Terminology
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Uveitis: inflammation of the uveal tract.
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Anterior uveitis: iritis and iridocyclitis (iris and ciliary body).
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Intermediate uveitis: inflammation of the pars plana, the peripheral retina, and the vitreous.
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Posterior uveitis: inflammation posterior to the vitreous base.
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Retinitis: primary retinal focus of inflammation.
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Choroiditis: primary choroidal focus.
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Vasculitis: inflammation of veins, arteries, or both.
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Panuveitis: involvement of the entire uveal tract.
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Endophthalmitis: involves all intraocular tissues except the sclera.
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Panophthalmitis: involves the entire globe.
Clinical features
Acute anterior uveitis
Acute anterior uveitis (AAU) is the most common form of uveitis.
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Presentation: rapid onset of unilateral pain, photophobia, and redness.
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Signs: (a) ciliary injection, (b) miosis ( Fig. 11.1 ), (c) endothelial dusting by inflammatory cells progresses to fine/medium keratic precipitates (KP) after a few days, (d) hypopyon in intense inflammation ( Fig. 11.2 ), (e) anterior vitreous cells in iridocyclitis, and (f) aqueous flare ( Fig. 11.3 ).
Fig 11.1
Fig 11.2
Fig 11.3
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Duration: most episodes completely resolve within 5–6 weeks.
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Prognosis: good with adequate treatment.
Chronic anterior uveitis
Chronic anterior uveitis (CAU) is less common than the acute type, and it is more frequently bilateral; inflammation may be granulomatous or non-granulomatous.
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Presentation: often insidious and may be asymptomatic until complications such as cataract or band keratopathy develop.
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Signs: (a) eye may be mildly injected or white, depending on the cause, (b) aqueous cells vary in number according to activity, (c) aqueous flare may be more marked than cells in eyes with prolonged activity, (d) large KP with a greasy ‘mutton-fat’ appearance ( Fig. 11.4 ) and iris nodules in granulomatous disease ( Fig. 11.5 ); (e) posterior synechiae ( Fig. 11.6 ).
Fig 11.4
Fig 11.5
Fig 11.6
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Duration: prolonged; may last for months or even years with remissions and exacerbations.
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Prognosis: guarded because of complications such as cataract, glaucoma, hypotony, and phthisis bulbi ( Fig. 11.7 ).
Fig 11.7
Posterior uveitis
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Presentation: varies according to the location of the inflammatory focus and the presence of vitritis.
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Retinitis: whitish retinal opacities with indistinct borders ( Fig. 11.8 ) and overlying vitritis.
Fig 11.8
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Choroiditis: deep, round, yellow nodules ( Fig. 11.9 ).
Fig 11.9
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Vasculitis: (periphlebitis, periarteritis) may be primary or secondary to retinitis; yellowish or grey-white patchy perivascular cuffing which may be associated with haemorrhages ( Fig. 11.10 ).
Fig 11.10
Principles of treatment
Mydriatics
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Indications: (a) to relieve pain caused by iridociliary spasm, and (b) prevent (or break recently formed) posterior synechiae.
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Topical short-acting: (a) cyclopentolate (0.5%, 1%) acts for approximately 24 hr; (b) tropicamide and phenylephrine are shorter-acting.
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Topical long-acting: (a) homatropine 2% (approximately 2 days); (b) and atropine 1% (up to 2 weeks).
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Subconjunctival: Mydricaine (adrenaline, atropine, and procaine) if no response to drops.
Topical steroids
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Indications: anterior uveitis.
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Preparations: dexamethasone, prednisolone (range of concentrations available), betamethasone, loteprednol, fluorometholone, and rimexolone.
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Complications: glaucoma, cataract, and corneal infection.
Periocular steroid injection
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Advantages over topical administration: (a) higher posterior segment concentrations may be achieved, (b) water-soluble drugs, incapable of penetrating topically, but can enter trans-sclerally, and (c) prolonged effect can be achieved with ‘depot’ preparations.
