Large-vessel Giant Cell Arteritis Research Articles

Case Report: Giant Cell Arteritis presenting with 6th Nerve Palsy without Ischemic Optic Neuropathy

Purpose: Giant cell arteritis (GCA) can be a difficult condition to identify in the early stages especially in the absence of the pathognomonic arteritic anterior ischemic optic neuropathy. Optometrists serve an important role in correctly triaging and initiating appropriate work-up and treatment for this emergent condition. This case report and review serves as a refresher of the systemic and ocular signs and symptoms of GCA. Background: GCA is a systemic autoimmune condition characterized by granulomatous inflammation of medium and large arteries in patients over 50. The most well-known constellation of signs and symptoms include new onset headache, jaw claudication, scalp tenderness, temporal artery abnormality with painless vision loss secondary to arteritic anterior ischemic optic neuropathy. However, it is important to be aware of alternate presenting signs and symptoms including pain anywhere in the distribution of the external carotid (occipital, neck, tongue, throat, ear) and signs of large vessel GCA (arm/limb claudication, chest/back pain, Raynaud’s phenomenon) and Polymyalgia Rheumatica (PMR) (bilateral hip/shoulder pain and morning stiffness). Case Report: An 86-year-old Caucasian female presents for a referred exam regarding new onset diplopia with concurrent occipital headache, neck pain and sore throat originally dismissed as symptoms of her other systemic health conditions. ESR, CRP and platelets were elevated on serology and subsequent temporal artery biopsy was positive for GCA. Oral steroids were initiated, and she was lost to follow up after her ocular symptoms resolved. Conclusion: GCA can present with a large range of manifestations, and many are nonspecific and easily attributable to other causes especially when it deviates from the classic constellation of new onset headache, jaw claudication, scalp tenderness and temporal artery abnormality with painless vision loss. Optometrists as primary eye care providers may be the first point of contact and need to be cognizant of the broader set of manifestations to minimize delays in diagnosis and treatment of this life and vision threatening condition. CE Notification: This article is available as a COPE accredited CE course. You may take this course for 1-hour credit. Read the article and take the qualifying test to earn your credit. Click here to Enroll (https://www.crojournal.com/case-report-giant-cell-arteritis-presenting-with-6th-nerve-palsy-without-ischemic-optic-neuropathy) Please check COPE course expiry date prior to enrollment. The COPE course test must be taken before the course expiry date.

P295 Establishing adenosine deaminase 2 activity in a cohort of patients with large vessel vasculitis

Abstract Background/Aims Deficiency of adenosine deaminase 2 (DADA2) leads to a broad phenotype; some patients develope vasculitis and strokes, and others have predominantly haematological manifestations. Small, medium and large vessel vasculitis has been described. In contrast, plasma ADA2 activity is elevated in inflammatory conditions including systemic lupus erythematosus and systemic juvenile idiopathic arthritis with macrophage activation syndrome. The role of ADA2 and how its deficiency associates with the vasculitides is not well understood. Using a well-characterised large vessel vasculitis cohort, we sought to establish the potential role of ADA2 activity. Methods ADA2 activity was measured in adults with Takayasu arteritis (TA), large vessel giant cell arteritis (LV-GCA), intracranial vasculitis (IV) and healthy control (HC) subjects using a coupled spectrophotometric assay. Plasma from 11 patients with genotyped DADA2 and 10 patients heterozygous for pathogenic variants in ADA2 were used to define thresholds for deficiency and carrier status respectively (DADA2 activity 0 - 0.90 U/L, ADA2 carrier activity 2.02 - 10.62 U/L). Activity levels above the upper limit of normal (ULN) were defined as > 99th percentile of the HC activity (30.3U/L). To assess potential associations with TA disease status, ADA2 was measured prospectively in matched active and remission samples. TA disease activity was determined as active, grumbling or stable using clinical and radiological features, physician impression, CRP, ESR and Indian Takayasu clinical Activity Score (ITAS). Results No patients had deficient ADA2 activity while 7 subjects had activity within the carrier range (1 HC [1.9%], 5 TA [3.7%], 1 LV-GCA [2.3%]). Compared to HC levels (17.43 [14.11 - 20.78] U/L), activity in the TA group was significantly increased (TA 19.68U/L, p = 0.0061), while LV-GCA and IV were not significantly different. In 20 cases, activity exceeded the ULN; 16 TA (11.9%, p = 0.044; 4 active, 2 grumbling, 10 stable) and 4 LV-GCA (9.1%, ns). In TA, there was a positive correlation between ADA2 activity and ESR (Spearman R = 0.2863, p = 0.0009) but not with CRP or ITAS score. Subgroup analysis revealed ADA2 activity was most prominently elevated in the stable group relative to HCs (TA stable 20.54 U/L [8.08 - 48.66], median +17.8%, p = 0.0006), but not with active or grumbling disease. Consistent with this, prospective analysis demonstrated a significant increase in ADA2 activity in remission vs active disease (p = 0.024), with activity increasing in 18 (62%) patients achieving remission. Conclusion There were no cases of ADA2 deficiency in this cohort. Consistent with other inflammatory conditions, ADA2 activity was elevated in TA compared to HC subjects. Surprisingly, increased ADA2 activity was linked to stable TA in both cross-sectional and prospective analyses. This may suggest a potential role of ADA2 enzyme activity in vascular repair and regrowth - possibly tying in with known elevated levels of ADA2 activity in growing children. Disclosure A. Porter: None. R. Maughan: None. C. Pericleous: None. A. Kiprianos: None. H. Jee: None. P. Lee: None. T. Youngstein: None. J. Mason: None.

