Giant Cell Arteritis (GCA): Pathogenesis, Clinical Aspects and Treatment Approaches

2019 ◽  
Vol 15 (4) ◽  
pp. 259-268
Author(s):  
Andrea Ciofalo ◽  
Giampiero Gulotta ◽  
Giannicola Iannella ◽  
Benedetta Pasquariello ◽  
Alessandra Manno ◽  
...  

: Giant Cell Arteritis (GCA), or Horton’s Arteritis, is a chronic form of vasculitis of the large and medium vessels, especially involving the extracranial branches of the carotid arteries, in particular, the temporal artery, with the involvement of the axillary, femoral and iliac arteries too. Arterial wall inflammation leads to luminal occlusion and tissue ischemia, which is responsible for the clinical manifestations of the disease. : A substantial number of patients affected by GCA present head and neck symptoms, including ocular, neurological and otorhinolaryngological manifestations. : The aim of this article is to present pathogenesis, clinical aspects and treatment approaches of GCA manifestations.

2018 ◽  
Vol 69 (1) ◽  
pp. 152-154
Author(s):  
Vasilica Cristescu ◽  
Aurelia Romila ◽  
Luana Andreea Macovei

Polymyalgia rheumatica is a disease that occurs mostly in the elderly and is rarely seen in patients less than 50 years of age. Polymyalgia rheumatica is a vasculitis, which manifests itself as an inflammatory disease of the vascular wall that can affect any type of blood vessel, regardless of its size. It has been considered a form of giant cell arteritis, involving primarily large and medium arteries and to a lesser extent the arterioles. Clinical manifestations are caused by the generic pathogenic process and depend on the characteristics of the damaged organ. PMR is a senescence-related immune disorder. It has been defined as a stand-alone condition and a syndrome referred to as rheumatic polyarteritis with manifestations of giant cell arteritis (especially in cases of Horton�s disease and temporal arteritis) which are commonly associated with polymyalgia. The clinical presentation is clearly dominated by the painful girdle syndrome, with a feeling of general discomfort. Polymyalgia and temporal arteritis may coexist or be consecutive to each other in the same patient, as in most of our patients. The present study describes 3 cases of polymyalgia rheumatica, admitted to the Clinic of Rheumatology of Sf. Apostol Andrei Hospital, Galati. The cases were compared with the literature. Two clinical aspects (polymyalgia rheumatica and/or Horton�s disease) and the relationship between them were also considered. Polymyalgia rheumatica is currently thought to have a multifactorial etiology, in which the following factors play a role: genetic factors or hereditary predisposition (some individuals are more prone to this disease), immune factors and viral infections (triggers of the disease). Other risk factors of polymyalgia rheumatica include age over 50 years and the association with giant cell arteritis. The characteristic feature of the disease is girdle pain, with intense stiffness of at least one hour�s duration. Markers of inflammation, erythrocyte sedimentation rate and C-reactive protein are almost always increased at the onset of the disease. Diseases that can mimic the clinical picture of polymyalgia rheumatica are neoplasia, infections, metabolic disorders of the bone and endocrine diseases.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1541.1-1542
Author(s):  
M. Jasim ◽  
P. Magan ◽  
R. Awadalla ◽  
R. Brindley ◽  
D. Richards ◽  
...  

