General Medicine for the Optometrist
Dr Russell Watkins
Vasculitis The vasculitides, defined by inflammation of
the vessel wall are a heterogeneous group of diseases, affecting blood vessels of all types
Abnormal immune responses may be involved Vasculitis can be complicated by thrombosis
and severe cases result in permanent vessel wall damage
In milder cases the damage is transient & may be marked only by cellular infiltration & vessel wall damage by leakage of RBCs
Vasculitis Can be infective or non-infective Non-infective vasculitis sub-classified
according to size of BV involved Large, medium and small BVs Medium and small BVs Small BVs
Vasculitis Can also be classified according to
aetiology Hypersensitivity vasculitis (type III),
e.g. drug-induced, bacteraemia A component of a systemic
autoimmune disease, e.g. RA, SLE, sarcoidosis
The systemic vasculitides, e.g. PAN, WG, GCA
Giant Cell Arteritis Systemic disease Patients rarely less than 60yrs Affects ~1 in 1,500 per year of over-60s;
F>M (2:1) Large & medium muscular arteries (with
elastic tissue) affected, mainly in head & neck
Type IV hypersensitivity Patients will have temporal artery biopsy
and ESR measured
Giant Cell ArteritisSymptoms
Malaise Fever Anorexia Weight loss HEADACHE Tender temporal arteries & scalp May be jaw claudication or painful
tongue
Giant Cell Arteritis Complicated by
Aortitis Bowel infarct MI CVA
Associated with polymyalgia rheumatica Proximal muscle weakness & pain
Giant Cell Arteritis Ocular features
Anterior ischaemic optic neuropathy Less commonly, central retinal artery
occlusion Ocular palsies due to muscle or nerve
ischaemia Rarely, anterior segment ischaemia
Patients may complain of transient visual obscurations preceding AION
Polyarteritis Nodosa Necrotising inflammation affecting
medium & small arteries, associated with thrombosis
Affects ~1 in 10,000 per year; M>F (2:1)
Type III hypersensitivity ?Hepatitis B, CMV
Fatal unless treated with immunosuppression (90% remission)
Polyarteritis Nodosa General features
Kidney damage producing hypertension (often malignant) & renal failure
MI & angina Bowel & skin infarcts, including
erythema nodosum Arthritis Peripheral neuropathies
Polyarteritis Nodosa Ocular features
Necrotising scleritis/sclerokeratitis “Primary retinopathy” - vasculitis with
occlusion “Secondary retinopathy” - due to
hypertension Ischaemic optic neuropathy Transient focal detachments Rarely, retinal vessel aneurysms
Wegener’s Granulomatosis Necrotising vasculitis involving
Upper respiratory tract (haemoptysis) Kidneys (renal failure) Skin lesions Middle ear & sinuses
Wegener’s Granulomatosis Affects ~1 in 10,000; peak incidence in
adults 40-50 years of age; M>F (2:1) The cause is presumed to be immune
complex formation secondary to inhaled antigens
Necrotising, granulomatous arteritis with fibrinoid necrosis
Collagenolysis
Wegener’s Granulomatosis General features
Fever Weight loss Haemoptysis Runny nose and nasal mucosal
ulceration Nasal deformity Peripheral neuropathy Cerebral vasculitis
Wegener’s Granulomatosis 40-50% of patients may have ocular
involvement (mostly contiguous from the sinus or pharynx, but it may be isolated)
18-22% of patients demonstrate orbital involvement, usually bilateral
Treatment consists of immunosuppression
Improvement with systemic therapy is usual, with up to 90% remission
Wegener’s Granulomatosis Ocular features
Haemorrhagic conjunctivitis Episcleritis & scleritis Corneal infiltration & ulceration; guttering Nasolacrimal duct obstruction Orbital: proptosis & painful
ophthalmoplegia, chemosis, retinal venous congestion
Retinopathy: arterial narrowing, venous tortuosity, cotton wool spots, cystoid macular oedema, chorioretinitis
Sarcoidosis A multisystem granulomatous disease of
unknown aetiology; there is a deficiency of T-cells in blood but an excess in lung granulomas; ?Type IV hypersensitivity
Affects ~1 in 4,000; F>M (3:2); onset tends to be acute in younger adults and insidious in older adults
More common in New York African Americans and Ireland
Can spontaneously resolve, be progressive, or relapsing-remitting
Sarcoidosis Organ involvement
Lungs 95% Thoracic lymph nodes 50% Skin 30% Eyes 30%
10% present with ocular problems
Sarcoidosis
General features include No symptoms Fever, night sweats, weight loss Breathlessness Rashes including lupus pernio and
erythema nodosum Arthralgia Peripheral neuropathy Parotid gland enlargement & facial palsies
Sarcoidosis Ocular features
Eyelids - lupus pernio or nodules Band keratopathy Lacrimal gland enlargement Conjunctival follicles Nodular episcleritis & scleritis Acute non-granulomatous & chronic
granulomatous iridocyclitis Secondary cataracts & glaucoma
Sarcoidosis Ocular features (cont.)
Vitritis (classic sign) Choroiditis with white or yellow nodules Retinal periphlebitis with candle wax
exudates Retinal neovascularisation Pars planitis Choroidal & optic nerve granulomata Disc oedema
Rheumatoid Arthritis Chronic inflammatory condition Multi-system disorder characterised by
peripheral, symmetrical polyarthritis F>M Vasculitis part of the disease process
Rheumatoid Arthritis General features
Symmetrical arthritis Rheumatoid nodules - skin and lung Pericarditis Myopathy & neuropathy Vascular disease
Raynaud’s phenomenon Necrotising arteritis Skin ulceration Rarely organ infarction
Rheumatoid Arthritis Ocular features
Keratoconjunctivitis sicca Keratitis Peripheral corneal thinning - “contact
lens cornea” - may perforate Scleritis Episcleritis NB uveitis is NOT an association
Systemic Lupus Erythematosus
Multisystem autoimmune disease Autoantibodies to nuclear DNA Type III hypersensitivity Affects ~1 in 3,000; more common in
Asians and Afro-Caribbeans; F>M (8:1) Vasculitis part of the disease process
Systemic Lupus Erythematosus
General features Migratory symmetrical polyarthritis Renal failure & hypertension Skin
Butterfly rash Photosensitivity Discoid rash Raynaud’s phenomenon
Pleurisy & pericarditis Peripheral neuropathy & psychosis
SLE Ocular features of SLE
Eyelid erythema (with butterfly rash) Telangiectasia KCS Keratitis with peripheral corneal thinning Scleritis Retinopathy
Primary due to vasculitis - disc oedema + CWS Secondary – hypertension
Cranial nerve palsies
Sjögren’s Syndrome Chronic inflammatory autoimmune
disorder Characterised by mixed cellular
infiltration (plasma cells & lymphocytes with fibrosis) of lacrimal & salivary glands (amongst others)
Features Dry eye (xerophthalmia) Dry mouth (xerostomia) Dyspareunia & chest infections
Sjögren’s Syndrome Primary - the “sicca complex” Secondary - associated with
RA SLE Polymyositis/dermatomyositis Scleroderma PAN Et al
Vasculitis and the Optometrist Optometric management depends on ocular findings
and systemic symptoms If any systemic vasculitis is suspected, urgent
ophthalmic referral is indicated Have a high level of suspicion in elderly patients
with a new or ‘different’ headache Refer keratitis, scleritis, uveitis, orbitopathy, cranial
neuropathy and retinal vasculitis urgently If a disorder is suspected (but not diagnosed) for
another reason, refer to GP for medical investigation If patient is known to have a vasculitic disorder then
be on the lookout for possible ocular problems