Clinical profile and outcome of large-vessel giant cell arteritis in Japanese patients: A single-centre retrospective cohort study.

Recent advances in imaging revealed that giant cell arteritis (GCA) is frequently associated with large vessel involvement (LVI), but they may also contribute to earlier diagnosis and treatment of LV-GCA. We aimed to compare the clinical characteristics of GCA with or without LVI and evaluate its association with clinical outcomes. We retrospectively reviewed the medical records of 36 patients with GCA in Kyoto University Hospital. Eighteen patients each were assigned to the LVI(+) and LVI(-) groups. Five-year survival rates in the LVI(+) group were better than in the LVI(-) group (p = .034), while five-year relapse-free survival rates were similar between the groups (p = .75). The LVI(+) group required lower doses of glucocorticoid at month 6 (p = .036). Disease activity evaluated with the Birmingham Vasculitis Activity Score at disease onset was higher in the LVI(-) group (p = .014), and the Vasculitis Damage Index score examined at the last visit was higher in the LVI(-) group (p = .011). GCA without LVI had more active disease, severer vascular damage, and worse survival, possibly because of ophthalmic complications and their greater glucocorticoid requirement. Our results revisit the impact of cranial manifestations on disease severity and morbidity.

Mimickers of Large Vessel Giant Cell Arteritis.

Giant cell arteritis (GCA) is a large-vessel granulomatous vasculitis occurring in patients over 50-year-old. Diagnosis can be challenging because there is no specific biological test or other diagnoses to consider. Two main phenotypes of GCA are distinguished and can be associated. First, cranial GCA, whose diagnosis is usually confirmed by the evidence of a non-necrotizing granulomatous panarteritis on temporal artery biopsy. Second, large-vessel GCA, whose related symptoms are less specific (fever, asthenia, and weight loss) and for which other diagnoses must be implemented if there is neither cephalic GCA nor associated polymyalgia rheumatica (PMR) features chronic infection (tuberculosis, Coxiella burnetti), IgG4-related disease, Erdheim Chester disease, and other primary vasculitis (Behçet disease, relapsing polychondritis, or VEXAS syndrome). Herein, we propose a review of the main differential diagnoses to be considered regarding large vessel vasculitis.

Diagnostic accuracy of vascular ultrasound in patients with suspected giant cell arteritis (EUREKA): a prospective, multicentre, non-interventional, cohort study.

