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Editor,—Takayasu's (pulseless) disease is a chronic inflammatory arteriopathy of unknown aetiology and was first described in 1908 by an ophthalmologist, Takayasu.1Although he described some retinal features of the disease he did not discuss the absence of any arterial pulses. It occurs more commonly in young oriental women and affects the aorta and/or its major branches giving rise to end organ hypoperfusion.2 The major complications attributed to the disease include Takayasu's retinopathy, secondary hypertension, aortic regurgitation, and arterial aneurysms. The ophthalmological complications are generally late manifestations of the disease and include ischaemia of the retina, choroid, and anterior segment.3 We describe a patient with Takayasu's disease who presented with scleritis of her left eye.
A 39 year old Asian woman presented to Moorfields Eye Hospital casualty department with a red, painful left eye and was diagnosed as having anterior scleritis (Fig 1). Her visual acuities were 6/5 right and left and intraocular pressures were normal. There was no retinal vasculitis and no history of any previous ocular problems. She had been investigated eighteen months earlier for left intermittent arm pain with claudication and had been diagnosed as having left subclavian artery occlusion. She was a non-smoker and no underlying cause for the arterial disease had been identified at that time. Investigations following the diagnosis of scleritis included a raised erythrocyte sedimentation rate at 72 mm in the first hour, microcytic anaemia, and raised PEG immune complexes at 24.8. Antinuclear antibodies (ANA) and antineutrophilic cytoplasmic antibodies (ANCA) were negative. Clinical examination revealed absent pulses in the radial, ulnar, and brachial arteries of her left arm with carotid and subclavian bruits. The right arm blood pressure (BP) measured 100/60 and the leg BP was 180/100. An arch aortogram (Fig 2) showed complete occlusion of the left common carotid and subclavian arteries and a tight stenosis of the proximal right subclavian artery with a mild stenosis of the right carotid artery. An echocardiogram showed mild mitral valve regurgitation. A diagnosis of Takayasu's disease made, and the patient was started on oral cyclophosphamide (2 mg/kg/day), prednisolone (60 mg daily), and amilodipine to treat the hypertension (the leg BP was taken as the true BP reading). Her scleritis rapidly resolved with this regimen, and she subsequently underwent successful reconstructive arterial surgery.
Takayasu's disease has an incidence of 2.6 per million per year with a female to male ratio of 9:1.4 Although more commonly affecting orientals it has been described in all races5 and classically presents in the second to fourth decades of life. Scleritis is a feature of many of the vasculitides6 7 but has not previously been described as a presenting feature of Takayasu's disease. There have been reports of patients with Takayasu's disease and scleritis who were later diagnosed as having Wegener's granulomatosis.8 9 These patients were reported to have raised ANCA titres, however, which is rare in Takayasu's disease.10-12 The ANCA titre was negative in the patient we describe. This case alerts us to the possibility of Takayasu's disease presenting as a more acute inflammatory ocular picture before any signs of the chronic ischaemic changes in the eye can be found.