scholarly journals Giant Cell Arteritis: Current Advances in Pathogenesis and Treatment

2020 ◽  
Author(s):  
Marília A. Dagostin ◽  
Rosa M.R. Pereira
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Visual Loss ◽  
Imaging Techniques ◽  
Limb Ischemia ◽  
Temporal Artery ◽  
Medical Emergency ◽  
Common Symptom ◽  
Irreversible Damage ◽  
Temporal Arteries

Giant cell arteritis (GCA) is the most common vasculitis in adults, with the incidence increasing with the advancing age. The aorta and its branches, especially the carotid extracranial branches, are the classic targets of inflammation in GCA. Visual loss, upper limb ischemia, and stroke are complications described. Suspicion of GCA is a medical emergency, and patients need to be quickly diagnosed/treated to prevent irreversible damage. Headache is the most common symptom, and a new-onset headache in older adults should always raise the suspicion of GCA. Patients may also present with scalp tenderness or tongue/jaw pain. GCA is often found to be the cause of an obscure-origin fever in older patients. A positive temporal artery biopsy is considered the gold standard for the diagnosis, but imaging techniques enable the assessment of cranial and extracranial arteries and the aorta. Ultrasound of temporal arteries is recommended and noncompressible “halo” sign is the typical finding. PET, MRI, or CT may be useful for the detection of the disease in the aorta and other vessels. The treatment must be started promptly with prednisone 1 mg/kg/day. When visual symptoms/unilateral visual loss is present, methylprednisolone pulse is recommended. Methotrexate, leflunomide and tocilizumab may be effective and well-tolerated glucocorticoid-sparing agents in GCA. Cardiovascular diseases are the leading causes of death in patients.

scholarly journals Imaging Tests in the Early Diagnosis of Giant Cell Arteritis

2021 ◽  
Vol 10 (16) ◽  
pp. 3704
Author(s):  
Diana Prieto-Peña ◽  
Santos Castañeda ◽  
Isabel Martínez-Rodríguez ◽  
Belén Atienza-Mateo ◽  
Ricardo Blanco ◽  
...  
Keyword(s):  
Early Diagnosis ◽  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Early Recognition ◽  
Imaging Modality ◽  
Imaging Techniques ◽  
Vascular Complications ◽  
Temporal Artery ◽  
Temporal Artery Biopsy ◽  
Temporal Arteries

Early recognition of giant cell arteritis (GCA) is crucial to avoid the development of ischemic vascular complications, such as blindness. The classic approach to making the diagnosis of GCA is based on a positive temporal artery biopsy, which is among the criteria proposed by the American College of Rheumatology (ACR) in 1990 to classify a patient as having GCA. However, imaging techniques, particularly ultrasound (US) of the temporal arteries, are increasingly being considered as an alternative for the diagnosis of GCA. Recent recommendations from the European League Against Rheumatism (EULAR) for the use of imaging techniques for large vessel vasculitis (LVV) included US as the first imaging option for the diagnosis of GCA. Furthermore, although the ACR classification criteria are useful in identifying patients with the classic cranial pattern of GCA, they are often inadequate in identifying GCA patients who have the extracranial phenotype of LVV. In this sense, the advent of other imaging techniques, such as magnetic resonance imaging (MRI), computed tomography (CT), and positron emission tomography (PET)/CT, has made it possible to detect the presence of extracranial involvement of the LVV in patients with GCA presenting as refractory rheumatic polymyalgia without cranial ischemic manifestations. Imaging techniques have been the key elements in redefining the diagnostic work-up of GCA. US is currently considered the main imaging modality to improve the early diagnosis of GCA.

scholarly journals POS0821 THE DIFFERENT SUBTYPES OF GIANT CELL ARTERITIS BY ULTRASOUND: RESULTS FROM A FAST-TRACK CLINIC

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 664.3-664
Author(s):  
I. Monjo ◽  
E. Fernández-Fernández ◽  
J. Ortega ◽  
E. De Miguel
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Fast Track ◽  
Carotid Arteries ◽  
Imaging Techniques ◽  
Vascular Involvement ◽  
Temporal Arteries ◽  
Halo Sign ◽  
Cranial Vessels ◽  
Large Vessels

