraction?
Abnormally raised position of upper lid
What is lid lag?
Delayed following of upper lid as patient looks down
What are 2 things that can cause blindness in
- Optic nerve damage
2. Corneal perforation
What can cause optic nerve damage leading to blindness in Graves’ disease?
inflamed tissue in the orbit which compresses the nerve (irreparable)
What can cause corneal perforation leading to blindness in Graves’ disease?
eyelids and tear film provide barrier to surface of globe; when pushed forwards, lids can’t completely close –> drying, increased risk of infection, thinning and eventual perforation of the cornea
Where do the upper eyelids normally come down to (that is disrupted in graves’ disease: retraction)?
Normally rests just over the upper part of the cornea, covers the superior limbus (junction between cornea and sclera) - in Graves’ disease, can see continuous rim of sclera round cornea (see image)
What causes restricted eye movements in Graves’ disease?
Inflammation of infra-orbital and peri-ocular tissues, restricting movement of the globe
Look at an MRI of the orbit in Graves’ disease - how can the extra-ocular muscles be identified? What will be abnormal?
4 petal like structures = extra-ocular muscles just posterior to the globe. Grossly enlarged due to circulating auto-antibodies
Why can anything in neurology cause ophthalmic disease?
ophthalmology comprises the eye and visual system, and one third of the brain is devoted to the visual system –> any lesion that interferes with the visual pathways may present to an ophthalmologist
What are 8 neurological conditions with big implications for the eyes?
- CNIII palsy
- CNIV palsy
- CNVI palsy
- Horner’s syndrome
- Multiple sclerosis –> optic neuritis
- Myasthenia gravis
- Myotonic dystrophy
What are the three key symptoms of Horner’s syndrome, plus three additional symptoms?
- meiosis (small pupil), non-reactive pupil, anisocoria (different sized pupils)
- ptosis
- anhydrosis
- eye sunken back into socket
- facial flushing
- inability to completely close or open eye
What causes Horner’s syndrome?
Disruption in the pathway in the sympathetic nervous system i.e. compression of sympathetic chain (from hypothalamus to face and neck)
What are 3 points at which the cause of Horner’s syndrome can occur? Give examples for each.
- First order neuron: arises at hypothalamus –> through brainstem to upper spinal cord. [stroke, tumour, syringomyelia]
- Second order neuron: upper spinal cord, across upper thorax and into neck [Pancoast’s tumour in lung apex, schannoma, aortic disease]
- Third order neuron: neck to facial skin, muscles of eyelids and iris [base of skull tumour/ infection, cluster headaches/migraines, carotid artery dissection]
What 2 ocular symptoms will there be in myasthenia gravis?
Ptosis, ocular motility problems
What 3 ocular symptoms will there be in myotonic dystrophy?
Ocular motility problems and ptosis, double vision due to problems with eye movements and use of extraocular muscles
What position will the eye be in in a unilateral third nerve palsy?
Down and out
What is the main concern of the underlying cause in Horner’s syndrome?
carotid artery dissection
What visual problems can be caused by a pituitary adenoma?
pituitary located close to optic chiasm –> bitemporal visual field loss (in practice usually slightly different in each eye: incongruous temporal hemianopia)
What ophthalmic disease can hypertension cause in the eye?
hypertensive retinopathy
What ophthalmic diseases can arteriosclerosis and atherosclerosis lead to?
anterior ischaemic optic neuropathy (cause of artery and vein occlusions)
What can systemic hyperlipidaemia lead to in the eye?
greater chance of hard exudates in diabetic retinopathy
What can embolic phenomena systemically lead to in the eye?
- Central retinal artery occlusion
- Branch retinal artery occlusion
–> both are essential strokes, treated with referral for stroke review - Central retinal vein occlusion
Branch retinal vein occlusion
What are 9 rheumatological diseases which may cause eye disease?
- Rheumatoid arthritis
- Seronegative arthritides HLA-B27 positive e.g. ankylosing spondylitis, Reier’s disease
- Sjögren’s syndrome
- Juvenile idiopathic arthritis
- Behcet’s disease
- Sarcoidosis: intraocular inflammation
- SLE
- Granulomatosis with polyangiitis
- Giant cell arteritis
What type of eye disease can rheumatoid arthritis cause?
scleritis, episcleritis
What type of eye disease can HLA-B27 positive seronegative arthritides cause?
anterior uveitis
What type of eye disease can Sjögren’s disease cause?
