ANTERIOR UVEITIS
Clinical features
Acute
Signs
- •
Injection – intense and circumcorneal (ciliary – Fig. 8.1 ).
Fig. 8.1
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Pupil – small.
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Keratic precipitates (KP) – endothelium dusting by myriads of cells.
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Flare and cells – often intense ( Fig. 8.2 ).
Fig. 8.2
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Fibrinous exudate – if severe ( Fig. 8.3 ).
Fig. 8.3
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Hypopyon – if very severe.
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Iris – usually unremarkable; occasionally shows dilated capillaries (see Fig. 8.63 ).
Fig. 8.63
Complications
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Posterior synechiae (PS) – rare at presentation but may form later.
- •
Cataract – absent.
- •
Glaucoma – rare.
Chronic
Signs
- •
Injection – mild or absent.
- •
Pupil – unremarkable.
- •
KP – mutton-fat in granulomatous disease ( Fig. 8.4 ).
Fig. 8.4
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Flare and cells – variable.
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Fibrinous exudate – absent.
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Hypopyon – rare.
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Iris – nodules in granulomatous disease, especially sarcoidosis ( Fig. 8.5 ).
Fig. 8.5
Complications
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PS – common at presentation ( Fig. 8.6 ).
Fig. 8.6
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Cataract – rare at presentation but may develop later.
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Glaucoma – rare at presentation but may develop later.
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Anterior uveitis without systemic associations
Fuchs syndrome
Definition
- •
A unilateral idiopathic chronic anterior uveitis (CAU) with an insidious onset that typically presents in early adult life.
Signs
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Injection – absent.
- •
Pupil – unremarkable.
- •
KP – small, grey-white, scattered throughout endothelium and frequently associated with feathery fibrin filaments ( Fig. 8.7 ).
Fig. 8.7
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Flare and cells – mild to moderate.
- •
Fibrinous exudate – absent.
- •
Hypopyon – absent.
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Iris – small stromal nodules ( Fig. 8.8 ), small pupillary nodules and stromal atrophy ( Fig. 8.9 ), and heterochromia.
Fig. 8.8
Fig. 8.9
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Vitritis – may be severe.
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Angle – fine twig-like vessels over the trabeculum ( Fig. 8.10 ).
Fig. 8.10
Complications
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PS – absent.
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Cataract – common at presentation.
- •
Glaucoma – rare at presentation but common in long-standing cases.
HLA-B27 associated
HLA-B27 patients may suffer from severe recurrent acute anterior uveitis (AAU) often associated with a fibrinous exudate (see Fig. 8.3 ).
Lens-induced
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Phacoanaphylactic endophthalmitis – bilateral CAU.
- •
Phacogenic – unilateral CAU.
Drug-induced
Drugs that may cause bilateral AAU are:
- •
Rifabutin.
- •
Cidofovir.
Posner–Schlossman syndrome
Definition
- •
A rare condition characterised by recurrent unilateral attacks of severe elevation of intraocular pressure associated with mild AAU and small KP (see Fig. 7.27 ).
Systemic associations of anterior uveitis
Ankylosing spondylitis
Incidence
- •
Severe recurrent AAU affects about 30% of cases and is frequently associated with a fibrinous exudate.
Look for
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HLA-B27 – positive in 90% of cases.
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Pain and stiffness in the lower back or buttocks, more marked in the mornings due to sacroiliitis.
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Rigid neck with lack of extension ( Fig. 8.11 ).
Fig. 8.11
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Limitation of spinal movements due to syndesmophyte formation and ossification of anterior spinal ligaments (bamboo spine – Fig. 8.12 ).
Fig. 8.12
Reiter syndrome
Incidence
- •
AAU is common but acute conjunctivitis is universal.
Look for
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HLA-B27 – positive in 85%.
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Urethritis is the presenting feature.
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Painless mouth ulceration.
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Asymmetrical arthritis most frequently involving the knees and ankles.
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Circinate balanitis ( Fig. 8.13 ).
Fig. 8.13
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Keratoderma blenorrhagica involving the palms and soles (see Fig. 3.40 ).
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Sacroiliitis, particularly in carriers of HLA-B27, resulting in irregularity and loss of cortical margins with subsequent sclerosis, narrowing and fusion ( Fig. 8.14 ).
Fig. 8.14
Psoriatic arthritis
Incidence
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AAU is uncommon.
Look for
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HLA-B27 – uncommon.
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Well-demarcated, scaly patches on extensor surfaces.
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Asymmetric arthritis involving the knuckles and distal interphalangeal joints that may result in severe deformity (arthritis mutilans – Fig. 8.15 ).
Fig. 8.15
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Nail dystrophy ( Fig. 8.16 ).
