Ankylosing spondylitis

About 50 per cent of Caucasian patients who are HLA-B27 positive will have some signs of ankylosing spondylitis. Young adult males are predominantly affected with a male:female ratio of 3:1. Attacks of anterior uveitis occur acutely, usually in one eye and are not related to exacerbations of joint disease, its severity or any other known predisposing factor. The attacks usually subside over 4–6 weeks with treatment and with prompt treatment do not tend to cause residual ocular damage. Most patients can expect several attacks of acute anterior uveitis in either eye before the disease burns out in later life but the degree to which any one patient is affected is extremely variable. There is evidence that immune cross-reactivity to bowel flora may have a pathogenetic role.

Fig. 10.15 About 50 per cent of HLA-B27-positive patients will have evidence of ankylosing spondylitis which is seen in its earliest form in the sacroiliac joints. There is sclerosis of the periarticular bone with narrowing and irregularity of the joint space, progressing eventually to ankylosis. Similar changes are seen in the spine.

Fig. 10.16 This patient has the typical kyphoscoliotic posture of ankylosing spondylitis. He has had a total hip replacement for ankylosis of the joint. Note the swelling of the right knee and the deformity of the feet.

Reiter’s syndrome

This syndrome is a triad of arthritis, urethritis or dysentery, and acute anterior uveitis. Typically a seronegative (i.e. rheumatoid factor negative) arthritis affecting large peripheral joints such as the knees and the sacroiliac joints follows a few weeks after bacterial dysentery or a nonspecific urethritis. Conjunctivitis and uveitis occur in 30–50 per cent of patients. Other systemic features are plantar fasciitis and keratoderma blennorhagica on the penis, palms or soles of the feet. Aortic incompetence is a rare late complication. Males are usually affected; HLA-B27 is virtually always present and there is evidence that the disease is related to chlamydial genital infection if it follows a nonspecific urethritis. Partial manifestations of the syndrome are common. In most patients the uveitis recovers over a few weeks but the arthritis can progress to chronic joint destruction.

Fig. 10.17 Typical keratoderma blennorhagica of the penis.

Herpes zoster ophthalmicus

Keratitis (see Ch. 4) and anterior uveitis are common features of herpes zoster ophthalmicus and may occur independently of each other. It is said that keratitis and uveitis are particularly frequent if the vesicles appear along the side of the nose, the cutaneous distribution of the nasociliary nerve that also innervates the iris and pupil but this is not invariably so.

The uveitis is frequently subacute in onset and is often associated with keratitis. It may persist for many months. Sector atrophy of the iris is commonly seen and is due to an occlusive vasculitis of the iris vessels; retroillumination of the iris shows sectorial translucency to advantage. Corneal anaesthesia is commonly present and persists. Ocular nerve palsies and optic neuritis are occasionally seen and post-herpetic neuralgia can be disabling in a minority of patients. Oral antiviral agents should be given in the acute vesicular cutaneous stage of the disease. The rash heals more quickly with less post-herpetic neuralgia and a lower incidence of ocular involvement. The severity of the cutaneous disease does not necessarily correlate with the degree of severity of ocular involvement. Herpes zoster in young patients may indicate an underlying systemic immunosuppressive illness such as human immunodeficiency virus (HIV) infection or malignancy.

Fig. 10.18 Herpes zoster ophthalmicus with involvement of the nasociliary nerve along the lateral border of the nose. Cutaneous lesions may vary in severity from confluent vesicles to the occasional punctate lesion.

Fig. 10.19 Sectorial iris atrophy following herpes zoster uveitis. Retroillumination of the iris would show loss of iris pigment epithelium.

Juvenile idiopathic arthritis

Juvenile idiopathic arthritis can be divided into three main subtypes according to the presenting pattern of joint disease. These are the systemic, polyarticular and pauciarticular types. Ocular involvement is seen most commonly in the latter group, particularly in girls, and especially if antinuclear antibodies are present. HLA-DR5 carries an increased risk of ocular involvement. Occasionally the uveitis may precede joint symptoms by up to 2 years. Conversely development of uveitis after 5 years of joint disease is unusual. Typically the ocular disease is bilateral and the eye is completely white and painless. Accordingly there is a need to screen such children on a regular basis, particularly those at high risk. Visual loss occurs from band keratopathy, glaucoma or cataract. Many patients can be treated by topical or local steroid orbital injections; methotrexate is useful to control both the arthritis and eye disease without the problems of growth retardation from systemic steroids. Cataract surgery is frequently required; IOL implantation remains highly controversial in these children and at present is probably best avoided. Secondary glaucoma is a serious complication that responds poorly to standard medical treatment; surgical drainage with antimetabolites has considerably improved the prognosis.

Fig. 10.20 This little girl aged 4 years with pauciarticular disease has a swollen right knee on which she cannot weight bear.

By courtesy of Dr B Ansell.

Fig. 10.21 Typical features of chronic anterior uveitis in juvenile idiopathic arthritis. There is a moderately advanced band keratopathy with posterior synechiae and a dense cataract. There is no conjunctival injection.

By courtesy of Mr J Kanski.

Fuchs’ heterochromic cyclitis

This is a distinctive entity with many features not seen with other forms of uveitis. Small diffuse KPs are scattered over the whole of the corneal endothelium with a fluffy or feathery appearance of their border in contrast to the well circumscribed and inferiorly sited KPs seen with other types of uveitis. The eye is white and posterior synechiae do not form. The iris has a characteristic moth-eaten appearance and becomes de-pigmented, showing a bluish tinge in Caucasian patients. This depigmentation is not as obvious in heavily pigmented eyes where iris stromal atrophy is the hallmark. Heterochromic cyclitis is usually unilateral although bilateral cases rarely occur and are more difficult to diagnose. Glaucoma may develop and is associated with a fine neovascularization of the iris and angle (see Ch. 8). Cataracts are common and are hastened by steroid therapy; the benefit of steroids in this condition is unproven. Histopathological examination of iris specimens shows stromal atrophy with loss of pigment, hyalinization of blood vessel walls, proliferation of vascular endothelial cells and patchy loss of pigment epithelium. There is an inflammatory cell infiltrate of eosinophils, mast cells, lymphocytes and plasma cells; Russell bodies (immunoglobulins) are present. Recent evidence suggests that the condition is due to persistent localized rubella viral infection.

Fig. 10.22 The right eye of this patient is normal but the left shows stromal atrophy consistent with Fuchs’ heterochromic cyclitis. The left iris has a slightly bluish tinge. Note the peripheral iridectomy from previous glaucoma surgery.

Fig. 10.23 In African Caribbean patients, heterochromia is not seen and iris stromal atrophy is the hallmark. Superficial nodules can be seen on the stromal surface in some patients.

Fig. 10.24 This photograph shows the typical KPs of Fuch’s heterochromic cyclitis. These cover the whole of the endothelial surface and have ‘spidery’ margins. A stringy cellular infiltrate can often be seen in the vitreous.

By courtesy of Mr K Barton.

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