Background:Giant cell arteritis (GCA) is the most common type of large vessel vasculitis. Typically it presents in patients over the age of 50 with a combination of temporal headaches, scalp tenderness, jaw claudication, raised inflammatory markers and visual disturbance. The diagnosis of GCA is often challenging and there is a difficult balance of over and under investigation. There have been several proposed scoring systems to help clinicians risk stratify patients who may present with suspected GCA. One such scoring system, published in 2017, showed clinical utility in a large international multi-centre study. Following analysis by logistic regression on data from 530 biopsies, Ing et al. developed a parsimonious prediction model comprising 5 candidate criteria: age, jaw claudication, ischemia-related loss of visual acuity, platelet count and logCRP (Figure 1).[1]Objectives:Increasingly, ultrasound doppler imaging is recognised and accepted as satisfactory means of confirming the diagnosis of GCA, with the presence of the halo sign characteristic for GCA. The aim of our study was to determine whether this GCA prediction model accurately predicts positive temporal artery biopsies in a large, real world UK cohort. In addition, we assessed whether this model accurately predicts positive temporal artery ultrasounds.Methods:A retrospective cohort study was performed using electronic medical records of patients referred for temporal artery biopsy (TAB) and temporal artery ultrasound (USTA) for suspected GCA. All TAB performed at the Royal Wolverhampton NHS Trust between June 2014 - June 2018 and all USTA performed between January 2015 - January 2019 were analysed. Patients who undergo USTA for suspected GCA at our centre routinely have bilateral temporal and axillary arteries scanned. Patients were excluded if they already had a previous diagnosis of GCA (and the clinical question was suspected flare), or if there was insufficient information available.Results:The total number of patients who underwent a confirmatory diagnostic test (either TAB or USTA) for suspected GCA was 187. Thirteen of these patients met the exclusion criteria, the remaining 174 patients were included for analysis. 126/174 patients underwent a TAB, 63/174 had an USTA. 15/174 had both these were included in the USS cohort because for all these patients the ultrasound was the first diagnostic test performed (Table 1). Our results appear to closely mirror the original multi-centre results with regards to prediction of biopsy positive GCA, with the centiles closely following those in the inception cohort. 0% of the ‘low’ risk probability biopsy cohort were misclassified - none had a positive biopsy. However, 8% of the ‘low’ risk probability ultrasound cohort were misclassified - 2 had a positive ultrasound.Table 1.Investigation outcome summaryTotal number of patients who underwent TAB +/or USS TA for?GCA187 - 13 patients rejectedN = 174TAB = 111USS = 63Of these 15 patients hadbothUSS & TABPositive TAB =31 (28%)Negative TAB =80 (72%)Positive USS =24 (38%)Negative USS =39 (62%)Conclusion:Our study, highlights that a probability score for GCA derived from a large multi-centre cohort of patients who were biopsy positive, predicts ultrasound positivity with similar accuracy. Our work reveals that scoring systems are not infallible but can be helpful in guiding clinical decision makingReferences:[1]Ing EB, Lahaie Luna G, Toren A, et al. Multivariable prediction model for suspected giant cell arteritis: development and validation.Clin Ophthalmol. 2017;11:2031–2042. Published 2017 Nov 22.Acknowledgments:Many thanks to the Rheumatology, Opthalmology & Ultrasound teams at Royal Wolverhampton NHS TrustDisclosure of Interests:None declared


2021 ◽  
Vol 108 (Supplement_1) ◽  
Author(s):  
BJW Chew ◽  
A Khajuria ◽  
J Ibanez

Abstract Introduction Guidelines recommend temporal artery biopsy (TAB) for patients suspected of having giant cell arteritis (GCA). We evaluated the impact of TAB on the diagnosis and management of patients with suspected GCA at a tertiary plastic surgery unit. Method A retrospective review of all TAB procedures performed at our centre over 7 years was performed. One hundred and one patients were included in the study. Patients were classified into 3 diagnostic groups: confirmed (positive TAB), presumed (negative TAB with high clinical suspicion) and unlikely (negative TAB with low clinical suspicion). The clinical presentation and management for each group were compared. Result The average American College of Rheumatology (ACR) score was 3.07. The number of patients with an ACR score of ≥3 before TAB was 72 (71.3%) and remained the same after TAB. The number of patients who remained on steroid therapy was lower in the group with an unlikely diagnosis of GCA compared to the group with a confirmed diagnosis (p<0.05). Conversely, there was no significant difference in steroid therapy between those with a presumed and confirmed diagnosis (p>0.05). Conclusion This study found a significant difference in steroid treatment between those with confirmed GCA and those where the diagnosis was unlikely showing that TAB may support decisions regarding steroid therapy. However, TAB was inappropriately requested for patients whose pre-TAB ACR score was ≥3 as this score is sufficient for the diagnosis of GCA. Therefore, the use of TAB should be limited to cases of diagnostic uncertainty. Take-home message while temporal artery biopsy has a role to play in the diagnosis of GCA, its use should be limited to cases of diagnostic uncertainty and not requested for every patient with a suspicion of Giant Cell Arteritis.


2010 ◽  
Vol 38 (2) ◽  
pp. 331-338 ◽  
Author(s):  
ELISE BELILOS ◽  
JUDY MADDOX ◽  
ROBERT M. KOWALEWSKI ◽  
JOLANTA KOWALEWSKA ◽  
GEORGE K. TURI ◽  
...  

