The spectrum of giant cell arteritis and polymyalgia rheumatica: Revisiting the concept of the disease

Christian Dejaco, Christina Duftner, Frank Buttgereit, Eric Lawrence Matteson, Bhaskar Dasgupta

Research output: Contribution to journalReview article

52 Citations (Scopus)

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.

Original languageEnglish (US)
Article numberkew273
Pages (from-to)506-515
Number of pages10
JournalRheumatology (United Kingdom)
Volume56
Issue number4
DOIs
StatePublished - Apr 1 2017

Fingerprint

Polymyalgia Rheumatica
Giant Cell Arteritis
Dendritic Cells
Adventitia
T-Lymphocytes
Pattern Recognition Receptors
Temporal Arteries
Arteritis
Disease Management
Vasculitis
Headache
Extremities
Inflammation

Keywords

  • Biological therapies
  • Diagnostic imaging
  • Giant cell arteritis
  • Immunosuppressants
  • Polymyalgia rheumatica
  • Vasculitis

ASJC Scopus subject areas

  • Rheumatology
  • Pharmacology (medical)

Cite this

The spectrum of giant cell arteritis and polymyalgia rheumatica : Revisiting the concept of the disease. / Dejaco, Christian; Duftner, Christina; Buttgereit, Frank; Matteson, Eric Lawrence; Dasgupta, Bhaskar.

In: Rheumatology (United Kingdom), Vol. 56, No. 4, kew273, 01.04.2017, p. 506-515.

Research output: Contribution to journalReview article

Dejaco, Christian ; Duftner, Christina ; Buttgereit, Frank ; Matteson, Eric Lawrence ; Dasgupta, Bhaskar. / The spectrum of giant cell arteritis and polymyalgia rheumatica : Revisiting the concept of the disease. In: Rheumatology (United Kingdom). 2017 ; Vol. 56, No. 4. pp. 506-515.
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