Dry eye
What type of eye disease can juvenile idiopathic arthritis cause?
anterior uveitis in children
What type of eye disease can Behcet’s disease cause?
Devastating panuveitis
What type of eye disease can sarcoidosis cause?
intraocular inflammation
What are dry eyes in rheumatological disease often a sign of and what can they cause?
rheumatoid arthritis, can cause secondary Sjögren’s
What rheumatological disease is usually the cause of episcleritis?
Rheumatoid arthritis
What are the symptoms of episcleritis? see image
- localised area of superficial redness
- not particularly painful, may have some discomfort
- SEGMENTALLY red rather than entire sclera (e.g. just nasal or temporal aspect)
How is episcleritis (usually secondary to rheumatoid arthritis) usually treated?
Lubricants or short course of anti-inflammatory medication
What are 4 diseases that uveitis can be secondary to?
- Behcet’s
- Sarcoidosis
- Juvenile idiopathic arthritis
- Seronegative arthropathy HLA-B27+
What rheumatological disease can retinal vascular occlusion occur secondarily to?
SLE
What rheumatological disease can orbital disease/ inflammation occur secondary to?
Granulomatosis with polyangiitis
What disease can anterior ischaemic optic neuropathy occur secondary to, and what else can this present with?
Giant cell arteritis (can also present with CRAO)
What are the 2 key symptoms of scleritis and how is it different from episcleritis?
- severe pain - patient in agony
2. redness - different quality from episcleritis, much deeper vessels dilated
What type of scleritis causes problems for ophthalmologists and why?
Posterior scleritis - little to see at front, patients still complain of discomfort
How might evidence of posterior scleritis be able to be detected?
Ophthalmoscopy: choroidal folds
What conditions can corneal melt be associated with?
Rheumatoid arthritis, and other inflammatory conditions
Look at an image showing corneal melt. How can it occur?
Dilation of local blood vessels at limbus (junction sclera-cornea), area line of whiteness, round shaped: peripheral infiltrate at the limbus and area of thinning where cornea is disintegrating
What are the dangers of corneal melt?
sight and eye threatening: can rapidly progress to corneal perforation/intra-ocular inflammation
What is the management of corneal melt?
- needs urgent referral to ophthalmologist and treatment with systemic high dose immunosuppressant medication
- corneal graft
When might a corneal graft be needed?
In cornea melt, may be left with very thinned or melted area of the cornea, possible to patch graft the area
How does a corneal graft work following corneal melt?
Small, circular button applied over the area of thinning (see image)
What are 3 characteristic features of sarcoidosis to look out for in the case of uveitis (to see if sarcoidosis is underlying cause)?
- noncaseating epithelioid granulomas in affected tissues
- affects YOUNG adults of either sex
- most often begins as bilateral hilar lymphadenopathy, pulmonary infiltrates, and skin and eye lesions
- erythema nodosum
- wheezing, cough, SOB if lungs; fever, large lymph nodes, rash, EN –> Löfgren syndrome
What are the classical features of ankylosing spondylitis?
gradual onset low back pain, radiating from sacroiliac joints to buttocks, progressive loss of spinal movement, enthesitis (e.g. Achilles tendonitis, plantar fasciitis)
What type of eye disease is ankylosing spondylitis associated with?
Recurrent bilateral sequential anterior uveitis (affects one eye, then the second eye, etc.)
How is acute anterior uveitis detected in ankylosing spondylitis?
by ophthalmologist with slit lamp by microscopy
What 2 additional features may be seen in acute anterior uveitis in ankylosing spondylitis?
Posterior synechiae
Hypopyon
What does an irregular pupil in acute anterior uveitis indicate?
posterior synechiae: caused due to inflammatory exudate in anterior chamber, adhesions form between iris and anterior lens capsule –> eye tries to dilate and links have formed between iris and anterior lens; iris may even pull away and leave lump of pigment stuck to lens
What is the treatment for posterior synechiae in acute anterior uveitis in ankylosing spondylitis?
topical cycloplegic (paralysis of ciliary muscle, loss of accommodation) such as cyclopentolate, to dilate eye
What is Behcet’s disease?
idiopathic multisystem disorder
What can Behcet’s disease lead to that involves the eyes in 75% of cases?
Occlusive vasculitis
At what age does Behcet’s disease present?
30s and 40s
How is a diagnosis of Behcet’s disease made?
ORAL ULCERATION with two of the following 4:
- recurrent genital ulceration
- skin lesions - folliculitis, erythema nodosum
- Positive pathergy test (pustule reaction following breaking of skin with needle)
- Eye involvement
What are 4 forms of eye involvement that may be seen in Behcet’s disease?