Temporal artery biopsy is considered the diagnostic gold standard for giant cell arteritis, despite approximately 39% of patients who are negative for the condition by biopsy subsequently being given a clinical diagnosis of giant cell arteritis. We aimed to assess the diagnostic accuracy of ultrasound examination in patients with suspected giant cell arteritis. In this prospective, multicentre, non-interventional, cohort study (evaluation of ultrasound's role in patients suspected of having extracranial and cranial giant cell arteritis; EUREKA), we consecutively recruited patients aged 50 years or older, with clinically suspected giant cell arteritis from three Danish hospitals (South West Jutland Hospital in Esbjerg, Silkeborg Regional Hospital, and Rigshospitalet, Glostrup). Participants had a bilateral ultrasound of the temporal, facial, common carotid, and axillary arteries. Ultrasounds were done by ultrasonographers who were systematically trained in vascular ultrasound using appropriate equipment and settings. Participants then had a temporal artery biopsy within 7 days of initiation of corticosteroid treatment. A blinded ultrasound expert assessed all ultrasound images. Ultrasound vasculitis was defined in cranial arteries as a homogeneous, hypoechoic, intimamedia complex thickness and a positive compression sign and as a homogeneous intimamedia complex of 1 mm in thickness or wider in the axillary arteries and of 1·5 mm thickness or wider in the common carotid artery. Participants were followed up at 6 months. During this 6 month period, clinicians were able to collect data from all clinical examinations to enable a full clinical diagnosis at 6 months. Clinical diagnosis was based on the expert opinion of the treating rheumatologist. The diagnostic criterion standard was diagnosis confirmed after 6 months of follow-up. We used logistic regression analyses to calculate the odds ratio and 95% CI of ultrasound as a predictor for giant cell arteritis. Between April 1, 2014, and July 31, 2017, 118 patients were screened for inclusion, of whom 106 had both ultrasound examinations and an eligible temporal artery biopsy and were included in the intention-to-diagnose population. The mean age was 72·7 years (SD 7·9), 63 (59%) participants were women, and 43 (41%) were men. Temporal artery biopsy was positive in 46 (43%) of 106 patients, and 62 (58%) of 106 patients had a clinically confirmed diagnosis of giant cell arteritis at 6 months (temporal artery biopsy sensitivity 74% [95% CI 62-84], specificity 100% [95% CI 92-100]). Cranial artery ultrasound was positive in all patients who had a positive temporal artery biopsy, and seven (58%) of 12 patients who were positive by ultrasound and negative by temporal artery biopsy were confirmed to have large-vessel giant cell arteritis via other imaging methods. The sensitivity of ultrasound diagnosis of giant cell arteritis was 94% (84-98) and specificity was 84% (70-93). Logistic regression analysis confirmed that ultrasound was the strongest baseline predictor for a clinically confirmed diagnosis of giant cell arteritis at 6 months (crude odds ratio 76·6 [95% CI 21·0-280·0]; adjusted for sex and age 141·0 [27·0-743·0]). Vascular ultrasound might effectively replace temporal artery biopsy as a first-line diagnostic method in patients suspected of having giant cell arteritis when done by systematically trained ultrasonographers using appropriate equipment and settings. The Institute for Regional Research at Hospital of Southwest Jutland, Esbjerg, Denmark.

A systematic review of clinical and preclinical evidences for Janus kinase inhibitors in large vessel vasculitis.

Corticosteroid-sparing disease-modifying anti-rheumatic drugs are an area of active exploration in large vessel vasculitis (LVV), i.e., Takayasu arteritis (TAK) and Giant Cell Arteritis (GCA). The role of Janus kinase (JAK) inhibitors has been recently identified in different inflammatory rheumatic diseases. We conducted a systematic review of the use of JAK inhibitors in LVV across MEDLINE, Scopus, Web of Science, EMBASE, PubMed Central, Cochrane database of controlled trials, clinicaltrials.gov, and major recent international conferences. We identified four cohort studies and ten case reports. The JAK inhibitors used in these studies were tofacitinib, baricitinib, and ruxolitinib. A cohort study in TAK compared 27 patients treated with tofacitinib with 26 others treated with methotrexate, with better clinical outcomes with tofacitinib but similar angiographic stabilization, relapses, corticosteroid-sparing effect, and adverse events in both groups. Most of the other studies favored clinical responses with JAK inhibitors in LVV but with a paucity of data on other outcomes. Most of the included studies were of moderate quality. Evidence from pre-clinical models of LVV as well as limited in vivo data in patients with TAK appears to suggest that JAK inhibition reduces adventitial fibrosis, intimal proliferation, and inflammatory T lymphocyte infiltration in the media as well as reduces resident memory T cells in the vascular wall (which are otherwise resistant to corticosteroids). Ongoing clinical trials of tofacitinib, baricitinib, and upadacitinib in LVV shall help to further clarify the potential promise of JAK inhibitors for LVV (PROSPERO registration number CRD42021273359). KEY POINTS : •Tofacitinib appeared to associate with better clinical outcomes than methotrexate in TAK. •JAKinibs reduce adventitial fibrosis, intimal proliferation, and inflammatory vascular infiltrate in pre-clinical models of LVV. •Tofacitinib downregulates resident memory vascular T lymphocytes in pre-clinical models of LVV.