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Indications: (a) primary treatment of intermediate and certain types of posterior uveitis, (b) to supplement systemic therapy or when it is contraindicated, (c) poor compliance with topical or systemic medication, and (d) perioperatively in uveitic eyes.
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Complications: (a) globe penetration, (b) elevation of IOP (may be refractory with depot preparations), (c) ptosis, (d) subdermal fat atrophy, (e) ocular motility paresis, (f) optic nerve injury, (g) retinal and choroidal vascular occlusion, and (h) cutaneous hypopigmentation.
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Technique: via the superotemporal conjunctiva (posterior sub-Tenon; Fig. 11.11 ), or to the orbital floor via skin or conjunctiva.
Fig 11.11
Intraocular steroids
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Intravitreal injection: triamcinolone acetonide can be used for some forms of posterior uveitis unresponsive to other modalities and for refractory cystoid macular oedema (CMO).
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Complications: (a) elevation of IOP, (b) cataract, (c) endophthalmitis (sterile or infectious), (d) haemorrhage, and (e) retinal detachment.
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Slow-release implants: biodegradable insert or a slow-release reservoir (fluocinolone acetonide, dexamethasone) offer the benefits of intravitreal steroid injection but over an extended period of up to 3 years; complications are similar to those of intravitreal triamcinolone injection.
Systemic steroids
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Indications: (a) intermediate uveitis unresponsive to periocular injection, and (b) sight-threatening posterior uveitis, particularly if bilateral.
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Contraindications: (a) poorly-controlled diabetes (relative contraindication), (b) peptic ulceration, (c) osteoporosis, (d) active systemic infection, and (e) psychosis on previous exposure to steroids.
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Preparations: oral prednisolone and intravenous methylprednisolone.
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Common cause of failure: inadequate dosage; a large dose should be used initially and subsequently tapered.
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Side effects with short-term use: (a) dyspepsia, (b) mental changes, (c) electrolyte imbalance, (d) aseptic necrosis of the head of the femur, and (e) destabilization of diabetic control.
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Side effects with long-term use: (a) Cushingoid state, (b) osteoporosis, (c) limitation of growth in children, (d) reactivation of infections such as TB, (e) cataract, and (f) elevation of IOP.
Antimetabolites
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Indications: (a) sight-threatening uveitis, typically bilateral, non-infectious and reversible, after failure to respond to adequate steroid therapy, (b) steroid-sparing therapy in patients with intolerable side effects from systemic steroids, and (c) chronic relapsing disease requiring a daily dose of prednisolone above approximately 10 mg.
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Preparations: azathioprine, methotrexate, and mycophenolate mofetil.
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Side effects: (a) gastrointestinal disturbance, (b) bone marrow suppression, and (c) hepatotoxicity.
Other drugs
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Ciclosporin: drug of choice for Behçet syndrome; side effects include nephrotoxicity and hepatotoxicity.
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Tacrolimus: alternative to ciclosporin in intolerant or unresponsive patients.
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Biological blockers: interleukin receptor antagonists (daclizumab, anakinra) and tumour necrosis factor-alpha antagonists (infliximab, adalimumab).
Intermediate uveitis
Definition: intermediate uveitis (IU) is a chronic/relapsing condition that may be idiopathic or associated with systemic disease. It is typically bilateral but asymmetrical. Systemic associations include (a) multiple sclerosis (MS), (b) sarcoidosis, (c) Lyme disease, and (d) TB. The course and prognosis are very variable.
Diagnosis
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Presentation: in childhood or early adult life with insidious onset of blurred vision and floaters.
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Signs: (a) mild anterior uveitis, (b) vitreous cells with anterior predominance, (c) inferior vitreous ‘snowballs’ ( Fig. 11.12 ), (d) peripheral phlebitis, (e) inferior ‘snowbanking’ ( Fig. 11.13 ), and (f) neovascularization on the snowbank or the optic nerve head.
