title: The spectrum of giant cell arteritis and polymyalgia rheumatica: revisiting the concept of the disease.
abstract: GCA and PMR are conditions of older persons that frequently overlap. The traditional concept of GCA has focused on cranial symptoms such as headache and visual disturbance, but extra-cranial manifestations such as constitutional symptoms, polymyalgia and limb claudication have also long been recognized. These symptoms may coincide with cranial GCA, occur as an independent clinical subset [large-vessel (LV) GCA] or overlap with PMR. Imaging studies have demonstrated that up to one-third of patients with PMR have subclinical LV inflammation at disease outset. The implication of this finding for PMR management is unclear. Pathophysiological studies have emphasized the pivotal role of dendritic cells (DCs) and T cells in the pathogenesis of GCA, and the activation of certain pattern recognition receptors on DCs may determine the clinical subset of GCA. In patients with only PMR clinically, it is conceivable that transmural arterial inflammation has either not yet started or is prevented by unexplored regulatory pathways. This concept is supported by vasculitis of peri-adventitial small-vessels and activated DCs in the adventitia of temporal arteries, in the absence of media-infiltrating T cells. This review examines the clinical and pathophysiological spectrum of GCA and its subsets with PMR, the role of newer imaging techniques for GCA diagnosis and the management of these diseases.
authors.affiliation: Department of Rheumatology and Immunology, Medical University Graz, Graz.
authors.forename: Christian
authors.initials: C
authors.lastname: Dejaco
authors.affiliation: Department of Internal Medicine VI, Medical University Innsbruck, Innsbruck.
authors.forename: Christina
authors.initials: C
authors.lastname: Duftner
authors.affiliation: Department of Rheumatology and Clinical Immunology, Charité University Medicine, Berlin, Germany.
authors.forename: Frank
authors.initials: F
authors.lastname: Buttgereit
authors.affiliation: Division of Rheumatology and Division of Epidemiology, Departments of Internal Medicine and Health Sciences Research, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: Eric L
authors.initials: EL
authors.lastname: Matteson
authors.affiliation: Department of Rheumatology, Southend University Hospital, Southend, UK.
authors.forename: Bhaskar
authors.initials: B
authors.lastname: Dasgupta
mesh_terms: D000368:Aged; D016207:Cytokines / Q000502:physiology; D003713:Dendritic Cells / Q000502:physiology; D003937:Diagnosis, Differential; D019788:Fluorodeoxyglucose F18; D013700:Giant Cell Arteritis / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D018810:Magnetic Resonance Angiography; D011111:Polymyalgia Rheumatica / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D049268:Positron-Emission Tomography; D019275:Radiopharmaceuticals; D013601:T-Lymphocytes / Q000502:physiology
chemical_list: D016207:Cytokines; D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18
keywords: biological therapies; diagnostic imaging; giant cell arteritis; immunosuppressants; polymyalgia rheumatica; vasculitis