Background:Giant cell arteritis (GCA) is a vasculitis that affects the medium and large vessels (LV). Although cranial artery involvement is better known, awareness of the importance of LV involvement is increasing. Imaging techniques currently constitute the basis for the diagnosis of LV-GCA and have improved its diagnosis and prevalence. In recent years, differences in clinical patterns and different inflammatory and etiopathogenic mechanisms of the disease have been suggested. Therefore, improving sensitivity to diagnosis is essential to improve the knowledge and care of our population.Objectives:The aim of this study was to know the prevalence of the different ultrasound patterns of GCA in our area.Methods:Retrospective records of available data were collected from all patients referred to our ACG fast track clinic in the past three years. The clinical and laboratory characteristics were evaluated at the time of referral. All patients underwent an ultrasound scan of cranial vessels (superficial temporal arteries (TA) and their frontal and parietal branches) and large vessels (axillary, subclavian and carotid arteries). The doctor confirmed the GCA diagnosis after at least six months of follow-up. The OMERACT definitions of halo sign with a hypoechoic wall thickness ≥ 0.34 mm were used for TA pathology for the ultrasound diagnosis of GCA and for axillary, subclavian and carotid arteries and homogeneous hypoechoic thicknesses ≥ 1 mm of the arterial wall were applied. Atherosclerosis lesions were evaluated to detect this disease as a possible false positive halo sign. An Esaote Mylab Twice with a 13 MHz probe in BT and 22 MHz for cranial vessels in 2017-2019 and an Esaote Mylab X8plus with a 15 MHz probe for BT and a 24 MHz probe for cranial arteries in 2019-2020 were used by two rheumatologist with long experience in ACG ultrasound.Results:A total of 261 patients (180 women / 81 men) with suspected GCA were evaluated in our fast track clinic. The mean age (± SD) was 76 ± 9.2 years and CRP at diagnosis was 75.7 ± 68.6 mg/L. The time elapsed since the first symptoms was less than 6, 6-12, 12-24 or >24 weeks in 37.5%, 19.9%, 12.3% and 15.7% respectively. Of the 261 cases explored, 160 had GCA, of which 102 were women and 58 men, and had a mean age of 77.21 ± 7.9 years. The ultrasound patterns of GCA were: 71 patients had exclusive involvement of the TA (cranial-GCA), 54 had a mixed pattern with involvement of both TA and LV (mixed-CGA), and 35 had isolated involvement of the LV (LV-GCA). That is, 125 patients had cranial involvement with or without LV involvement and 89 had LV-GCA associated or not with cranial involvement (Figure 1).Figure 1.Ultrasound patters of GCAConclusion:Ultrasound is a useful tool for the screening of GCA and its different subtypes of vascular involvement. The isolated cranial subtype or associated with LV-GCA is the most common (78% of cases), but LV-GCA is also very common (55.6% of cases of GCA) and 21.9% presents as an isolated LV-GCA standard. The LV arteries should be included in the ultrasound examination for suspected GCA.Disclosure of Interests:Irene Monjo Speakers bureau: Roche, Novartis, UCB, Gedeon Richter, Consultant of: Roche, Elisa Fernández-Fernández: None declared, Javier Ortega: None declared, Eugenio de Miguel Speakers bureau: AbbVie, Novartis, Pfizer, MSD, BMS, UCB, Roche, Grunental, Janssen, Sanofi, Paid instructor for: Janssen, Novartis, Roche, Consultant of: AbbVie, Novartis, Pfizer, Galapagos, Grant/research support from: Abbvie, Novartis, Pfizer

The sensitivity of temporal artery biopsy in the detection of giant cell arteritis is independent of tissue length

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S28-S29
Author(s):  
H J Hurley ◽  
P Q Deb
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Average Length ◽  
Statistical Significance ◽  
Temporal Artery ◽  
Temporal Artery Biopsy ◽  
Temporal Arteries ◽  
Biopsy Specimens ◽  
Two Samples ◽  
Left And Right