Objective.To investigate the occurrence, clinical correlates, and immunohistochemical phenotype of temporal small-vessel inflammation (TSVI) in temporal artery biopsies from patients presenting with clinical features of giant cell arteritis (GCA).Methods.We retrospectively reviewed 41 temporal artery biopsy specimens for the presence of inflammatory infiltrates in small vessels external to the temporal artery adventitia (TSVI); 33 had sufficient clinical and pathological data for detailed analysis. Clinical and laboratory features at presentation and corticosteroid treatment patterns of patients with isolated TSVI were compared to those of patients with positive and negative biopsies. The cellular composition of the infiltrates was further characterized by immunohistochemistry.Results.Twenty-three (70%) specimens had evidence of TSVI including 10 with concurrent GCA and 13 (39%) with isolated TSVI. TSVI was found in all positive temporal artery biopsies. The proportion of macrophages and of lymphocyte subpopulations differed between infiltrates observed in TSVI and those of the main temporal artery wall. Initial erythrocyte sedimentation rate (ESR) was similar in the TSVI and positive biopsy groups and was significantly higher than in the negative biopsy group. Patients with isolated TSVI more often had symptoms of polymyalgia rheumatica compared to the positive biopsy group. Patients with TSVI received corticosteroid doses that were intermediate between patients with positive and those with negative biopsies.Conclusion.A significant number of patients with clinical features of GCA demonstrated isolated TSVI. Differences in the clinical presentation and cellular composition suggest that TSVI may represent a subset of GCA and should be considered in the interpretation of temporal artery biopsies and treatment decisions.


Author(s):  
Lara Sánchez Bilbao ◽  
Iñigo González-Mazón ◽  
D. Prieto-Peña ◽  
Monica Calderón-Goercke ◽  
José Luis Martín-Varillas ◽  
...  

VASA ◽  
2005 ◽  
Vol 34 (4) ◽  
pp. 269-271 ◽  
Author(s):  
Pfadenhauer ◽  
Rüll

Inflammation of the arterial wall has been demonstrated by 18 Fluoro-Deoxyglucose PET imaging in patients with Takayasu’s and temporal arteritis. We used ultrasonography and FDG-PET for structural and metabolic imaging of the carotid artery to diagnose giant cell arteritis without biopsy. In a 72 years old patient with isolated clinical and paraclinical signs of severe systemic inflammation ultrasonogaphy showed concentric hypoechogenic mural thickening of the carotid arteries and high FDG uptake in the left carotid, both axillary and subclavian arteries and the aorta. Clinical and paraclinical abnormalities showed a typical response to steroid treatment. In conclusion a combined approach using ultrasound and FDG-PET seems to be helpful in the diagnosis of GCA of large arteries particularily in patients with atypical manifestations of the disease and negative or unavailable biopsy of the temporal artery.


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Charlotte A Sharp ◽  
Jayne Little ◽  
Aqsam Shahbaz ◽  
E F Wood ◽  
Surabhi Wig ◽  
...  