- Acute anterior uveitis
- Vitritis
- Vasculitis
- Retinitis
What is the treatment of Behcet’s disease?
Systemic immunosuppression
What is the danger of Behcet’s disease in terms of eye disease in teh young adult?
devastating, potentially blinding eye disease
What part of the world is Behcet’s disease most common in?
not UK, tends to affect countries in the old silk route - China to Mediterranean
What are 2 examples of possible features on examination of eye disease seen in Behcet’s?
- hypopyon
- retinal vasculitis on dilated examination: areas of sheathing of blood vessels (can see in fundal fluorescein angiogram: sheathed vessels, leaking)
What causes the risk of blindness in giant cell arteritis?
anterior ischaemic optic neuropathy
What is the risk of systemic involvement in giant cell arteritis?
could lead to strokes or MIs
what often precedes giant cell arteritis?
amaurosis: painless temporary loss of vision; may be partial or total blindness
What are the key symptoms of GCA?
temporal headaches, jaw claudication, weight los, night sweats, transient visual obscuraions
What rheumatological disease is GCA associated with?
polymyalgia rheumatica
What proportion of cases of GCA will affect the other eye if not diagnosed and treated?
70%
What are 8 skin diseases that can have implications for the eyes?
- Acne rosacea - abnormal flushing of face
- Topical allergy
- Stevens-Johnson syndrome (erythema multiforme)
- Psoriasis
- Herpes zoster ophthalmicus
- Mucous membrane pemphigoid
- Sturge-Weber (port wine stain + intracranial arteriovenous malformations)
- Lid tumours: BCC, SCC
What eye problems can acne rosacea cause?
problems to lid margin–> blepharitis, reduced tear film, secondary severe dry eye disease
How can topical skin allergy affect the eyes?
Can affect conjunctiva
What effect can Stevens-Johnson have on the eyes?
Ocular surface problems
What eye disease is psoriasis associated with?
Uveitis
How does herpes zoster ophthalmicus affect the eyes?
shingles affecting ophthalmic branch of trigeminal nerve –> also innervates the eye, chance of ophthalmic shingles and inflammation within the eye
How can mucous membrane pemphigoid affect the eyes?
ocular surface disease
How can Sturge-Weber disease affect the eyes? 2 key things.
- conjunctival, episcleral, choroidal haemangiomas
2. Glaucoma (due to vascular formations in trabecular mesh-work) in 50%
What is acne rosacea?
ubiquitous seborrheic disorder of the Meibomian glands
Where in the eye are Meibomian glands located? What do they do?
lid margin at the base of eye lashes, normally secrete oily layer onto tear film to prevent evaporation of aqueous
What happens if the Meibomian glands of the eyelids become blocked?
causes mild gritty sensation; bacteria which normally live within lid margins can proliferate, get secondary hypersensitivity reaction on the surface of the eye –> blepharitis
What is the treatment of blepharitis, which an occur secondary to acne rosacea? 2 key aspects
- lid hygiene: hot compresses across eye for 5-10 minutes twice a day, wipe around eyelid with cotton wool bud and cool bowl of water to try and remove some of debris from the glands
- oral tetracyclines: e.g. doxycycline 50-100mg daily for up to 6 months
What is Stevens Johnson syndrome?
Acute reaction to drugs and infection; Fas-FasL mediated keratinocyte apoptosis
What are 4 features of the prodrome to Stevens-Johnson syndrome?
Fever, malaise, sore throat, arthralgia
What are 2 possible effects of Stevens-Johnson syndrome on the eyes?
- Pseudomembranous conjunctivitis
2. Cicatrisation (scarring) of the conjunctiva and lids
What is cictrisation?
abnormal adhesions between the conjunctiva and eyelid leading to a loss of the usual fornices, disturbance of ocular sufrace
Whta are 3 things that can be found on the skin in Stevens-Johnson syndrome?
- erythematous maculopapules
- vesicobullous lesions
- skin denudation
What are 3 types of lesions that may be found in the mouth in Stevens-Johnson syndrome?
- bullae
- erosions
- haemorrhagic crusts
What is an important area to check on examination in herpes zoster ophthalmicus and why?
Check the nose on the affected side: if vesicles along side of the nose, suggests ciliary branch involvement –> greater risk of ocular involvement
What are 3 possible ocular complications of herpes zoster ophthalmicus?