Abstract Introduction/Objective Giant cell arteritis (GCA) is the most common vasculitis of the elderly, and the most common primary systemic vasculitis overall, with an annual incidence of 200/million. The long term sequelae, namely vision loss and stroke, are permanent and devastating. While GCA is often treated empirically based on clinical presentation, panarteritis on temporal artery biopsy is required for diagnosis. However, these biopsies have the tendency to be falsely negative due to skip lesions, a common feature of GCA. Therefore, we set out to determine whether longer biopsy specimens were more sensitive in the detection of GCA. Methods/Case Report A census of temporal artery biopsies performed with the indication of clinical symptoms of GCA was taken at our institution. The patient age, sex, biopsy laterality, biopsy length, and pathological diagnosis were recorded for each cataloged sample. Statistical significance of difference in biopsy length was tested using an unpaired t-test in R 4.1.0. Results (if a Case Study enter NA) A total of 114 temporal artery specimens were biopsied from 94 different patients with the indication of GCA and assigned a definitive positive or negative diagnosis. Of the 94 patients, 54 were female and 40 were male. Of the total pathological specimens, 11 were positive and 103 were negative. The overall average length of biopsy specimens was 2.13 cm with a standard deviation of 0.65 cm. The average positive biopsy was 2.26 cm long, and the average negative was 2.12 cm, an insignificant difference (0.14 cm, t = 0.7, p = 0.43). In 25 patients, biopsies were taken from both the left and right temporal arteries. Of those patients, 2 were positive for GCA and the remaining 23 were negative. Interestingly, the biopsy result in every case was identical between the left and right samples; we found no instances of pathological evidence of GCA in only one of the two samples from the same patient. Conclusion According to data taken at our institution, there is no indication to lengthen the biopsy requirements from the existing 1.5 cm. However, we have demonstrated evidence that it may be unnecessary to biopsy both temporal arteries in a single patient. Larger studies would be required to confirm our findings.

scholarly journals Blocking interferon γ reduces expression of chemokines CXCL9, CXCL10 and CXCL11 and decreases macrophage infiltration in ex vivo cultured arteries from patients with giant cell arteritis

2015 ◽  
Vol 75 (6) ◽  
pp. 1177-1186 ◽  
Author(s):  
Marc Corbera-Bellalta ◽  
Ester Planas-Rigol ◽  
Ester Lozano ◽  
Nekane Terrades-García ◽  
Marco A Alba ◽  
...  
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Mononuclear Cells ◽  
Ex Vivo ◽  
Selective Reduction ◽  
Temporal Artery ◽  
Interferon Γ ◽  
Peripheral Blood Mononuclear ◽  
Temporal Arteries ◽  
Infiltrating Cells

BackgroundInterferon γ (IFNγ) is considered a seminal cytokine in the pathogenesis of giant cell arteritis (GCA), but its functional role has not been investigated. We explored changes in infiltrating cells and biomarkers elicited by blocking IFNγ with a neutralising monoclonal antibody, A6, in temporal arteries from patients with GCA.MethodsTemporal arteries from 34 patients with GCA (positive histology) and 21 controls were cultured on 3D matrix (Matrigel) and exposed to A6 or recombinant IFNγ. Changes in gene/protein expression were measured by qRT-PCR/western blot or immunoassay. Changes in infiltrating cells were assessed by immunohistochemistry/immunofluorescence. Chemotaxis/adhesion assays were performed with temporal artery-derived vascular smooth muscle cells (VSMCs) and peripheral blood mononuclear cells (PBMCs).ResultsBlocking endogenous IFNγ with A6 abrogated STAT-1 phosphorylation in cultured GCA arteries. Furthermore, selective reduction in CXCL9, CXCL10 and CXCL11 chemokine expression was observed along with reduction in infiltrating CD68 macrophages. Adding IFNγ elicited consistent opposite effects. IFNγ induced CXCL9, CXCL10, CXCL11, CCL2 and intracellular adhesion molecule-1 expression by cultured VSMC, resulting in increased PBMC chemotaxis/adhesion. Spontaneous expression of chemokines was higher in VSMC isolated from GCA-involved arteries than in those obtained from controls. Incubation of IFNγ-treated control arteries with PBMC resulted in adhesion/infiltration by CD68 macrophages, which did not occur in untreated arteries.ConclusionsOur ex vivo system suggests that IFNγ may play an important role in the recruitment of macrophages in GCA by inducing production of specific chemokines and adhesion molecules. Vascular wall components (ie, VSMC) are mediators of these functions and may facilitate progression of inflammatory infiltrates through the vessel wall.

Increased expression of the endothelin system in arterial lesions from patients with giant-cell arteritis: association between elevated plasma endothelin levels and the development of ischaemic events

2009 ◽  
Vol 69 (2) ◽  
pp. 434-442 ◽  
Author(s):  
E Lozano ◽  
M Segarra ◽  
M Corbera-Bellalta ◽  
A García-Martínez ◽  
G Espígol-Frigolé ◽  
...  
Keyword(s):  
Western Blot ◽  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Protein Level ◽  
Messenger Rna ◽  
Tissue Concentration ◽  
Temporal Artery ◽  
Glucocorticoid Treatment ◽  
Temporal Arteries ◽  
Endothelin System