Abstract Background/Aims  In 2017 an audit and survey of giant-cell arteritis (GCA) services were conducted across northwest England (reported previously). This re-survey in 2020, following publication of revised BSR guidance, sought to identify what changes were made in the intervening period, and provided the opportunity to assess the impact of COVID-19. Methods  Rheumatologists from 16 hospitals in northwest England were invited to complete a survey in July 2020. Questions focused on service provision for GCA, including pathways, diagnostics and steroid prescription. Results  Responses were received from 14/16 sites in 2017, and 15/16 in 2020. 9/15 (60%) sites reported that the 2017 audit and survey prompted changes to GCA services, with two (13%) stating that it clarified the need for implementation of existing plans. Two sites had a GCA pathway in 2017. Four of the seven sites who committed to introducing one have now done so, bringing the total in 2020 to six. Eight of the nine remaining sites plan to implement one, six with a specific date within six months. Six (40%) have completed additional local audit/QI since 2017. Temporal artery (TA) ultrasound (US) is now available in an additional four sites, bringing the total to 6/15 (40%) in 2020. Two sites reported improvement in both time between first rheumatology consultation and TA biopsy, and time to receive results (now <7 days for each task in 6/15 (40%)). Six additional sites reported providing leaflets on steroids routinely, bringing the total in 2020 to 12/15 (80%), versus 6/14 (43%) previously. Four sites (27%) now have a database of GCA patients (one in 2017). There was no major change in sites having a standard protocol for steroid taper (n = 8 2017; n = 7 2020, 89% and 100% of whom respectively use BSR guidance), nor in the number of patients routinely provided steroid cards (six in 2017; five in 2020). The three sites who do not report giving leaflets on steroids routinely, all had a pathway. 8/15 (53%) reported COVID-19 having an adverse effect upon services, including: reduced access to diagnostics (n = 7: TA US, biopsy, and PET-CT); delayed appointments (n = 4); delayed referrals (n = 3). The tertiary referral centre reported an improvement because access to tocilizumab was facilitated by a relaxation of rules by NHS England. Conclusion  The original audit and survey of current GCA practice in 2017 highlighted areas for improvement for each site, and regionally. Sites contributing to this re-survey report that the exercise stimulated them to improve their current care. The 2017 exercise showed a strong correlation between reported practice (survey) and actual practice (audit), leading us to have confidence that responses provided a true picture of care. This work demonstrates the power of audit to drive improvement, at a regional level. Disclosure  C.A. Sharp: None. J. Little: None. A. Shahbaz: None. E.F. Wood: None. S. Wig: None. P. Watson: None. S. Varughese: None. L. Teh: None. P. Shah: None. C. Saleh: None. L. Ottewell: None. L. Newton: None. S.R. Moore: None. E. McCarthy: Consultancies; E.M. has received consultancy fees from Chugai. E. MacPhie: None. K. Lazarewicz: None. J. Brockbank: None. J. Bluett: None. L. Mercer: None.


2002 ◽  
Vol 57 (4) ◽  
pp. M241-M245 ◽  
Author(s):  
H. Blain ◽  
I. Abdelmouttaleb ◽  
J. Belmin ◽  
A. Blain ◽  
J. Floquet ◽  
...  

2008 ◽  
Vol 32 (1) ◽  
pp. 3-6 ◽  
Author(s):  
Shahriar Nabili ◽  
Priya Bhatt ◽  
Fiana Roberts ◽  
Alastair Gracie ◽  
Robert McFadzean

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 95.3-95
Author(s):  
A. Sachdev ◽  
S. Dubey ◽  
C. Tiivas ◽  
M. George ◽  
P. Mehta

Background:A number of centres are now running fast track pathways for diagnosis and management of Giant cell arteritis with ultrasound as the first port of call for diagnosis1. Temporal artery biopsies (TABs) have become the second line of investigation, and it is unclear how useful TAB is in this setting.Objectives:This study looked at accuracy of Temporal artery biopsy (TAB) in patients with suspected Giant Cell arteritis (GCA) with negative/inconclusive ultrasound (U/S) and how duration of treatment on steroids prior to these investigations and arterial specimen size affected it.Methods:Prospective study of all patients with suspected GCA referred for TAB when U/S was negative or inconclusive, as part of the local fast-track pathway (Coventry). Database included clinical findings, serological work up, U/S and TAB results and treatment. Sensitivity and specificity of U/S and TAB was calculated and compared based on duration of treatment with steroids.Results:One hundred and nine patients were referred for TAB via Coventry fast-track-pathway. The sensitivity of U/S in this cohort of patients was 9.08% and specificity was 93.33%. After 3 days of steroid this was 0% and 100% respectively. For TAB when done within 10 days of starting steroids, this was 65% and 87.5% respectively. After 20 days of steroids this was 0 % and 100%. The sensitivity and specificity was 20% and 85% when arterial specimen size was 11-15mm and 47% and 100% when specimen size was 16 mm or more. Sensitivity and specificity of U/S of 644 suspected GCA patients was 48% and 98%.Conclusion:Our study demonstrates that TAB plays a relevant role in GCA fast-track-pathways, when U/S is negative/inconclusive. TAB was more sensitive than U/S in this cohort of patients, but overall sensitivity of U/S was higher when calculated for all patients suspected with GCA. Both remain useful tests if performed early. TAB specimen size should ideally be 16mm or more and done within 10 days of starting steroids.References:[1]Jonathan Pinnell, Carl Tiivas, Kaushik Chaudhuri, Purnima Mehta, Shirish Dubey, O38 The diagnostic performance of ultrasound Doppler in a fast-track pathway for giant cell arteritis,Rheumatology, Volume 58, Issue Supplement_3, April 2019, kez105.036,https://doi.org/10.1093/rheumatology/kez105.036Disclosure of Interests:None declared


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