scholarly journals Diagnosis and Assessment of Disease Activity in Takayasu Arteritis: A Childhood Case Illustrating the Challenge

2014 ◽  
Vol 2014 ◽  
pp. 1-6 ◽  
Author(s):  
L. Watson ◽  
P. Brogan ◽  
I. Peart ◽  
C. Landes ◽  
N. Barnes ◽  
...  
Keyword(s):  
Disease Activity ◽  
Takayasu Arteritis ◽  
Inflammatory Markers ◽  
Fatal Outcome ◽  
Large Vessel Vasculitis ◽  
Large Vessel ◽  
Childhood Onset ◽  
The Third ◽  
Assessment Of Disease Activity ◽  
Active Disease

Takayasu Arteritis (TA) is a rare, debilitating large vessel vasculitis occurring in patients of all ages, including infants, but the disease most commonly presents in the third decade. Diagnosis is often delayed and consequently TA is associated with significant morbidity and mortality. Accurate methods of monitoring disease activity or damage are lacking and currently rely on a combination of clinical features, blood inflammatory markers, and imaging modalities. In this report we describe a case of a 14-year-old boy with childhood-onset TA who, despite extensive negative investigations, did indeed have on-going active large vessel vasculitis with fatal outcome. Postmortem analysis demonstrated more extensive and active disease than originally identified. This report illustrates and discusses the limitations of current modalities for the detection and monitoring of disease activity and damage in large vessel vasculitis. Clinicians must be aware of these limitations and challenges if we are to strive for better outcomes in TA.

Assessment of Disease Activity in Takayasu Arteritis: Potential Role of Contrast-Enhanced Ultrasound

2019 ◽  
Vol 40 (05) ◽  
pp. 638-645 ◽  
Author(s):  
Christian Lottspeich ◽  
Claudia Dechant ◽  
Anton Köhler ◽  
Maximilian Tischler ◽  
Karla Maria Treitl ◽  
...  
Keyword(s):  
High Resolution ◽  
Disease Activity ◽  
Takayasu Arteritis ◽  
Clinical Symptoms ◽  
Inactive Disease ◽  
Contrast Enhanced Ultrasound ◽  
Acute Phase Reactants ◽  
Contrast Enhanced ◽  
Assessment Of Disease Activity ◽  
Active Disease

Abstract Purpose To assess the diagnostic value of intima media thickness measurements and contrast-enhanced ultrasound (CEUS) of the supraaortic arteries in the assessment of disease activity in Takayasu arteritis (TA). Materials and Methods Patients with TA and involvement of the carotid and/or subclavian/axillary arteries underwent CEUS imaging in addition to B-mode and color duplex ultrasound. The maximum IMT (mIMT) was measured and high-resolution CEUS of the most prominently thickened vessel segment was performed. Two blinded readers reviewed the CEUS movies, with semiquantitative assessment of microbubble enhancement of the arterial wall (grade 0: no or minimal; grade 1: moderate; grade 2: pronounced). Clinical symptoms, acute phase reactants, and established indices of clinical disease activity (NIH criteria, ITAS score) were recorded. Results 40 examinations in 17 patients were analyzed. According to clinical judgement, 27 and 13 cases were classified as clinically inactive and active, respectively. An mIMT-cutoff of > 2.7 mm identified active disease with a sensitivity and specificity of 69.2 % and 88.9 %, respectively (area under the curve 0.83). The interobserver agreement of CEUS analysis was substantial (Cohen’s kappa 0.76). By consensus reading, 17, 15, and 8 cases were classified as uptake grade 0, grade 1 and grade 2, respectively. Grade 0 uptake was exclusively present in 17 clinically inactive patients. Grade 1 uptake was seen in 10 patients with clinically inactive disease and 5 patients with clinically active disease. Grade 2 uptake was exclusively present in 8 patients with active disease. Conclusion Both sonographic IMT measurements and high-resolution CEUS are promising in the ad-hoc assessment of disease activity in patients with TA.

