Match!

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

Published on Aug 1, 2016in Rheumatology5.149
· DOI :10.1093/rheumatology/kew273
Christian Dejaco27
Estimated H-index: 27
(University of Graz),
Christina Duftner24
Estimated H-index: 24
+ 2 AuthorsBhaskar Dasgupta35
Estimated H-index: 35
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.
  • References (108)
  • Citations (46)
📖 Papers frequently viewed together
1,528 Citations
207 Citations
201651.27JAMA
112 Citations
78% of Scinapse members use related papers. After signing in, all features are FREE.
References108
Newest
#1Malte A. KlugerH-Index: 11
#2Simon MelderisH-Index: 2
Last. Oliver M. SteinmetzH-Index: 32
view all 13 authors...
Systemic lupus erythematosus (SLE) is a complex and potentially fatal autoimmune disorder. Although Th17 cells are thought to be central mediators of SLE, mechanisms underlying their counter regulation remain largely unknown. To help define this, we studied the function of the newly defined Stat3-dependent Th17-specific regulatory T cells (Treg17). Treg-specific deletion of Stat3 was achieved by generating Foxp3 Cre × Stat3 fl/fl mice and SLE was induced by intraperitoneal injection of pristane....
37 CitationsSource
#1Andreas P. Diamantopoulos (Hospital of Southern Norway)H-Index: 17
#2Glenn Haugeberg (NTNU: Norwegian University of Science and Technology)H-Index: 33
Last. G. Myklebust (Hospital of Southern Norway)H-Index: 7
view all 4 authors...
Permanent visual impairment has been reported to occur in up to 19% of GCA patients. The aim of this study was to examine whether implementation of a fast-track approach could reduce the rate of permanent visual impairment and inpatient days of care in GCA patients.A fast-track outpatient GCA clinic (FTC) was implemented in the Department of Rheumatology, Hospital of Southern Norway Trust Kristiansand, Norway in 2012. The patients included in this study were subsequently recruited between March ...
48 CitationsSource
#1Javier NarváezH-Index: 31
#2Paula EstradaH-Index: 3
Last. Joan M. NollaH-Index: 31
view all 8 authors...
Abstract Objective To investigate the frequency and type of giant cell arteritis (GCA)-related ischemic complications in a series of patients with GCA who, for a substantial period of time (i.e., at least 3 mo), lacked vascular symptoms and presented with apparently isolated polymyalgia rheumatica (PMR). Methods Retrospective follow-up study of an unselected population of 167 patients with GCA diagnosed from 1985 to 2014. Results In all, 18 patients (11%) developed GCA on a background of a prior...
22 CitationsSource
#1Francesco Ciccia (University of Palermo)H-Index: 30
#2Aroldo RizzoH-Index: 20
Last. Giovanni Triolo (University of Palermo)H-Index: 35
view all 11 authors...
OBJECTIVE: GCA is a large- and medium-vessel arteritis characterized by a range of histological patterns of vascular wall injury. The aim of this study was to immunologically characterize the various histological patterns of GCA. METHODS: Thirty-five consecutive patients with biopsy-proven GCA and 15 normal controls were studied. IL-8, IL-9, IL-9R, IL-17, IL-4, TGF-β and thymic stromal lymphopoietin expression was evaluated by RT-PCR and immunohistochemistry on artery biopsy specimens. Confocal ...
31 CitationsSource
#1C. Lavado-Pérez (UC: University of Cantabria)H-Index: 4
#2Isabel Martínez-Rodríguez (UC: University of Cantabria)H-Index: 11
Last. José M. Carril (UC: University of Cantabria)H-Index: 16
view all 13 authors...
Abstract Purpose Polymyalgia rheumatica (PMR) may present together with large vessel vasculitis (LVV), and frequently requires a more intensive therapy. The aim of the study was to evaluate the impact of 18 F-FDG PET/CT in the diagnosis and management of LVV associated to PMR. Material and methods This prospective study included 40 consecutive patients (27 women/13 men, 68.10 ± 10.27 years) with PMR and suspicion of associated LVV submitted for 18 F-FDG PET/CT. A PET/CT scan was obtained 180 min...
14 CitationsSource
#1Jack N. AlpertH-Index: 1
#2Shiv SaidhaH-Index: 24
Last. Omar Al-LouziH-Index: 8
view all 3 authors...