Vascular Ultrasound for Giant Cell Arteritis: Establishing a Protocol Using Vascular Sonographers in a Fast-Track Clinic in the United States.

ObjectiveWe developed a fast‐track clinic (FTC) to expedite the evaluation of patients suspected of having giant cell arteritis (GCA) using vascular ultrasound. Though FTCs have demonstrated efficacy in Europe, no protocolized clinic in the United States has been developed. This study introduces a new FTC model unique to the United States, using vascular sonographers, and describes the protocols used to develop reliable findings. We evaluate clinical outcomes using vascular ultrasound and temporal artery biopsy (TAB).MethodsA retrospective review included all subjects referred to the University of Washington FTC aged 50 years old or older who received both ultrasound and TAB between November 2017 and November 2019. Ultrasound was performed by a vascular sonographer trained in GCA detection. Ultrasound results were read by a vascular surgeon and reviewed by four rheumatologists certified in musculoskeletal ultrasound who had completed a course in vascular ultrasound use in GCA and large‐vessel vasculitis.ResultsA total of 43 subjects underwent both vascular ultrasound and TAB. Six subjects had both positive ultrasound and TAB results. There were also seven positive ultrasound results in patients with negative TAB results, most due to detection of large‐vessel GCA (LV‐GCA). All 29 subjects with negative ultrasound results had negative TAB results.ConclusionThis is the first study in the United States to demonstrate a reliable FTC protocol using vascular sonographers. This protocol demonstrated good agreement between ultrasound and TAB and allowed for the detection of additional cases of LV‐GCA by vascular ultrasound. Vascular ultrasound improved the rate of GCA diagnosis primarily by detecting additional cases of LV‐GCA.

Idiopathic Large-Vessel Vasculitis Presenting as Acute Abdomen and Mesenteric Ischemia

Objective: The term “large-vessel vasculitis” encompasses the spectrum of primary vasculitis that causes chronic granulomatous inflammation predominantly of the aorta and its major branches. The two major categories of large-vessel (LV) vasculitis are giant cell arteritis and Takayasu arteritis. LV vasculitis of unknown etiology presenting as mesenteric ischemia is presented here. Materials and Methods: This is a single-center retrospective analysis of 17 patients who presented with mesenteric ischemia over a period of 7 years. Their age ranged from 18 to 69 years. There were five females and the rest were male. The presenting symptoms were abdominal pain, nausea, malaise, and low-grade fever. Peripheral pulses were normal. Computed tomography (CT) aortogram showed typical wall edema and inflammatory tissue encasing the superior mesenteric artery, celiac artery, and the adjacent aorta in all the patients. Thrombosis and localized dissection in the superior mesenteric and celiac arteries was seen in some. Erythrocyte sedimentation rate and C-reactive protein were elevated, but other vasculitis markers were normal. Results: All of them were treated with glucocorticoids with immediate relief from the symptoms. Anticoagulation was given for those with thrombus, stenosis, and dissection. None had any intervention. At 1-year follow-up, there was no recurrence, and all were asymptomatic. Conclusion: Idiopathic LV vasculitis can present as mesenteric ischemia and acute abdomen. CT aortogram typically shows inflammatory tissue around the visceral artery and the aorta. The etiology is still uncertain. However, this condition has many similarities with LV giant cell arteritis (GCA-LV). Hence, all investigations should be done to rule out GCA. They respond very well to glucocorticoids. Correct diagnosis can avoid an unnecessary surgery. Long-term follow-up is required in order not to miss any late complications This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

HLA-B52 allele in giant cell arteritis may indicate diffuse large-vessel vasculitis formation: a retrospective study