F-18 FDG PET for assessment of disease activity of large vessel vasculitis: A systematic review and meta-analysis

2018 ◽  
Vol 26 (1) ◽  
pp. 59-67 ◽  
Author(s):  
Sang-Woo Lee ◽  
Seong-Jang Kim ◽  
Youngduk Seo ◽  
Shin Young Jeong ◽  
Byeong-Cheol Ahn ◽  
...  
Keyword(s):  
Systematic Review ◽  
Disease Activity ◽  
Fdg Pet ◽  
Meta Analysis ◽  
Large Vessel Vasculitis ◽  
Large Vessel ◽  
Assessment Of Disease Activity

scholarly journals PET/MRI in large-vessel vasculitis: clinical value for diagnosis and assessment of disease activity

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Charlotte Laurent ◽  
Laure Ricard ◽  
Olivier Fain ◽  
Irene Buvat ◽  
Amir Adedjouma ◽  
...  
Keyword(s):  
Disease Activity ◽  
Large Vessel Vasculitis ◽  
Large Vessel ◽  
Clinical Value ◽  
Assessment Of Disease Activity

scholarly journals OP0069 THE ROLE OF POSITRON EMISSION TOMOGRAPHY/COMPUTED TOMOGRAPHY (PET/CT) IN DISEASE ACTIVITY ASSESSMENT IN PATIENTS WITH LARGE VESSEL VASCULITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 37.2-37
Author(s):  
E. Galli ◽  
F. Muratore ◽  
L. Boiardi ◽  
M. I. Casali ◽  
A. Versari ◽  
...  
Keyword(s):  
Disease Activity ◽  
Large Vessel Vasculitis ◽  
Large Vessel ◽  
Emission Tomography ◽  
Clinical Judgement ◽  
Positron Emission ◽  
Pet Ct ◽  
Active Disease ◽  
Age And Sex

Background:Assessment of disease activity in large vessel vasculitis (LVV) is still an unmet need. PET Vascular Activity Score (PETVAS) is a new composite score aimed at quantifying the overall inflammatory burden by adding together PET qualitative visual scores (0-3, according to Meller) in nine selected arterial regions (1). In two independent cohorts, PETVAS showed to be effective in discriminating between patients with clinically active and inactive vasculitis.Objectives:To assess the role of PET/CT and the performance of PETVAS in differentiating between clinically active and inactive vasculitis in a single center cohort of patients with LVV.Methods:One-hundred patients with radiographic evidence of LVV were enrolled by the Rheumatology Unit of Reggio Emilia Hospital (Italy) between June 2007 and September 2020. All subjects underwent full clinical, laboratory and imaging evaluation (including PET/CT) at baseline, annually and when a relapse was suspected. Medical records of recruited patients were retrospectively reviewed from baseline visit until 30 September 2020, last follow-up or death.For each PET/CT test, the nuclear medicine physician’s interpretation of scans (active/inactive vasculitis) was compared with disease activity clinical judgement (active disease/remission). The latter was based on comprehensive signs/symptoms assessment, laboratory and imaging (excluding PET/CT) data and was considered the reference standard.For each PET/CT scan, PETVAS score was calculated and its performance in discriminating between patients with active and inactive disease was compared to clinical judgement.Results:In the study period 100 LVV patients [51 giant cell arteritis (GCA), 49 Takayasu arteritis (TAK)] underwent a total of 474 PET scans. Nuclear medicine physician’s interpretation of PET/CT was able to discriminate between patients in clinically active LVV (n 167) and those in clinical remission (n 307) with a sensitivity of 60% (95% CI, 51 to 69%) and a specificity of 80% (95% CI, 75 to 84%). The following sensitivity and specificity values were found in LVV subgroups: 73% (95% CI, 59 to 84%) and 77% (95% CI, 70 to 83%) for TAK, and 51% (95% CI, 38 to 63%) and 82% (95% CI, 76 to 88%) for GCA, respectively.LVV patients with higher PETVAS scores were more frequently classified as having active disease: age and sex adjusted OR 1.15 (95% CI, 1.11 to 1.19), p<0.0001. Similar results were found in LVV subgroups, [age and sex adjusted OR 1.12 (95% CI, 1.08 to 1.17) for GCA and 1.22 (95% CI, 1.14 to 1.31) for TAK, all p<0.0001].The area under receiver operating characteristics (ROC) curve (AUC) of PETVAS in differentiating between clinically active and inactive LVV was 0.73 (95% CI, 0.68 to 0.79). Similar results were found in LVV subgroups, [0.70 (95% CI, 0.62 to 0.78) for GCA, and 0.79 (95% CI, 0.71 to 0.87) for TAK]. A PETVAS ≥10 provided 61% sensitivity and 80% specificity in differentiating between clinically active and inactive LVV (52% sensitivity and 82% specificity in GCA subgroup and 73% sensitivity and 78% specificity in TAK subgroup).Conclusion:In our cohort PET/CT has shown to be useful in monitoring LVV disease activity.PETVAS seems to be a reliable tool in helping clinicians to discriminate between LVV patients with active disease and those in remission.References:[1]Grayson PC, Alehashemi S, Bagheri AA, Civelek AC, Cupps TR, Kaplan MJ, Malayeri AA, Merkel PA, Novakovich E, Bluemke DA, Ahlman MA. 18 F-Fluorodeoxyglucose-Positron Emission Tomography as an Imaging Biomarker in a Prospective, Longitudinal Cohort of Patients with Large Vessel Vasculitis. Arthritis Rheumatol. 2018 Mar;70(3):439-449. doi: 10.1002/art.40379. Epub 2018 Feb 6. PMID: 29145713; PMCID: PMC5882488.Disclosure of Interests:None declared