Fig. 8.16
Behçet disease
Incidence
- •
AAU associated with hypopyon is common but the eye usually lacks ciliary injection ( Fig. 8.17 ).
Fig. 8.17
Look for
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HLA-B51 – common.
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Recurrent painful oral ( Fig. 8.18 ) and genital ulceration (see Figs 15.99 and 15.100 ).
Fig. 8.18
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Cutaneous hypersensitivity (dermatographia – Fig. 8.19 ), erythema nodosum and acneiform lesions.
Fig. 8.19
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Recurrent thrombophlebitis.
Acute sarcoidosis
Incidence
- •
AAU is uncommon.
Look for
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Löfgren syndrome – fever, erythema nodosum ( Fig. 8.20 ) and hilar lymphadenopathy ( Fig. 8.21 ).
Fig. 8.20
Fig. 8.21
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Heerfordt syndrome – fever and parotid enlargement ( Fig. 8.22 ).
Fig. 8.22
Chronic sarcoidosis
Incidence
- •
Granulomatous anterior uveitis (GAU) is common.
Look for
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Cutaneous lesions – lupus pernio and granuloma ( Fig. 8.23 ).
Fig. 8.23
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Pulmonary disease – parenchymal infiltrates and fibrosis ( Fig. 8.24 ).
Fig. 8.24
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Neurological lesions – multifocal peripheral neuropathy with a predilection for the facial nerve, meningeal infiltration and CNS granulomas.
Vogt–Koyanagi–Harada syndrome
Incidence
- •
Anterior uveitis which is initially acute and then becomes chronic and granulomatous is common.
Look for
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Hispanic, Japanese or pigmented individual.
- •
Alopecia, poliosis and vitiligo ( Fig. 8.25 ).
Fig. 8.25
Juvenile idiopathic arthritis
Incidence
- •
CAU is common in pauciarticular-onset disease and may lead to band keratopathy and cataract ( Fig. 8.26 ).
Fig. 8.26
Look for
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Child under the age of 16 years.
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Pauciarticular-onset arthritis most frequently involving the knees ( Fig. 8.27 ).
Fig. 8.27
Inflammatory bowel disease
Incidence
- •
Ulcerative colitis and Crohn disease may be associated with AAU particularly in patients with associated sarcoiliitis.
Look for
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Weight loss, fever and diarrhoea.
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Pyoderma gangrenosum ( Fig. 8.28 ) and erythema nodosum.
Fig. 8.28
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Anal tags and fistulae in Crohn disease.
Relapsing polychondritis
Incidence
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AAU is uncommon.
Look for
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Recurrent inflammation of pinnae.
- •
Collapse of nasal cartilage ( Fig. 8.29 ).
Fig. 8.29
Other associations
- a.
Tuberculosis – CAU and GAU.
- b.
Acquired syphilis – AAU and GAU.
- c.
Tubulointerstitial nephritis – CAU.
- d.
IgA glomerulonephritis – AAU.
- e.
Leprosy – AAU and CAU.
- f.
Lyme disease – GAU.
- g.
Brucellosis – CAU.
- h.
Neonatal-onset multisystem inflammatory disease – CAU.
IRIS SPOTS AND NODULES
Solitary
Naevus
Signs
- •
Unilateral flat or slightly elevated pigmented lesion involving the superficial layers of the iris ( Fig. 8.30 ).
Fig. 8.30
- •
Mild pupillary distortion and ectropion uveae are occasionally seen.
- •
In the Cogan–Reese syndrome the naevus is more diffuse, obscures the normal pattern of iris crypts and gives rise to hyperchromic heterochromia ( Fig. 8.31 ).
Fig. 8.31
Melanoma
Definition
- •
A very slow-growing tumour of relatively low malignancy which typically presents during the 5th decade of life.
Signs
- •
Pigmented or non-pigmented nodule, at least 3 mm in diameter and 1 mm in thickness, located in the inferior half of the iris ( Fig. 8.32 ).
Fig. 8.32
- •
Vascularisation is easier to detect in a non-pigmented tumour ( Fig. 8.33 ).
Fig. 8.33
- •
Pupillary distortion, ectropion uveae and secondary lens opacities.
- •
Angle involvement may give rise to raised intraocular pressure (see Figs 7.8 and 7.9 ).
Leiomyoma
Signs
- •
Similar to an amelanotic melanoma except that it is not confined to the inferior part of the iris ( Fig. 8.34 ).
Fig. 8.34
Melanocytoma
Signs
- •
Black friable nodule with a mossy granular surface that has a predilection for the inferior iris root ( Fig. 8.35 ).
Fig. 8.35
- •
Aqueous seeding may cause elevation of intraocular pressure.
Metastatic carcinoma
Signs
- •
Fast-growing amelanotic lesion anywhere on the iris ( Fig. 8.36 ).