Fig 11.12
Fig 11.13
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Complications: (a) CMO in 30% is the major cause of visual impairment, (b) epimacular membranes, and occasionally (c) retinal detachment (tractional, rhegmatogenous, or exudative).
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Treatment
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Medical: (a) initially topical and/or periocular steroids, (b) systemic steroids and immunosuppressive agents may be required, and (c) interferon beta for IU in MS.
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Vitrectomy: following failure of systemic steroids to control CMO; other indications include retinal detachment, vitreous haemorrhage, and epiretinal membranes.
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Photocoagulation: to peripheral retina for neovascularization at the vitreous base.
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Uveitis in spondyloarthropathies
Ankylosing spondylitis
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Definition: an inflammatory disease leading to ossification of joints with bony ankylosis of the axial skeleton. It typically affects adult males, of whom 90% are HLA-B27 positive (6–8% of U.S. Caucasians). Radiology of the sacroiliac joints and spine reveals characteristic changes.
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Ocular features: AAU occurs in approximately 25% of patients with ankylosing spondylitis; conversely, 25% of males with AAU will have ankylosing spondylitis.
Reiter syndrome
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Definition: Reiter syndrome comprises the triad of (a) non-specific urethritis, (b) conjunctivitis, and (c) arthritis. Those affected are commonly men in the 3rd and 4th decades, of whom 85% are positive for HLA-B27.
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Ocular features: (a) conjunctivitis is common and usually precedes the arthritis, with spontaneous resolution occurring within 7–10 days; (b) AAU occurs in up to 12% of patients but is higher in carriers of HLA-B27.
Psoriatic arthritis
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Definition: arthritis developing in patients with psoriasis.
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Ocular features: (a) AAU occurs in approximately 7%; (b) other uncommon manifestations include conjunctivitis, marginal corneal infiltrates, and secondary Sjögren syndrome.
Uveitis in juvenile arthritis
Juvenile idiopathic arthritis
Definition: idiopathic arthritis of at least 6 weeks’ duration occurring before the age of 16 years. Juvenile idiopathic arthritis (JIA) is by far the most common disease associated with childhood anterior uveitis. Girls are affected more commonly than boys. Positivity for ANA is associated with a higher risk of uveitis.
Diagnosis
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Pauciarticular onset (60% of cases): four or fewer joints, most commonly the knees, are involved during the first 6 months with a peak age of onset around 2 years; approximately 75% of children are ANA-positive and uveitis affects approximately 20%.
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Polyarticular onset (20%): five or more joints are involved; may commence throughout childhood; 40% ANA-positive and uveitis affects 5%.
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Systemic onset (Still’s disease; 20%): presents with fever, maculopapular rash, generalized lymphadenopathy, hepatosplenomegaly, and serositis; arthritis may be absent or minimal; most are ANA-negative and uveitis does not occur.
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Features of anterior uveitis in JIA: (a) onset is usually asymptomatic with a white eye, (b) chronic non-granulomatous inflammation, (c) 70% bilateral, and (d) complications include band keratopathy, cataract ( Fig. 11.14 ), glaucoma, and hypotony.
Fig 11.14
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Differential diagnosis: (a) idiopathic juvenile chronic iridocyclitis, (b) other types of juvenile arthritis and uveitis (e.g. juvenile spondyloarthropathies, sarcoidosis, Lyme disease, IU), and (c) masquerade syndromes (e.g. anterior segment involvement by retinoblastoma).
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Treatment
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Screening: regular slitlamp examinations at intervals ranging from (a) 9-monthly in low-risk ANA-negative polyarticular-onset patients; (b) 2-monthly in high-risk ANA-positive pauciarticular-onset children; (c) not required in systemic-onset disease.
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Treatment: topical steroids are usually effective; poor responders may benefit from periocular steroids or other agents.
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Uveitis in bowel disease
Ulcerative colitis
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Definition: idiopathic chronic inflammatory disease presenting in the 2nd and 3rd decades, affecting principally the large intestine and rectum. Extra-intestinal manifestations include mucocutaneous lesions, arthritis, and hepatic disease.