scholarly journals 43. Large vessel vasculitis and sarcoidosis: co-existence or one disease?

2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Faidra Laskou ◽  
Philip Sajik ◽  
Leena Yalakki
Keyword(s):  
Lymph Nodes ◽  
Takayasu Arteritis ◽  
Inflammatory Markers ◽  
Erythema Nodosum ◽  
Conflicts Of Interest ◽  
Large Vessel Vasculitis ◽  
Large Vessel ◽  
Corneal Ulceration ◽  
Ct Angiogram ◽  
Ulcerative Keratitis

Abstract Introduction Takayasu arteritis (TA) is a large-vessel vasculitis that preferentially affects the aorta and its major branches, is rare, predominately affects women of child-bearing age and its precise aetiology is unknown. TA causes chronic vascular inflammation. Sarcoidosis, too, is a systemic inflammatory condition which can affect any organ system; the pulmonary system is the most common site. Large-vessel vasculitis is rare in sarcoidosis, but overlap between the two conditions has been reported. It is unclear whether they co-exist or manifest as one disease entity. We report a case of a 50-year-old lady with pulmonary sarcoidosis on a background of TA. Case description A 40-year-old female presented in 2010 with constitutional symptoms, erythema nodosum (confirmed on biopsy), audible murmurs over her carotids and subclavian arteries and raised inflammatory markers (CRP 100). She was diagnosed with Takayasu arteritis following CT angiogram which demonstrated periarterial cuffing and thickening of her carotids, subclavian and thoracic aorta. Her medical history consist of pericarditis in 1992, a thromboembolic event in 1995, ulcerative keratitis in 2006 and incidental aortic regurgitation in 2009.  She was treated with oral corticosteroids and started on azathioprine as a steroid sparing agent. Inflammatory markers normalised. Further cardiology assessments confirmed evidence of a dilated ascending aorta in 2015 and she was also diagnosed with corneal ulceration in September 2016. In July 2017, intermittent ankle swelling was reported which was associated with mildly raised inflammatory markers (CRP of 12, ESR of 27). Accentuating murmurs noted and in view of raised inflammatory markers, CT angiogram was repeated; that showed stable appearances of TA.  In May 2018, her azathioprine was reduced to 100mg from 125mg as she remained clinically and radiologically stable. In July 2018, she reported recurrence of night sweats and she had marginally raised CRP of 7 and ESR of 8. PET-CT, to look for active TA, demonstrated high uptake on bilateral mediastinal lymph nodes and no evidence of active TA. It was noted retrospectively that mediastinal lymphadenopathy was present on her CT back in 2017. She then underwent endobronchial ultrasound bronchoscopy in August 2018 which showed reactive lymph nodes. Other potential causes were excluded by extensive microbiological and immunology studies. Mediastinoscopy and lymph node excision was arranged as a lymphoproliferative/infective disease needed to be excluded in view of prolonged immunosuppression. Biopsy supported the diagnosis of sarcoidosis showing granulomatous changes. Oral prednisolone 40mg initiated and azathioprine was increased to 125mg. ACE levels remained normal. Discussion This case report emphasises the need for consideration of other systemic conditions in patients with known inflammatory diseases as they can co-exist. Patients who are presented with symptoms that are not fully consistent with a specific phenotype of a disease as in this case the ocular symptoms (corneal ulceration, ulcerative keratitis) and the erythema nodosum, could raise the possibility of a different or co-existent disease. It does also suggest that the prevalence of TA, or related forms of arteritis, may be higher than expected and should be considered, especially in younger patients with non-characteristic cardiovascular symptoms and suspected systemic inflammatory disease.  Moreover, the association with sarcoidosis in this and other previously described cases suggests that the two diseases may be related, and that TA or TA-like vasculitis may even be a complication of sarcoidosis. Other causes of large vessel vasculitis should be excluded as TB and lymphoproliferative diseases which can also present with lymphadenopathy especially as it is well known that large vessel vasculitis, especially in elderly population, could be part of a para-neoplastic syndrome.  Other diseases have been reported associated with TA but rarely sarcoidosis. TA and sarcoidosis may be related as they are characterized by certain nonspecific immunoinflammatory abnormalities. In most case reports sarcoidosis precedes TA diagnosis. In this case, TA was found 9 years before the diagnosis of sarcoidosis was made. Key learning points TA can precede the diagnosis of sarcoidosis. In case of relapsing or refractory TA, further investigations should be considered to exclude other co-existent pathologies as sarcoidosis. TA and sarcoidosis may be related as they are characterized by certain nonspecific immunoinflammatory abnormalities. It has been reported that TA stands as pathology-associated with sarcoidosis. Complete vascular clinical examination should be performed to detect inflammatory arteritis, especially in cured sarcoidosis presenting a relapse of the biological inflammatory process. Conflicts of interest The authors have declared no conflicts of interest.