Al-Louzi et al.1 reported a patient with temporal arteritis diagnosed by PET scan following a negative temporal artery biopsy (TAB). The patient had strong history for temporal arteritis and an increased erythrocyte sedimentation rate (ESR), prompting a temporal artery biopsy, which was negative. Two weeks later, after discontinuing oral prednisolone, she developed jaw claudication, scalp tenderness, and pelvic and shoulder pain. ESR was 107 mm/h and C-reactive protein was 151.1 mg/L. The patien...
1 CitationsSource
#1Sarah L. Mackie (Chapel Allerton Hospital)H-Index: 19
#2John C. Taylor (Chapel Allerton Hospital)H-Index: 1
Last. Ann W. Morgan (Chapel Allerton Hospital)H-Index: 44
view all 13 authors...
Introduction Giant cell arteritis (GCA) is an autoimmune disease commonest in Northern Europe and Scandinavia. Previous studies report various associations with HLA-DRB1*04 and HLA-DRB1*01; HLA-DRB1 alleles show a gradient in population prevalence within Europe. Our aims were (1) to determine which amino acid residues within HLA-DRB1 best explained HLA-DRB1 allele susceptibility and protective effects in GCA, seen in UK data combined in meta-analysis with previously published data, and (2) to de...
11 CitationsSource
#1Hiroyuki TakahashiH-Index: 10
#2Hiroyuki YamashitaH-Index: 13
Last. Akio MimoriH-Index: 25
view all 10 authors...
AbstractObjectives. To compare the fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) findings in patients with elderly-onset rheumatoid arthritis (EORA) with those in patients with polymyalgia rheumatica (PMR), two conditions with similar clinical presentations.Methods. We retrospectively analyzed the FDG-PET/CT findings in 10 patients with EORA and 27 patients with PMR admitted to our department between 2006 and 2012.Results: No significant difference was observed...
20 CitationsSource
#1Tanaz A. Kermani (UCLA: University of California, Los Angeles)H-Index: 18
#2Cynthia S. Crowson (Mayo Clinic)H-Index: 72
Last. Kenneth J. Warrington (Mayo Clinic)H-Index: 33
view all 6 authors...
Abstract Objective To compare clinical and imaging characteristics of patients with giant cell arteritis (GCA) and upper extremity (UE) arterial involvement to patients with Takayasu arteritis (TAK). Methods A cohort of patients seen at the Mayo Clinic with TAK diagnosed between 1984 and 2009 and a cohort of patients with GCA and UE arterial involvement diagnosed between 1999 and 2008 were studied. Results The TAK cohort consisted of 125 patients (91% female); the mean age (±SD) at diagnosis was...
22 CitationsSource
#1J. Goupil de Bouillé (François Rabelais University)H-Index: 2
#2M. Lecouffe-Desprets (François Rabelais University)H-Index: 1
Last. Elisabeth Diot (François Rabelais University)H-Index: 31
view all 8 authors...
Resume Nous rapportons le cas d’une patiente de 76 ans avec un phenomene de Raynaud unilateral et isole, revelateur d’une arterite a cellules geantes (maladie de Horton), compliquee d’une atteinte arterielle etendue et d’une stenose ostiale bilaterale des arteres renales. Une echographie-Doppler retrouvait une stenose des arteres sous-clavieres et axillaires bilaterales. Une cartographie arterielle par angioscanner et TEP-scanner montrait une atteinte arterielle diffuse interessant principalemen...
2 CitationsSource
Cited By46
Newest
#1Pieter H Nienhuis (UMCG: University Medical Center Groningen)
#2Maria Sandovici (UMCG: University Medical Center Groningen)H-Index: 11
Last. Elisabeth Brouwer (UMCG: University Medical Center Groningen)H-Index: 36
view all 5 authors...
Abstract Background and aim Assessing cranial artery inflammation plays an important role in the diagnosis of cranial giant cell arteritis (C-GCA). However, current diagnostic tests are limited. The use of fluorine-18-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT imaging is an established tool for assessing large vessel inflammation but is currently not used for assessment of the cranial arteries. This study aimed to evaluate the accuracy of FDG-PET/CT in the diagnosis of biopsy...
Source
#1Tal Gazitt (UW: University of Washington)
#2Devy ZismanH-Index: 15
Last. Gregory C. Gardner (UW: University of Washington)H-Index: 12
view all 3 authors...
PURPOSE OF THE REVIEW Polymyalgia rheumatica (PMR) is one of the most common inflammatory rheumatologic condition occurring in older adults. It is characterized by proximal pain and stiffness in the shoulders, neck, and/or pelvic girdle in individuals over 50 years of age along with evidence of an intense systemic inflammatory response. Although the above clinical symptoms are very characteristic for the condition, it can be mimicked by other autoimmune, infectious, malignant, and endocrine diso...