BackgroundThis study aimed to identify new characteristics of elderly onset large-vessel vasculitis (EOLVV) by focusing on human leucocyte antigen (HLA) genotype, polymyalgia rheumatica (PMR), and affected vascular lesions observed on positron emission tomography/computed tomography (PET/CT) imaging.MethodsWe retrospectively studied 65 consecutive Japanese patients with large-vessel vasculitis (LVV) who had extracranial vasculitis lesions and underwent PET/CT imaging. PET/CT images were assessed using the semi-quantitative PET visual score of each affected vessel, and the PET vascular activity score (PETVAS) and number of affected vessels were calculated. Subjects were subsequently grouped based on age at onset, superficial temporal artery (STA) involvement, and presence of PMR and compared each group according to HLA genotype. Unsupervised hierarchical cluster analysis was used to identify the patients with similar characteristics in terms of affected vascular lesions detected through PET/CT imaging. The clinical characteristics and PET/CT findings of the population newly identified in this study were examined.ResultsTwenty-seven patients with EOLVV did not meet the American College of Rheumatology 1990 criteria for giant cell arteritis (GCA) and Takayasu arteritis and were considered as unclassified EOLVV (UEOLVV). The unsupervised hierarchical cluster analysis revealed that UEOLVV with PMR and large-vessel GCA (LV-GCA) formed a cluster of LVV with GCA features (i.e., PMR and/or STA involvement) when restricted to patients who were HLA-B52-positive. Patients who were HLA-B52-positive with LVV and GCA features had similar clinical characteristics and patterns of affected vessels and presented with diffuse LVV lesions. HLA-B52-positive patients who had LVV with GCA features also presented with higher PETVAS, more affected vessels, and lower rates of biologics usage and relapse compared to HLA-B52-positive patients with TAK.ConclusionsPatients who had UEOLVV with PMR had similar characteristics to patients with LV-GCA. Patients who were HLA-B52-positive and had LVV with GCA features presented with diffuse vascular lesions and may comprise a core population of Japanese patients with EOLVV. The findings of HLA-B52 positivity and diffusely affected vessels in patients with EOLVV can be considered as suspicious findings of LV-GCA.

Presenting features and outcomes of cranial-limited and large-vessel giant cell arteritis: a retrospective cohort study

Objectives: To compare the presenting features and outcomes of patients with cranial-limited (C-) and large-vessel (LV-) giant cell arteritis (GCA). Methods: Data from our GCA cohort were collected retrospectively. Patients who underwent total-body large-vessel imaging within 10 days after commencing steroid therapy were included. Patients with LV involvement were classified as LV-GCA. Presenting features, treatments, and outcomes of LV-GCA and C-GCA patients were compared. Results: 161 patients were included (LV-GCA, n = 100). At baseline, LV-GCA patients were younger than those with C-GCA (73.2 ± 8.9 vs 76 ± 8.8 years, p = 0.018) and had a longer delay to diagnosis (3.5 ± 4.6 vs 2.3 ± 4.9 months, p = 0.001). C-GCA patients had a higher incidence of headache (p = 0.006) and ischaemic optic neuropathy (p < 0.001), whereas LV-GCA patients had more systemic symptoms (fever, p = 0.002; fatigue, p < 0.001; weight loss, p < 0.001; night sweats, p = 0.015) and dry cough (p = 0.031). Corrected cumulative prednisone dose, relapse-free survival, relapse-rate, and incidence of ascending aortic aneurysms were not significantly different between the two subgroups. A steroid-sparing agent was added in 73% of LV- and 55.7% of C-GCA patients (p = 0.027), but was introduced more frequently at baseline in LV-GCA patients (52% vs 23.5%, p = 0.006). LV-GCA patients initially treated with glucocorticoid monotherapy relapsed sooner (relapse-free survival, HR = 0.56, 95% CI 0.41–0.78, p < 0.001) and had a higher relapse rate (relapses per 10 person-years, 6.73 ± 11.50 vs 3.82 ± 10.83, p = 0.011). Conclusion: LV-GCA patients were younger at diagnosis and suffered a longer diagnostic delay. The outcomes of the two subgroups were similar. An earlier introduction of steroid-sparing agents in LV-GCA patients might have played a positive role.

Ревматическая полимиалгия в клинических рекомендациях 2018–2020 гг. Часть II: диагностика васкулита