scholarly journals POS0817 A NOVEL MODEL TO ASSESS DISEASE ACTIVITY IN TAKAYASU ARTERITIS BASED ON 18F-FDG-PET/CT: A CHINESE COHORT STUDY

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 661.3-662
Author(s):  
L. Ma ◽  
B. Wu ◽  
X. Jin ◽  
Y. Sun ◽  
X. Kong ◽  
...  
Keyword(s):  
Disease Activity ◽  
Takayasu Arteritis ◽  
Fdg Pet ◽  
Large Vessel ◽  
Activity Assessment ◽  
Pet Ct ◽  
Chinese Cohort ◽  
18F Fdg ◽  
Fdg Pet Ct ◽  
Active Disease

Background:Takayasu arteritis (TA) is a condition characterized by major large-vessel vasculitis (LVV), and is most commonly found in young women (age <40 years) of East Asia countries. 18F-FDG-PET/CT has been widely used in the diagnosis and follow-up of cancers to gather functional information based on metabolic activity. In the present study, we evaluated the value of different parameters in 18F-FDG-PET/CT for assessing active TA disease, and we establish a simple, quantifiable, and effective disease activity evaluation model based on 18F-FDG-PET/CT. A comparison in the ability to identify active disease was performed between the established Kerr score and the new 18F-FDG-PET/CT was also performed.Objectives:To investigate the utility of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG-PET/CT) in assessing disease activity in TA.Methods:Ninety-one patients with TA, were recruited from a Chinese cohort from October 2017 to January 2019. Clinical data, acute-phase reactants (APRs), and 18F-FDG-PET/CT findings were simultaneously recorded. The Physician Global Assessment was used as the gold standard to assess TA disease activity. The value of using 18F-FDG-PET/CT to identify active disease was evaluated, using erythrocyte sedimentation rate (ESR) as a reference. Disease activity assessment models were constructed and concordance index (C-index), net reclassification index (NRI), and integrated discrimination index (IDI) were evaluated to compare the benefits of the new modes with ESR and Kerr score.Results:In total, 64 (70.3%) cases showed active disease. Higher levels of ESR and CRP, and lower interleukin (IL)-2R levels, were observed in active cases. 18F-FDG-PET/CT parameters, including SUVmean, SUVratio1, SUVratio2, sum of SUVmean, and sum of SUVmax, were significantly higher in active disease groups. The C index threshold of ESR to indicate active disease was 0.78 (95% CI: 0.69-0.88). The new activity assessment model combining ESR, sum of SUVmean, and IL-2R showed significant improvement in C index over the ESR method (0.96 vs. 0.78, P < 0.01; NRI 1.63, P < 0.01; and IDI 0.48, P < 0.01). The new model also demonstrated modest superiority to Kerr score assessment (0.96 vs. 0.87, P = 0.03; NRI 1.19, P < 0.01; and IDI 0.33 P < 0.01).Conclusion:A novel 18F-FDG-PET/CT-based method that involves combining the sum of SUVmean with ESR score and IL-2R levels demonstrated superiority in identifying active TA compared to conventional methods.References:[1]Kerr GS, Hallahan CW, Giordano J, Leavitt RY, Fauci AS, Rottem M, et al. Takayasu arteritis. Ann Intern Med 1994;120:919-29.[2]Hoffman GS, Ahmed AE. Surrogate markers of disease activity in patients with Takayasu arteritis. A preliminary report from The International Network for the Study of the Systemic Vasculitides (INSSYS). Int J Cardiol 1998;66 Suppl 1:S191-4; discussion S195.[3]Misra R, Danda D, Rajappa SM, Ghosh A, Gupta R, Mahendranath KM, et al. Development and initial validation of the Indian Takayasu Clinical Activity Score (ITAS2010). Rheumatology (Oxford) 2013;52:1795-801.[4]Bardi M, Diamantopoulos AP. EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice summary. Radiol Med 2019;124:965-972.[5]Spick C, Herrmann K, Czernin J. 18F-FDG PET/CT and PET/MRI Perform Equally Well in Cancer: Evidence from Studies on More Than 2,300 Patients. J Nucl Med 2016;57:420-30.Disclosure of Interests:None declared

