Scleritis Occurring in Association With Takayasu Disease

Takayasu disease (TD) (pulseless disease, aortic arch syndrome) is a rare but potentially life-threatening chronic giant cell vasculitis. We describe a patient with Takayasu disease who initially had scleritis. Associated with other systemic vasculitic conditions in approximately one half of cases,¹ a relationship between TD and scleritis has not been well documented.

A 36-year-old Filipino-Hawaiian woman had a 4-year history of fluctuating bilateral ocular redness and episodes of vision loss, along with intermittent numbness and stiffness in her left arm and neck, with exertion, palpitations, breathlessness, lightheadedness sometimes progressing to syncope, and fatigue. Her medical history included anemia.

Visual acuities were 20/25 OD and 20/20 OS. There was non-necrotizing sectorial scleral inflammation OS, as well as bilateral circumferential scleral thinning, but both corneas were healthy. Further ocular examination was unremarkable. Systemic examination revealed a systolic cardiac murmur, bilateral carotid and subclavian bruits, and diminished carotid and left arm pulses. Upper limb blood pressures were 160/90 (right arm) and 80/60 (left arm).

Laboratory results included an erythrocyte sedimentation rate of 102 mm/h and hypergammaglobulinemia. Syphilis serology was nonreactive. Antinuclear antibodies, rheumatoid factor, and anti-neutrophil cytoplasmic antibodies were not detected. Chest x-ray film showed an enlarged aortic knob. Aortography demonstrated dilatation of the arch and irregular stenoses of its branches, particularly the left common carotid and subclavian arteries (Figure 1). During this outpatient evaluation, the patient experienced a transient ischemic attack that caused expressive aphasia.

Treatment began with 3 intravenous methylprednisolone pulses (1 g/d), followed by high-dose oral prednisone (60 mg/d) that was slowly tapered, in combination with oral methotrexate (20 mg/wk) and folate supplementation and aspirin (80 mg/d). High-grade left common carotid stenosis prevented stenting, but stents have been deployed in the left subclavian and right innominate arteries. Two years after diagnosis, the patient was taking methotrexate, and remained systemically well with inactive scleritis.

Takayasu vasculitis generally involves large- and medium-sized arteries, in particular, the great vessels. A presumed T cell–mediated autoimmune inflammation of the vasa vasorum progresses to fibrosis, and resultant arterial stenoses, occlusions, and aneurysms cause various ischemic symptoms.² Estimated incidence is 2.6 cases per million persons per year, and most new patients are women of reproductive age, with an Asian or Hispanic ethnic background.²

Our patient's case was typical, meeting all 6 American College of Rheumatology 1990 criteria. Three diagnostic criteria are required, including onset by age 40, claudication of the extremities, decreased brachial pulse(s), upper limb systolic blood pressure difference greater than 10 mm Hg, aortic or subclavian artery bruit(s), and angiographic narrowing of large arteries.² Prominent constitutional symptoms are also common, as are the laboratory findings of anemia, raised erythrocyte sedimentation rate, and elevated serum γ globulins.² Syphilis, an important differential diagnosis, was excluded by serological testing. There was no clinical or laboratory evidence for other autoimmune diseases clearly associated with scleritis, eg, rheumatoid arthritis and Wegener granulomatosis.

Ocular abnormalities are common in TD. Characteristic Takayasu retinal vasculopathy with potential neovascular complications occurs in a third of cases, and hypertensive retinopathy affects 20% of patients.³ Visual aberrations may also result from cerebral ischemia. To our knowledge scleritis has not previously been well recognized as a feature of this disease. Two reports describe scleritis in patients with TD, but both individuals also had Wegener granulomatosis, a well-known cause of scleritis.^4,5 Causality cannot be proven, but the strong temporal relationship between active vasculitis involving sclera and great vessels strongly suggests a real association between TD and scleritis. Furthermore, temporal arteritis, the more common form of giant cell arteritis, may present with scleritis.⁶

Management of TD involves systemic immunosuppression for acute lesions and surgery for fibrotic pathology.² Corticosteroids reduce inflammation but are not curative. Methotrexate may be useful in inducing a remission. Our patient's scleritis and constitutional symptoms responded to immunosuppression, but surgery was required to relieve the symptoms of cicatrizing arterial stenoses. Aspirin was prescribed empirically, as there is no established role for anticoagulants in managing this disease.²

We document an association between TD and scleritis. Although rare, it is often possible to diagnose TD from the clinical history. A 5-year mortality rate as high as 35%² requires that this condition not be overlooked. The diagnosis would have been expedited in our patient if there had been previous documentation of a clear association between TD and scleritis. Since acceptance of this paper, Jain et al⁷ have published a report that describes a case of TD occurring in association with scleritis.

We wish to acknowledge the support of grant EYO6484 from the National Institutes of Health (Dr Rosenbaum), Bethesda, Md; Research to Prevent Blindness Inc, New York, NY (Dr Rosenbaum); and grant 997099 from the National Health and Medical Research Council of Australia, Canberra ACT (Dr Smith).

Corresponding author: Justine R. Smith, MBBS, PhD, Casey Eye Institute, Oregon Health Sciences University, 3375 SW Terwilliger Blvd, Portland, OR 97201-4197 (e-mail: smithjus@ohsu.edu).