Source
PURPOSE OF REVIEW The aim of this review paper is to evaluate the current data regarding clinical use of ultrasound (US) for diagnosis of systemic vasculitis. RECENT FINDINGS In recent years, US has emerged as an image modality taking a central role in the diagnosis and monitoring of vasculitides by measuring vessel wall inflammation and changes in vessel wall thickness. US has been recognized as an important tool predominantly in the diagnosis of large vessel vasculitis (LVV). Signs like the "h...
Source
#1Hannah Lyons (University of Birmingham)H-Index: 1
#2Vanessa QuickH-Index: 1
Last. Susan P. MollanH-Index: 12
view all 5 authors...
The landscape of the investigation and management of giant cell arteritis (GCA) is advancing. In this review we will outline the recent advances by searching the current English literature for relevant articles using key words of giant cell arteritis, temporal arteritis, Horton’s disease, investigation, and treatment. Delay in diagnosis, diagnostic uncertainty and glucocorticoid (GC) morbidity are among the highest concerns of clinicians and patients in this disease area. The positive news is th...
2 CitationsSource
#1Hiroshi Hori (Jichi Medical University)
#2Tsuyoshi Kobashigawa (Jichi Medical University)H-Index: 12
Last. Hitoshi Sugawara (Jichi Medical University)H-Index: 12
view all 4 authors...
A 77-year-old man visited the hospital with a chronic cough persisting for 2.5 months accompanied with night sweats, weight loss (3.5 kg) and elevated C-reactive protein level. Chest CT of the lung field was normal, but aortic wall thickening accompanied by a contrast effect was noted. Positron emission tomography-CT (PET-CT) showed that the aorta and subclavian artery were inflamed, suggesting large-vessel vasculitis. Ultrasonography showed thickening of the superficial temporal artery wall (ma...
Source
#1Endre KolossváryH-Index: 6
#2György BalázsH-Index: 9
Last. Katalin Farkas (Semmelweis University)H-Index: 1
view all 6 authors...
According to the nomenclature of the Chapel Hill Consensus Conference, giant-cell arteritis and Takayasu's arteritis belong to the group of large-vessel vasculitis. Recognition of these diseases is primarily based on the clinical assessment and the use of various vascular imaging modalities. With regard to the latter one, significant technological advances have been observed in recent years, which allow not only to make a diagnosis but also to evaluate the extent of the disease and the degree of...
Source
Giant cell arteritis (GCA) is a granulomatous vasculitis involving large- and middle-sized arteries, with a predilection for cranial arteries. In this paper, we aim to describe a case of a biopsy- and ultrasound-proven GCA, presenting with anterior ischemic optic neuropathy (AION) and normal CRP and ESR at diagnosis and during follow-up, and to review the literature on GCA cases with low inflammatory markers, with a particular focus on the presence of eye involvement. Both CRP and ESR are import...
Source
#1Emma Rubenstein (SLU: Saint Louis University)
#2Carla Maldini (SLU: Saint Louis University)H-Index: 1
Last. A. Mahr (SLU: Saint Louis University)H-Index: 7
view all 5 authors...
OBJECTIVES: Temporal artery biopsy (TAB) is a reference test for the diagnosis of GCA but reveals inflammatory changes only in a subset of patients. The lack of knowledge of TAB sensitivity hampers comparisons with non-invasive techniques such as temporal artery ultrasonography. We performed a systematic literature review and meta-analysis to estimate the sensitivity of TAB in GCA and to identify factors that may influence the estimate. METHODS: A systematic literature review involved searching ...
6 CitationsSource
Giant cell arteritis (GCA) may affect the brain-supplying arteries, resulting in ischemic stroke, whereby the vertebrobasilar territory is most often involved. Since etiology is unknown in 25% of stroke patients and GCA is hardly considered as a cause, we examined in a pilot study, whether screening for GCA after vertebrobasilar stroke might unmask an otherwise missed disease. Consecutive patients with vertebrobasilar stroke were prospectively screened for GCA using erythrocyte sedimentation rat...
Source
A 74-year-old woman presented with a 6-month history of malaise, generalized fatigue, significant unintentional weight loss, and night sweats, as well as pain, weakness, and stiffness in the shoulder girdle most severe in the morning. She reported no headache, visual disturbances, or jaw
Source