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