Takayasu arteritis: severe consequences of delayed diagnosis

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