Ординатура / Офтальмология / Английские материалы / Moorfields Manual of Ophthalmology_Jackson_2007

neovascularization. Control inflammation with the minimum effective dose and duration of treatment. Consider: location and immediacy of the threat to vision; whether the disease is potentially reversible; if unilateral or bilateral; systemic factors including age, general health, and pregnancy; patient compliance and preference. In general, use periocular steroids if the patient is not a steroid responder, particularly in unilateral disease. Otherwise, use oral steroids in the first instance with the addition of ciclosporin or mycophenolate if unable to induce remission or reduce the relapse rate. If the disease is still not controlled, consider intravitreal triamcinolone if not a steroid responder (sometimes necessary despite known steroid response). Alternatively, use a combination of steroids/ciclosporin/mycophenolate.

Corticosteroids are the mainstay of management.

■Local administration : topical steroids treat anterior uveitis and anterior vitreous cells but not posterior segment inflammation. In contrast, local steroid injections (periocular or intravitreal) are effective in posterior uveitis and should be used whenever possible. The commonest indications are unilateral or bilateral asymmetric disease, especially where high-dose systemic steroids are contraindicated, e.g. pregnancy, or while obtaining diabetic or hypertensive control. Periocular steroids can take several weeks to work and are unsuitable when there is an immediate threat to vision. They are also contraindicated in steroid responders. Local injections are most useful in controlling vitritis and chronic CMO.

Posterior sub-Tenon triamcinolone (40 mg in 1 mL) or orbital floor methylprednisolone (Depomedrone 40 mg in 1 mL) appear equally effective. These can be repeated after 6 weeks but the mean time to best VA is 9 weeks. Discontinue if there is no response after 3 injections. Intravitreal triamcinolone (Kenalog 4 mg in 0.1 mL) is more potent and is given using the same technique as intravitreal antibiotics (p. 385). Measure

IOP 15 minutes after injection. The risk of steroid-induced glaucoma is ≈30%, but may be up to 80% in patients under 30 years, so check IOP after 3 weeks when steroid response is usually first seen. Injections can be repeated after 3–4 months if the IOP is normal.

■Systemic steroids : use if the disease is bilateral, vision threatening, or local steroids have failed, but discuss the risks

versus benefits. If the vision is threatened that day or VA ≤6/60, a typical starting dosage is prednisolone 80

UVEITIS 8 Chapter

Posterior uveitis

o.d. 1 week, 40 mg o.d. 2 weeks, and thereafter guided by clinical effect. If VA is 6/12–6/18 and the visual threat is less severe, consider 40 mg o.d. (0.5–1 mg/kg) for 2 weeks then 30 mg o.d. 2 weeks. The required duration of treatment is difficult to predict. Aim to taper slowly, usually over months, to avoid reactivation. If unilateral reactivation occurs, consider local steroid injection to avoid increasing oral steroids. Some patients require indefinite treatment.

■Side effects : hypertension, hypokalaemia, diabetes, osteoporosis, cushingoid appearance, and peptic ulceration. Cover with omeprazole 20 mg o.d. or ranitidine 150 mg b.d. Review drug interactions in the British National Formulary or equivalent.

■Baseline tests : weight, BP, blood glucose, FBC, U&E, and CXR if indicated (history of TB/TB risk). Steroid-induced bone loss is worse at very high doses and aggravated by ciclosporin. Significant loss occurs in the first 6 months, so if this duration of treatment is likely then prescribe alendronate (Fosamax)

70 mg once weekly from the outset. Alternatively, reserve alendronate for high-risk patients and use calcium supplements (Calcichew D3 Forte b.d.) for lower-risk cases (avoid in sarcoid). A baseline bone scan will help define the risk.

■Monitoring : measure weight, BP, and blood glucose at each visit. Check FBC (microcytic anaemia from GI blood loss, neutrophilia and lymphopaenia) and U&E (hypokalaemia) every 1–3 months. Advise patients to carry a steroid card and seek medical attention if they develop fever or lose their tablets.

Second-line agents Steroid-sparing agents are used to augment or help reduce steroid treatment, e.g. when steroid dose ≤10 mg produces frequent relapses. At all ages, balance the risk of lymphoma from treatment and try to avoid second-line agents in unilateral disease.

■Young healthy patients : ciclosporin 3–7.5 mg/kg/day p.o. in two divided doses is the most widely used second-line agent although some clinicians prefer to avoid it for long-term use, selecting azathioprine or mycophenolate instead. Ciclosporin takes 2–6 weeks to work, depending on the dose. Renal impairment, liver disease, hypertension, and abnormal FBC are relative contraindications. Review drug interactions in the

creatinine by 30% above baseline, reduce the dose by 25% immediately. Once stable, monitor BP and U&E every 3 months. Tacrolimus has a similar mode of action to ciclosporin; it is more potent but has a high incidence of renal toxicity. Methotrexate and azathioprine are often selected as third-line drugs. Cyclophosphamide and chlorambucil both have significant toxic side effects, so avoid unless absolutely necessary for severe destructive scleral inflammatory disease refractive to other agents, e.g. Wegener’s granulomatosis.

■Older patients (>55–60 years) are generally less tolerant of ciclosporin in the long term (nephrotoxicity and hypertension). Mycophenolate (Cellcept) 0.5–1 g p.o. b.d. is increasingly used, or azathioprine 2–3 mg/kg/day in divided doses (recommended maximum of 100 mg b.d.). For azathioprine check TPMT (thyopurine methyltransferase) before starting and in carriers reduce to 0.5–1 mg/kg, or if low, avoid. Allow 3–6 weeks for effect and monitor FBC/LFTs.