Совершенствование диагностики ревматических болезней пожилых людей и заболеваний аорты— актуальные задачи медицины. В патогенезе аневризм, расслоений, разрывов аорты важна роль воспаления и структурных изменений ее стенки, которые выявляют с помощью визуализирующих методов. Ведению ревматической полимиалгии, гигантоклеточного артериита, аневризм аорты посвящен ряд международных рекомендаций. С аортитом сопряжено до 40% случаев ревматической полимиалгии. Клиническое подозрение на него основано на выявлении асимметрии артериального давления и пульса, шума аортальной регургитации, сосудистых шумов, персистенции ревматической полимиалгии, воспалительной боли в спине, области таза и нижних конечностях. В 2020 году именно позитронно-эмиссионная томография/компьютерная томография рекомендована Итальянским обществом ревматологов для диагностики васкулита при ревматической полимиалгии во вторичном звене и Европейской федерацией головной боли для диагностики крупнососудистого гигантоклеточного артериита в неврологической практике. Приведен обзор рекомендаций Европейской ассоциации ядерной медицины, Общества ядерной медицины и молекулярной визуализации и Американского общества ядерной кардиологии по применению позитронно-эмиссионной томографии с фтордеоксиглюкозой в сочетании с компьютерной томографией (ангиографией) при васкулитах крупных сосудов и ревматической полимиалгии (2018). Они сопоставлены с клиническими руководствами, другими рекомендациями обществ ядерной медицины и новыми научными данными. Описана процедура исследования и подготовка к нему. Рассмотрены критерии оценки васкулита, предложенные для клинической практики и для клинических исследований, а также факторы, влияющие на результаты теста и их интерпретацию (атеросклероз, диабет, возраст, индекс массы тела, уровни гликемии и маркеров острой фазы). В рекомендациях обоснована польза применения позитронно-эмиссионной томографии и ее сочетания с компьютерной томографией для выявления экстракраниального васкулита и ценность компьютерной томографии-ангиографии на разных стадиях болезни. Требует усиления доказательная база стандарта времени экспозиции фтордеоксиглюкозы и пользы сочетания позитронной эмиссионной томографии с компьютерной томографией-ангиографией, в том числе для выявления рецидивов васкулита и мониторинга терапии. Необходим консенсус по раннему выполнению исследования, стандартизация оценок его результатов, обеспечение реимбурсации, внедрение новых методик визуализации сосудов черепа. В перспективе доказательный подход к ведению васкулита дополнит терагностика.

Rheumatologic diseases impact the risk of progression of MGUS to overt multiple myeloma

Monoclonal gammopathy of undetermined significance (MGUS), a premalignant condition, is associated with various chronic inflammatory rheumatic diseases (RDs) and is frequently observed as an incidental finding during routine work-up. The association of MGUS and chronic RDs is well established, but the impact of RDs on the risk of transformation into overt multiple myeloma (MM) has not been evaluated so far. MGUS patients diagnosed between January 2000 and August 2016 were identified and screened for concomitant RDs. RDs were grouped into antibody (Ab)-mediated RDs and non-Ab–mediated RDs (polymyalgia rheumatica, large-vessel giant cell arteritis, spondyloarthritis, and gout). Progression to MM was defined as a categorical (yes/no) or continuous time-dependent (time to progression) variable. Of 2935 MGUS patients, 255 (9%) had a concomitant RD. MGUS patients diagnosed with non-Ab–mediated RDs had a doubled risk of progression compared with those without a concomitant RD (hazard ratio, 2.1; 95% CI, 1.1-3.9; P = .02). These data translate into a 5-year risk of progression of 4% in MGUS patients without rheumatologic comorbidity, 10% in those with concomitant non-Ab–mediated RDS, and 2% in those with Ab-mediated RDs. By using the complex risk stratification model that includes myeloma protein (M-protein) concentration, immunoglobulin type, and level of free light chain ratio as variables, patients with non-Ab–mediated RDs (n = 57) had the highest risk for progression (hazard ratio, 6.8; 95% CI, 1.5-30.7; P = .01) compared with patients with Ab-mediated RDs (n = 77). Chronic inflammatory diseases have an impact on the risk of MGUS progressing into overt MM, with a doubled risk of transformation observed in patients with non-Ab–mediated RDs. Future research can elucidate whether comorbidities such as RDs should be included in currently applied prognostic MGUS scores.

Comment on: Evaluation of adjunctive mycophenolate for large vessel giant cell arteritis.

Comment on: Evaluation of adjunctive mycophenolate for large vessel giant cell arteritis.

Comment on: Evaluation of adjunctive mycophenolate for large vessel giant cell arteritis. Reply.

Comment on: Evaluation of adjunctive mycophenolate for large vessel giant cell arteritis. Reply.

Long-Term Efficacy and Safety of Leflunomide in Large-Vessel Giant Cell Arteritis: A Single-Center, 10-Year Experience.

Long-Term Efficacy and Safety of Leflunomide in Large-Vessel Giant Cell Arteritis: A Single-Center, 10-Year Experience.

Evidence for uncoupling of clinical and 18-FDG activity of PET/CT scan improvement in tocilizumab-treated patients with large-vessel giant cell arteritis.

