Giant cell arteritis (GCA) is the most frequent primary systemic vasculitis, involving large vessels (LV) among patients over 50, particularly extracranial branches of the carotid arteries such as superficial temporal arteries. Characteristic symptoms include headache, scalp tenderness, jaw claudication, partial or complete vision loss, fever, asthenia, loss of weight, and polymyalgia rheumatica (PMR). It is now well known that patients may also have aortitis and/or inflammation of the main aortic branches, leading to complications such as aortic dissection or aneurism [1].

A striking but rare complication is the development of scalp, tongue, lip, or even nasal septum necrosis. Cooke et al. reported the first two cases of scalp necrosis in GCA and less than 150 cases have been reported so far [2]. The largest review of the literature retrospectively analysed 78 cases of GCA with scalp necrosis ranging from 1946 to 2007 [3]. Data suggest that scalp, tongue, or lip necrosis occur in older patients, is associated to higher incidence of vision loss and increased mortality [3], [4], [5], [6], [7]. However, precise and recent data on mortality, ophthalmic events, relapses or vascular events in GCA patients with such complications remain very scarce.

The aims of the present study were to compare baseline variables that distinguish patients with and without such necrosis and to compare treatment, outcome and prognosis of these two subtypes of GCA patients.