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Objective The main objective of this study was to analyse the prevalence and characteristics of subclinical GCA in patients with PMR. Methods This was a cross-sectional multicentre international study of consecutive patients with newly diagnosed PMR without symptoms or signs suggestive of GCA. All patients underwent US of the temporal superficial, common carotid, subclavian and axillary arteries. Patients with halo signs in at least one examined artery were considered to have subclinical GCA. The clinical, demographic and laboratory characteristics of the PMR group without subclinical vasculitis were compared with subclinical GCA, and the pattern of vessel involvement was compared with that of a classical single-centre GCA cohort. Results We included 346 PMR patients, 267 (77.2%) without subclinical GCA and 79 (22.8%) with subclinical GCA. The PMR patients with subclinical GCA were significantly older, had a longer duration of morning stiffness and more frequently reported hip pain than PMR without subclinical GCA. PMR with subclinical GCA showed a predominant extracranial large vessel pattern of vasculitic involvement compared with classical GCA, where the cranial phenotype predominated. The patients with PMR in the classical GCA group showed a pattern of vessel involvement similar to classical GCA without PMR but different from PMR with subclinical involvement. Conclusion More than a fifth of the pure PMR patients had US findings consistent with subclinical GCA. This specific subset of patients showed a predilection for extracranial artery involvement. The optimal screening strategy to assess the presence of vasculitis in PMR remains to be determined. [ABSTRACT FROM AUTHOR]

Background and aim: Giant cell arteritis (GCA) remains a medical emergency because of the risk of sudden irreversible sight loss and rarely stroke along with other complications. Because headache is one of the cardinal symptoms of cranial GCA, neurologists need to be up to date with the advances in investigation and management of this condition. The aim of this document by the European Headache Federation (EHF) is to provide an evidence-based and expert-based recommendations on GCA. Methods: The working group identified relevant questions, performed systematic literature review and assessed the quality of available evidence, and wrote recommendations. Where there was not a high level of evidence, the multidisciplinary (neurology, ophthalmology and rheumatology) group recommended best practice based on their clinical experience. Results: Across Europe, fast track pathways and the utility of advanced imaging techniques are helping to reduce diagnostic delay and uncertainty, with improved clinical outcomes for patients. GCA is treated with high dose glucocorticoids (GC) as a first line agent however long-term GC toxicity is one of the key concerns for clinicians and patients. The first phase 2 and phase 3 randomised controlled trials of Tocilizumab, an IL-6 receptor antagonist, have been published. It is now been approved as the first ever licensed drug to be used in GCA. Conclusion: The present article will outline recent advances made in the diagnosis and management of GCA. [ABSTRACT FROM AUTHOR]

Aortitis due to giant cell arteritis (GCA) is rare but probably underestimated given the frequent paucity of symptoms. Thus, early studies relied on the occurrence of complications to estimate the prevalence of GCA aortitis. With this method, aortitis was a feature in 3 to 18% of GCA patients. Since then, the introduction of modern imaging techniques has established that aortitis is more common than previously thought. Aortitis should be considered in patients with atypical clinical presentations of GCA consisting, for instance, in isolated laboratory evidence of systemic inflammation or a relapse during treatment. Aortitis may be difficult to diagnose, as temporal artery biopsy has limited sensitivity in patients with predominant large-vessel involvement. Positron emission tomography (PET) and magnetic resonance imaging (MRI) are both highly effective for the early diagnosis of aortitis. Case-series evaluating PET in patients with GCA found evidence of aortitis in over half the cases, with predominant involvement of the thoracic aorta. To date, no evidence is available about the potential usefulness of PET or MRI in monitoring patients with GCA aortitis over time. Involvement of the aorta and other large arteries does not change the treatment strategy, which rests on corticosteroid therapy. Administration of a corticosteroid-sparing drug should be considered, most notably when a relapse occurs. Aortitis is associated with an increased risk of aneurysm of the thoracic aorta. Consequently, all GCA patients should be monitored for aneurysm at regular intervals, even after treatment discontinuation. The recommended strategy is an annual evaluation including a chest radiograph, echocardiogram, and abdominal Doppler sonogram; these imaging studies can be replaced by contrast-enhanced computed tomography of the chest and abdomen., (Copyright © 2010 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.)

Cerebral vasculitis represents a rare form of vascular inflammatory involvement caused by heterogeneous conditions. In this study, the wide spectrum of cerebral vasculitis despite primary angiitis of the CNS is analyzed. Our cohort included 16 white patients with cerebral vasculitis treated in a single German institution between 2003 and 2008. Clinical and diagnostic features were obtained by retrospective chart review; follow-up information and outcome were obtained prospectively. The spectrum of conditions responsible for cerebral vasculitis included seven patients with Behçet syndrome and one case each of giant cell arteritis, Wegener's granulomatosis and Churg-Strauss syndrome, respectively. Vasculitis secondary to systemic diseases included two patients with systemic lupus erythematodes, one with sarcoidosis and one with ANA-positive systemic vasculitis. Two patients suffered from infectious angiitis caused by borreliosis and syphilis. The mean age at onset of cerebral symptoms was 41.38 years. The most frequent clinical symptoms were headache, gait disturbances and unilateral numbness. None of the patients with Behçet syndrome experienced any ischemic event, which was a significant difference compared with the other patients (P = 0.011). Gadolinium-enhancing lesions were significantly more frequent in Behçet syndrome compared to the other types of vasculitis (P = 0.041). There was no significant difference between vasculitis patients with or without Behçet syndrome regarding outcome parameters. The differential diagnosis of conditions responsible for cerebral vasculitis includes a wide spectrum of diseases. Clinical features and the course of cerebral vasculitis are highly variable. The enigma of cerebral vasculitis will only be solved by implementing large, prospective, multicenter databases.

Temporal arteritis is a disease of older individuals that manifests with variable signs, symptoms, and pain in the head and jaw. This disease should be considered in the differential diagnosis if the dentist encounters a patient with puzzling complaints that are not explained by oral and physical findings.