Clinical improvement following tocilizumab (TCZ) therapy in patients with large-vessel (LVV) giant cell arteritis (GCA) is well established. However, information on TCZ effect on imaging vascular activity is limited. We aimed to determine if clinical improvement correlated with reduction of vascular 18F-fluorodeoxyglucose (18F-FDG) uptake in positron emission tomography (PET/CT) scans. Observational study of patients with refractory LVV-GCA treated with TCZ who had a baseline and a follow-up 18F-FDG-PET/CT scan. For the visual analysis of 18F-FDG vascular uptake, a total vascular score (TVS) was defined, ranging from 0 to 15. Besides, a semiquantitative analysis was performed as a target to background ratio (TBR)= SUVmax thoracic aorta wall/SUVmax aortic vascular pool. The baseline and follow-up TVS and TBR were compared. Clinical and lab¬oratory outcomes were also assessed. We included 30 patients (24 women/6 men); mean age± standard deviation 65.7± 9.8 years. Baseline PET/CT scans were performed due to active disease at a median [interquartile range-IQR] of 1.5 [0.0-4.0] months before TCZ onset. Following TCZ therapy, 25 (83.33%) patients achieved clinical remission and reduction of 18F-FDG vascular uptake was also observed after a mean ± standard deviation of 10.8±3.7 months. TBR decreased from 1.70 ± 0.52 to 1.48 ± 0.25 (p=0.005) and TVS from 4.97±2.62 to 3.13±1.89 (p< 0.001). However, only 9 (30.0%) patients showed complete normalisation of TBR and only 3 (10%) normalisation of TVS. TBR and TVS showed a good correlation (r=0.576). Although most of LVV-GCA patients achieve clinical remission after TCZ therapy, less than one-third show normalisation of 18F-FDG vascular uptake.

Comment on: Diagnostic accuracy of ultrasound for detecting large vessel giant cell arteritis using FDG PET/CT as the reference: reply.

Comment on: Diagnostic accuracy of ultrasound for detecting large vessel giant cell arteritis using FDG PET/CT as the reference: reply.

Comment on: Diagnostic accuracy of ultrasound for detecting large-vessel giant cell arteritis using FDG PET/CT as the reference.

Comment on: Diagnostic accuracy of ultrasound for detecting large-vessel giant cell arteritis using FDG PET/CT as the reference.

Vascular ultrasound for the diagnosis of giant cell arteritis: a reliability and agreement study based on a standardised training programme

ObjectiveTo evaluate the impact of a standardised training programme including equipment adjustment for experienced musculoskeletal ultrasonographers without previous experience in vascular ultrasound (US) on the reliability of US in the...

Evaluation of adjunctive mycophenolate for large vessel giant cell arteritis.

ObjectivesGCA patients with large vessel involvement (LV-GCA) experience greater CS requirements and higher relapse rates compared with classical cranial GCA. Despite the distinct disease course, interventions in LV-GCA have yet to be investigated specifically. This study aimed to evaluate the CS-sparing effect and tolerability of first-line mycophenolate in LV-GCA.MethodsA retrospective cohort study was conducted in patients with LV-GCA identified from a regional clinical database between 2005 and 2019. All cases were prescribed mycophenolate derivatives (MYC; MMF or mycophenolic acid) at diagnosis and were followed up for ≥2 years. The primary outcome was the cumulative CS dose at 1 year. Secondary outcomes included MYC tolerance, relapse rates and CRP levels at 1 and 2 years.ResultsA total of 37 patients (65% female; mean age 69.4 years, SD 7.9 years) were identified. All cases demonstrated large vessel involvement via CT/PET (n = 34), CT angiography (n = 5) or magnetic resonance angiography (n = 2). After 2 years, 31 patients remained on MYC, whereas 6 had switched to MTX or tocilizumab owing to significant disease relapse. The mean (±SD) cumulative prednisolone dose at 1 year was 4960 (±1621) mg. Relapse rates at 1 and 2 years were 16.2 and 27%, respectively, and CRP levels at 1 and 2 years were 4 [interquartile range (IQR) 4–6] and 4 (IQR 4–4) mg/l, respectively.ConclusionTo our knowledge, this is the first attempt to assess the effectiveness of any specific agent in LV-GCA. MYC might be both effective in reducing CS exposure and well tolerated in this subpopulation. A future randomized controlled trial is warranted.