A 52-year-old Indian lady with no past medical history presented with three acute self-limiting episodes of a painful red right eye over the course of a year. There was photophobia but no loss of vision. Closer examination of her eye revealed anterior scleritis and scleromalacia (Figure 1A). She also had worsening dyspnoea, fatigue, and unintentional weight loss over the last few months. There was no history of limb claudication. On examination, peripheral pulses were present throughout but there was a differential brachial blood pressure in her arms. No bruits were auscultated over her carotid, subclavian, interscapular, and abdominal aorta regions. Cardiovascular evaluation revealed severe aortic regurgitation and cardiomyopathy. A computed tomography aortogram showed extensive ascending and descending thoracic aortitis and mediastinal lymphadenopathy, (Figure 1B, arrows) as well as inflammation of the right brachiocephalic and left carotid arteries. Her erythrocyte sedimentation rate was raised at 72 millimeter/hour (0 to 20 millimeter/hour). Anti-nuclear antibodies (ANA), anti-double stranded DNA antibodies (ds-DNA), and antineutrophil cytoplasmic antibodies (ANCA) were negative. Evaluation for tuberculosis and syphilis was negative. Our patient only fulfilled 2 of the 6 American College of Rheumatology (ACR) diagnostic criteria for Takayasu’s arteritis (differential BP and arteriographic narrowing). However, the ACR criteria are mainly used as a research tool and have limited use in clinical practice. Given the constellation of clinical features and extensive large-vessel involvement, a diagnosis of Takayasu’s arteritis was made. She was started on intravenous methylprednisolone and cyclophosphamide with improvement in her symptoms.

Takaysu’s arteritis is a large vessel vasculitis with a predilection for the aorta and its major branches. Ocular manifestations of Takayasu’s arteritis have a prevalence of 8.1–68% [1]. They include retinal artery and vein occlusion, hypertensive retinopathy, Takayasu’s retinopathy and treatment-related complications such as steroid-induced glaucoma. However, only a handful of cases in the literature describe an association between scleritis and the disease [2],[3],[4],[5],[6],[7],[8],[9]—a feature more commonly found in small vessel vasculitis. It is important to recognize this association as early treatment can lead to significant improvement in symptoms and better disease control.

Scleritis can be the first manifestation of Takayasu’s arteritis. Clinicians should be aware of this as it is a debilitating disease that can be easily treated.