Follow–up Varies but generally see patients starting systemic treatment at 1–4 weeks to monitor disease, side effects, and compliance. As inflammation settles, increase progressively to 3- monthly review. Advise patients to attend if symptoms recur. Monitor closely if there is a relapse, if re-introducing high-dose steroids, or starting second-line agents.

UVEITIS 8 Chapter

Sarcoidosis

Sarcoidosis

Background A systemic granulomatous disease commonest in Asian and Afro-Caribbean patients. Multiple sites may be affected but particularly the lungs, lymph nodes, skin, liver, and eyes.

Symptoms Those of anterior, intermediate, or posterior uveitis. Systemic disease may be asymptomatic.

Signs These include: unilateral or bilateral acute or chronic anterior uveitis; intermediate uveitis; inferior preretinal nodules; patchy periphlebitis and sheathing; venous occlusion and neovascularization; small, round, usually inferior, chorioretinal lesions (Fig. 8.5); optic nerve involvement (granulomas, papilloedema with CNS involvement); secondary cataract/ glaucoma. Look for iris infiltration with a velvety appearance and large, pink, peripheral nodules which tend to produce peripheral anterior synechiae.

History and examination Include extraocular assessment for lung, skin, bone, joint and muscle, salivary gland, and CNS involvement. Ask about cardiac pain.

Differential diagnosis Depends on the pattern of ocular/ systemic signs. Exclude TB (risk of reactivation with systemic steroids) and syphilis. Consider other causes of multifocal choroiditis (p. 342). Arteriolar involvement is rare and suggests Behçet’s disease, toxoplasma chorioretinitis, syphilis, or acute retinal necrosis.

Investigations Arrange CXR, sACE, syphilis serology, Mantoux/Heaf test if there is a risk of TB (best done by a physician). Biopsy skin/conjunctival lesions if obvious clinically. Refer those with systemic symptoms to a physician.

Treatment and follow-up Use topical, periocular, or systemic steroids as appropriate. Neovascularization may be secondary to ischaemia or inflammation, requiring laser or steroids, respectively, or sometimes both. Long-term follow-up is usually needed. Patients may go into remission but relapses are common.

Tuberculosis

Tuberculosis

Background At-risk groups include patients from Asia or those who are immunosuppressed, including HIV. Ocular manifestations may be due to direct infection or a hypersensitivity response to TB elsewhere.

History Many patients are systemically well. Pain, redness, blurred vision, and floaters are variable. Ask about BCG vaccination, previous TB/TB risk assessment, and evidence of a TB focus.

Examination Look for: conjunctival phlycten; nondescript anterior uveitis or associated with iris nodules; variably sized keratic precipitates; solitary choroidal granulomata (Fig. 8.6); multifocal choroiditis (lesions characteristically variable in size); subretinal abscess; retinitis; retinal vasculitis from infection (nonocclusive) or a hypersensitivity response with widespread peripheral ischaemia ± new vessels elsewhere (NVE) with associated vitreous haemorrhage (VH).

Differential diagnosis The diagnosis is often presumptive but exclude other granulomatous disease, especially sarcoidosis and Eales’ disease (widespread peripheral venous occlusion, NVE/ vitreous haemorrhage, but no anterior chamber/vitreous cells).

Investigations CXR, Heaf/Mantoux test (by a physician), and sACE (overlap with sarcoidosis).

Treatment and follow–up Manage with a specialist physician. Treatment may include TB chemotherapy, oral steroids for active inflammation, retinal laser for new vessels, and topical steroids for conjunctival phlycten and anterior uveitis. Adjust (double) steroid treatment for patients on rifampicin.

UVEITIS 8 Chapter

Behçet’s disease

Behçet’s Disease

Background A chronic systemic inflammatory disease most frequent in young men of Japanese, Arabic, or Mediterranean origin (particularly those from Turkey and Greece). Major diagnostic criteria are:

■Recurrent oral ulcers (have to be present)

■Genital ulcers

■Skin lesions (erythema nodosum and pustules)

■Positive pathergy test

■Uveitis

Symptoms Pain and photophobia, or rapid-onset floaters and blurred vision.

Signs Transient hypopyon is common and may accompany anterior uveitis. Chronic panuveitis with severe acute recurrences is characteristic, as is an ischaemic vasculitis involving both arteries and veins. White necrotic retinitis may be accompanied by retinal infiltrates, oedema, and haemorrhage (Fig. 8.7). Second eye involvement may be delayed for years.

Differential diagnosis Reiter’s syndrome, Crohn’s disease, other causes of arterial vasculitis (collagen/vascular disorders).

Investigations None. Clinical diagnosis.

Treatment and follow–up See page 342 for the treatment of uveitis. Second-line agents are often required from the outset and the long-term prognosis is guarded. Recurrent posterior uveitis may result in retinal vascular attenuation and secondary optic atrophy. Lifelong follow-up is typical for posterior segment disease.

Vogt-koyanagi-harada syndrome

Vogt-Koyanagi-Harada Syndrome

Background A systemic disease with ocular and skin involvement more common in Asian, Hispanic, and Japanese patients.

Symptoms Bilateral, synchronous or sequential blurred vision, with variable redness, pain, and photophobia. Patients may have a prodromal illness of pyrexia, headache, malaise, auditory or meningeal symptoms.

Signs Serous retinal detachments overlying diffuse or multifocal choroiditis, with vitritis, disc oedema or hyperaemia (characteristic pink disc) (Fig. 8.8). Anterior uveitis is common. Cutaneous signs (alopecia, poliosis, vitiligo) may develop later. Check colour vision and exclude an RAPD.

Differential diagnosis Consider sympathetic ophthalmia, posterior scleritis, sarcoidosis, and masquerade syndromes. Acute multifocal posterior placoid pigment epitheliopathy (AMPPE) rarely causes serous detachment.