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Ocular features: AAU occurs in approximately 5%.
Crohn disease
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Definition: an idiopathic chronic granulomatous inflammatory disease of the intestinal wall, most frequently involving the ileocaecal region. Presentation is in the 2nd and 3rd decades with fever, weight loss, diarrhoea, and abdominal pain. Extra-intestinal manifestations include mucocutaneous lesions and skeletal features.
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Ocular features: AAU occurs in approximately 3%.
Sarcoidosis
Pathogenesis: T-lymphocyte-mediated granulomatous inflammatory disorder of unknown cause. It is most common in colder climates, although it more frequently affects patients of African descent than Caucasians. A wide range of tissues can be involved, with lung disease being common; severity varies from mild single-organ involvement to potentially fatal multisystem disease.
Diagnosis
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Presentation: (a) acute with fever, erythema nodosum, arthralgia, and cranial nerve palsy, or (b) insidious with symptomatic pulmonary involvement.
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Pulmonary lesions: vary from symptomatic hilar lymphadenopathy to severe fibrosis.
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Skin lesions: (a) erythema nodosum, (b) granulomas, and (c) lupus pernio.
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Neurological disease: (a) facial palsy, (b) seizures, (c) meningitis, (d) peripheral neuropathy, and (e) psychiatric symptoms.
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Other: (a) arthropathy, (b) bone cysts, (c) renal disease, (d) lymphadenopathy, and (e) liver disease.
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Investigations: (a) chest X-ray (abnormal in 90%), (b) biopsy (lung, lymph node, skin, conjunctiva, lacrimal gland), (c) serum angiotensin-converting enzyme and lysozyme, (d) bronchoalveolar lavage, and (e) pulmonary function tests; Mantoux test is negative in most patients.
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Ocular features: (a) AAU typically occurs in patients with acute-onset sarcoid, (b) CAU, typically granulomatous ( Fig. 11.15 ), tends to affect older patients with chronic disease, (c) IU, (d) periphlebitis with ‘candle wax drippings’ ( Fig. 11.16 ), (e) choroidal infiltrates, (f) multifocal choroiditis, (g) retinal granulomas, (h) peripheral retinal neovascularization, (i) optic nerve granulomas, and (j) disc oedema.
Fig 11.15
Fig 11.16
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Treatment: anterior uveitis is treated with topical and/or periocular steroids; posterior uveitis often requires systemic steroids, and occasionally other immunosuppressive agents.
Behçet syndrome
Definition: idiopathic multisystem disease characterized by recurrent episodes of orogenital ulceration and vasculitis. It typically affects Eastern Mediterranean and Japanese individuals and is strongly associated with HLA-B51. The peak age of onset is in the 3rd decade; males are affected more frequently than females.
Diagnosis
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Recurrent oral ulceration: plus at least two of the following: (a) recurrent genital ulceration, (b) ocular inflammation, (c) skin lesions, and (d) positive pathergy test (pustule at needle prick site).
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Other systemic features: (a) vascular (aneurysms, coronary artery disease, venous thrombosis), (b) arthritis, (c) skin hypersensitivity, (d) gastrointestinal ulceration, and (e) occasionally neurological manifestations.
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Ocular features: common and often bilateral; (a) AAU (transient mobile hypopyon in a relatively white eye; Fig. 11.17 ), (b) persistent vitritis, (c) transient superficial white retinal infiltrates ( Fig. 11.18 ), (d) diffuse retinitis, (e) retinal vasculitis (veins and arteries, often occlusive; Fig. 11.19 ), and (f) CMO, disc oedema, and optic atrophy in end-stage disease ( Fig. 11.20 ).
Fig 11.17
Fig 11.18
Fig 11.19
Fig 11.20
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Treatment: anterior uveitis responds well to topical steroids; posterior uveitis requires systemic steroids, azathioprine, ciclosporin, subcutaneous interferon alpha, and possibly biological blockers (e.g. infliximab).
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