Objective:Approximately 15–20% of patients with giant-cell arteritis (GCA) develop ischaemic complications often preceded by transient ischaemia. The expression of the endothelin (ET) system in GCA lesions was investigated to assess its relationship with the development of ischaemic complications.Methods:Plasma ET-1 was quantified by immunoassay in 61 patients with biopsy-confirmed GCA and 16 healthy donors. ET-1, endothelin-converting enzyme (ECE-1) and endothelin receptor (ETAR and ETBR) messenger RNA were measured by real-time quantitative reverse transcriptase–PCR in temporal arteries from 35 of these patients and 19 control arteries. Proteins were measured by immunoassay and Western blot.Results:ET-1 concentration was increased at the protein level in temporal artery samples from GCA patients compared with controls (0.98 (SEM 0.32) vs 0.28 (SEM 0.098) fmol/mg, p = 0.028). ECE-1, ETAR and ETBR/actin ratios (Western blot) were also significantly higher in GCA patients. Intriguingly, mRNA expression of ET-1, ECE-1 and both receptors was significantly reduced in GCA lesions compared with control arteries. When investigating mechanisms underlying these results, platelet-derived growth factor and IL-1β, present in GCA lesions, were found to downregulate ET-1 mRNA in cultured human temporal artery-derived smooth muscle cells. Glucocorticoid treatment for 8 days did not result in significantly decreased endothelin tissue concentration (0.87 (SEM 0.2) vs 0.52 (SEM 0.08); p = 0.6). Plasma endothelin concentrations were higher in patients with ischaemic complications (1.049 (SEM 0.48) vs 1.205 (SEM 0.63) pg/ml, p = 0.032).Conclusions:The endothelin system is increased at the protein level in GCA lesions creating a microenvironment prone to the development of ischaemic complications. Recovery induced by glucocorticoids is delayed, indicating persistent exposure to endothelin during initial treatment.

Giant cell arteritis: advances in diagnosis and management

2019 ◽  
Vol 80 (8) ◽  
pp. 448-455
Author(s):  
Mehdi Raza ◽  
Yasser El Maideny ◽  
Nadia Bokhari
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Visual Loss ◽  
Temporal Artery ◽  
Temporal Artery Biopsy ◽  
Biological Therapies ◽  
Diagnosis And Management ◽  
American College Of Rheumatology ◽  
The World ◽  
Cranial Vessels

Giant cell arteritis has been widely studied throughout the world. Involvement of cranial vessels can lead to visual loss and strokes. This review primarily focusses on the presentation, diagnosis and treatment. The last 10 years have brought dramatic improvements in the imaging and medical therapies for this condition. After the American College of Rheumatology suggested criteria for the diagnosis of giant cell arteritis, many studies have been performed to find alternatives to a temporal artery biopsy. There is growing evidence that a biopsy may not be needed when one can make a convincing clinical and radiological diagnosis. Although glucocorticoids are the mainstay of treatment and their role has not changed, various biological and non-biological therapies are being used to reduce relapses and prolong remission of symptoms.

Polymyalgica rheumatica and giant cell arteritis

2008 ◽  
Vol 18 (2) ◽  
pp. 91-101 ◽  
Author(s):  
H A Bird ◽  
Helen Mac Iver
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Polymyalgia Rheumatica ◽  
Visual Loss ◽  
Temporal Arteritis ◽  
Morning Stiffness ◽  
Temporal Artery ◽  
Shoulder Girdle ◽  
Pelvic Girdle ◽  
Disease Spectrum

Polymyalgia rheumatica and giant cell arteritis are closely related conditions, considered by many to represent opposite poles of a single disease spectrum. They can occur together or separately.Polymyalgia rheumatica is characterized by pain and morning stiffness in the shoulder girdle and sometimes the pelvic girdle. The symptoms are felt to be related to synovitis of proximal joints and extra-articular synovial structures. Giant cell arteritis displays a frank vasculitis affecting the regions supplied by the temporal artery to give visual loss and scalp tenderness but is increasingly recognized to also affect the aorta and its extra-cranial branches. For this reason the term ‘giant cell arteritis’, which is descriptive of the pathology, is used instead of the alternative term ‘temporal arteritis’, which gives a misleading impression of localization but which was the term used in previous reviews for this journal, the most recent in 2003.

