Takayasu arteritis: severe consequences of delayed diagnosis

Abstract

Takayasu arteritis (TA) is a rare, large-vessel granulomatous vasculitis of unknown aetiology. TA is a panarteritis characterized by T lymphocyte, monocyte and macrophage infiltration of the arterial wall, leading to marked intimal myofibroblast proliferation and fibrosis of the media and adventitia. This results in severe arterial stenosis, occlusion or aneurysmal dilatation.1 TA affects primarily the aorta and its main branches, especially the common carotid (CCA), subclavian (SCA) and pulmonary arteries.2 The clinical manifestations follow two phases. In the early pre-pulseless phase, patients may complain of systemic symptoms including fever, weight loss, malaise, headaches, carotidynia, myalgia and arthralgia. In the later pulseless phase, commonly appearing months–years later,2 symptoms reflect end-organ ischaemia and include limb claudication and neurological symptoms. Decreased or absent peripheral pulses, aortic regurgitation, arterial bruits and systemic arterial hypertension are common. The non-specific nature of presenting symptoms and the lack of specific laboratory markers delays the diagnosis, with potentially life-threatening consequences.2,3 Moreover, although diagnostic criteria exist,4 patients presenting with early pre–stenotic disease may not fulfil them.5 Review of our 68 patients showed that 17 required angioplasty or surgery. We suggest that a significant delay in diagnosis of >12 months contributed to the need for surgery in 10 of the 17 patients. In support of this and with their consent, we use the histories of four patients that demonstrate the serious consequences associated with delayed diagnosis, and propose that this might be prevented by increased clinical awareness of TA and early use of non-invasive imaging. A 25-year-old British woman presented to her general practitioner (GP) with anterior neck pain, malaise and fatigue. Lymphadenopathy was diagnosed and she received treatment with antibiotics. The symptoms persisted and she developed occasional dizziness and transient loss of vision in the right eye. The …