- Keratitis
- Uveitis
- Glaucoma
What is mucous membrane pemphigoid?
rare, idiopathic, chronic progressive immune-mediated disease. Leads to inflammation followed by scarring
What gender is primarily affected by mucous membrane pemphigoid?
females
What is type 1 mucous membrane pemphigoid?
recurrent vesicobullous, non-scarring
What is type 2 mucous membrane pemphigoid?
localised erythematous plaques, with recurrent vesicles and bullae, healing to small atrophic scars
What can occur in the eye in the acute phase of mucous membrane pemphigoid?
Inflamed ocular surface
What can occur in the eye after resolution of inflammation in mucous membrane pemphigoid?
peripheral scarring
What mouth lesions may be present in mucous membrane pemphigoid?
submucosal blisters leading to erosions, scars and strictures
In what proportion of patients with mucous membrane pemphigoid is there skin involvement?
25%
In what proportion of patients with mucous membrane pemphigoid is there mouth involvement?
80%
In what proportion of patients with mucous membrane pemphigoid is there eye involvement?
50-70%
What are 3 types of eye problems experienced by people with mucous membrane pemphigoid?
- dry eye
- symblepharon
- keratopathy
What is a symblepharon (which occurs in mucous membrane pemphigoid)?
adhesions between lid and globe
What may happen to the cornea and conjunctiva in mucous membrane pemphigoid?
opaque cornea due to severe infiltrate and inflammation. injected conjunctiva surrounding cornea
What is the treatment for mucous membrane pemphigoid? 4 aspects
- topical and systemic steroids
- immunosupressants
- contact lenses
- lid surgery
What are 3 non-ocular features of Sturge Weber syndrome?
- Port-Wine stain
- Seizures
- Mental retardation (intracranial AVMs)
What can basal cell carcinoma of the lid margin cause?. 3 things. Look at image
- lid distortion of lid margin
- central ulceration
- telangectasia
What is the prognosis of lid BCCs?
do very well if removed early
What are 4 types of genitourinary diseases that implicate the eye and their ocular effects?
- chlamydia - conjunctivitis
- gonorrhoea - conjunctivitis
- HIV - intraocular inflammation, CMV (cytomegalovirus) retinitis
What does conjunctivitis cause in children?
ophthalmia neonatorum - conjunctivitis of newborn
When should you consider that conjunctivitis might be chlamydial conjunctivitis?
if conjunctivitis is not improving with treatment/ usual time course
What key clinical feature is chlamydial conjunctivitis associated with?
Pre-auricular lymphadenopathy
What is a treatment for chlamydia?
single dose azithromycin 1g (and refer to GUM)
When is gonorrhoea (which can cause conjunctivitis) most worrying?
when it occurs in the neonatal period
What can conjunctivitis due to gonorrhoea lead to?
can penetrate through the intact cornea, leading to intraocular problems
What happens in HIV leading to eye problems?
opportunistic infections as CD4 counts drop, especially CMV (cytomegalovirus)
What are 2 examples of non-ocular HIV-defining diseases?
- oral hairy leukoplakia
2. Kaposi’s sarcoma
What are 2 features of CMV retinitis that occurs as an opportunistic infection with HIV?
haemorrhage and infiltrate
What are 2 GI diseases that can cause eye problems and what eye disease do they lead to?
Crohn’s and ulcerative colitis –> both can cause anterior uveitis
What is a respiratory example of a disease that can have ocular manifestations?
Sarcoidosis (look out for bi-hilar lymphadenopathy) –> uveitis
What are 2 renal diseases that can have ocular manifestations?
- SLE
2. Granulomatosis with polyangiitis
What are 7 examples of drugs that can lead to ocular disease?
- corticosteroids
- hydroxychloroquine
- desferrioxamine
- tamoxifen
- quinine
- amiodarone
- vigabatrin
What 2 ocular side effects can corticosteroids have?
- posterior subcapsular cataract
2. secondary glaucoma
What ocular side effect can hydroxychloroquine have, and what should be done as a result?
maculopathy (need to be screened on an annual basis)
What ocular side effect can desferrioxamine have?
maculopathy
What ocular side effect can tamoxifen have?
maculopathy
What 2 ocular side effects can quinine have?
optic atrophy, arteriolar narrowing
What are 2 ocular side effects that amiodarone can have?
- corneal change - deposition of crystals
2. Vortex keratopathy (characteristic swirls on cornea)
What ocular side effect can vigabatrin have?
Visual field loss