scholarly journals AB1009 THE EFFICACY OF TOCILIZUMAB ON THE TREATMENT OF TAKAYASU ARTERITIS IN CHINESE CHILDREN

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1798.2-1798
Author(s):  
C. Wang ◽  
H. Song ◽  
Z. Yu ◽  
M. Quan
Keyword(s):  
Takayasu Arteritis ◽  
Case Series ◽  
Large Vessel Vasculitis ◽  
Chinese Children ◽  
Large Vessel ◽  
Efficacy And Safety ◽  
Imaging Data ◽  
Eular Recommendations ◽  
Interleukin 6 Receptor ◽  
Sparing Effect

Background:Takayasu arteritis (TA) is the most prevalent large-vessel vasculitis in children. Patients with TA have a high mobidity and mortality.It remains a therapeutic challenge because corticosteroids monotherapy can rarely cure TAK and the relapse rate is high during GC tapering.Objectives:The aim of this study is to investigate the efficacy and safety of tocilizumab (TCZ)in Chinese children with Takayasu arteritis(TAK).Methods:We retrospectively studied 6 TAK children treated with TCZ in our hospital from July 2017 to October 2018. The demographic and clinical data, laboratory examination results and vascular imaging data were collected.Results:Six pediatric patients with critical or refractory TAK treated with TCZ were analyzed, including 3 males and 3 females.The diagnosis age was ranging in age from 2 to 13 years(median age:7 years).Three patients were initially treated with TCZ and Mycophenolate Mofetil(MMF) as the first-line regimen without corticosteroid or with a quite rapid GC taper duration,two of which had lifte-threatening coronary arteries involved and heart failure.The other three paitients were swcithed to TCZ from conventional disease modifying anti-rheumatic drugs (DMARDs) or other biologics due to being refractory to them and recurrent relapses.Four patients were given TCZ at 4 weeks regular intervals for 10 to 22 months,while two patients withdrew TCZ because of disease deterioration and unbearable abdominal or chest pain after the second dose.After 6 months follow-up,four patients experienced significant clinical and biological improvement with angiographically progression in one patient. A corticosteroid-sparing effect is obvious. Drug-related side effects occur in 1 patients manifesting as a mild elevated liver fuction. Neither neutropenia nor infection was observed.Conclusion:Our study shows a clinical, biological, and radiological response in patients with refractory TAK treated with TCZ.References :[1]Hellmich B, Agueda A, Monti S,et al.2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis 2019;0:1–12. doi:10.1136/annrheumdis-2019-215672.[2]BravoMancheño B, Perin F, Guez Vázquez Del ReyMDMR, García Sánchez A, Alcázar Romero PP. Successful tocilizumab treatment in a child with refractory Takayasu arteritis.Pediatrics 2012;130(6):e1720-724.[3]Goel R, Danda D, Kumar S, Joseph G. Rapid control of disease activity by tocilizumab in 10 «difficult-to-treat» cases of Takayasu arteritis. Int J Rheum Dis 2013;16(6):754–61.[4]Cañas CA, Cañas F, Izquierdo JH, Echeverri A-F, Mejía M, Bonilla-Abadía F, et al. Efficacy and safety of anti-interleukin 6 receptor monoclonal antibody (tocilizumab) in Colombian patients with Takayasu arteritis. J Clin Rheumatol Pract Rep Rheum Musculoskelet Dis 2014;20(3):125–9.[5]Batu ED, Sönmez HE, Hazirolan T, Özaltin F, Bilginer Y, Özen S. Tocilizumab treatment in childhood Takayasu arteritis: case series of four patients and systematic review of the literature. Semin Arthritis Rheum 2017 Feb;46(4):529–35.Disclosure of Interests:None declared

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