scholarly journals AB0371 PATIENTS WITH PROLONGED SYMPTOMS BEFORE GCA DIAGNOSIS DO NOT INCUR HIGHER RATES OF VISUAL LOSS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1212.1-1212
Author(s):  
V. Yang ◽  
C. Mcmaster ◽  
C. Owen ◽  
J. Leung ◽  
R. Buchanan ◽  
...  
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Symptom Onset ◽  
Visual Loss ◽  
Temporal Artery ◽  
Classification Criteria ◽  
Delayed Presentation ◽  
American College Of Rheumatology ◽  
Before And After ◽  
History Of

Background:Giant cell arteritis (GCA), if left untreated, confers the threat of serious cranial ischaemic complications including permanent visual loss. Although achieving a prompt and accurate diagnosis remains challenging, early diagnosis is viewed as being paramount in preventing significant morbidity.1 This raises the question of whether GCA patients are at greater risk of developing visual sequelae if there is a longer window between symptom onset and presentation.Objectives:To compare the frequency of lasting visual loss in patients diagnosed with GCA undergoing temporal artery biopsy (TAB) within three months and after three months of symptom onset.Methods:Patients who underwent TAB from January 2011 to November 2020 were identified from the pathology database of an Australian rheumatology referral centre. The diagnosis of GCA was established for each patient based on either positive TAB or, in the setting of negative TAB, clinical diagnosis by a rheumatologist. Baseline demographics, symptoms and major confounders – including age, sex, history of polymyalgia rheumatica or inflammatory arthritis, headache, jaw pain, fatigue, temporal artery tenderness or diminished pulse, and number of 1990 American College of Rheumatology (ACR) classification criteria for GCA2 fulfilled – were manually extracted from electronic medical records, as was the duration between onset of GCA symptoms and TAB, and the presence of visual loss before and after TAB. Logistic regression log-likelihood tests were used to examine the two cohorts presenting before and after three months.Results:There were 167 patients who underwent TAB during the study period with accessible clinical information. Of these, 31 (19%) had a delayed presentation of greater than three months from symptom onset. There were no statistical differences in patient demographics between the two groups (Table 1). No patients with delayed presentation experienced lasting, objective visual loss. In contrast, there were three cases in the cohort of patients who presented more promptly; these included two patients who developed permanent unilateral blindness, and one who experienced unilateral vision loss with some improvement at three months of follow-up.Table 1.Patient characteristics by time from symptom onset to TAB.Presentation <3 monthsPresentation ≥3 monthsp-valueAge (years)73.45±10.0669.84±10.750.080Female92 (67.65%)20 (64.52%)0.738History of polymyalgia rheumatica23 (16.91%)4 (12.90%)0.586History of inflammatory arthritis6 (4.41%)2 (6.45%)0.633Headache110 (80.88%)23 (74.19%)0.406Jaw pain37 (27.21%)5 (16.13%)0.206Fatigue28 (20.59%)6 (19.35%)0.878Temporal artery tenderness or diminished pulse46 (33.82%)11 (35.48%)0.860ACR classification criteria2.83±0.992.58±0.890.199Conclusion:GCA patients with a lengthier course of symptoms before diagnosis did not experience any enduring visual loss. This may reflect a pattern of more aggressive disease leading to earlier presentation, but further study should explore whether longer symptom duration before diagnosis necessitates a higher degree of clinical concern.References:[1]Font C, Cid MC, Coll-Vinent B, López-Soto A, Grau JM. Clinical features in patients with permanent visual loss due to biopsy-proven giant cell arteritis. Br J Rheumatol. 1997 Feb;36(2):251-4. doi: 10.1093/rheumatology/36.2.251. PMID: 9133940.[2]Hunder GG, Bloch DA, Michel BA, Stevens MB, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum. 1990 Aug;33(8):1122-8. doi: 10.1002/art.1780330810. PMID: 2202311.Disclosure of Interests:None declared

Introducing an ANP-led temporal artery biopsy service for patients with suspected giant cell arteritis

2021 ◽  
Vol 30 (9) ◽  
pp. 512-519
Author(s):  
John Cooper
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Nurse Practitioner ◽  
Temporal Arteritis ◽  
Temporal Artery ◽  
Medical Emergency ◽  
Temporal Artery Biopsy ◽  
Sight Loss

Giant cell arteritis (GCA) is an uncommon autoimmune inflammatory vasculopathy that can lead to the destruction and occlusion of various arteries that consequently can cause serious complications such as stroke or sight loss. It is seen as a medical emergency. The most commonly affected vessel in GCA is the temporal artery in the side of the head, hence the condition is sometimes also referred to as ‘temporal arteritis’. This article discusses the introduction of an advanced nurse practitioner-led temporal artery biopsy service.

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