Cessation of immunosuppressive therapies in patients with Takayasu arteritis with sustained inactive 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.

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Management of Takayasu arteritis: a systematic literature review informing the 2018 update of the EULAR recommendation for the management of large vessel vasculitis

RMD Open ◽

10.1136/rmdopen-2019-001020 ◽

2019 ◽

Vol 5 (2) ◽

pp. e001020 ◽

Cited By ~ 12

Author(s):

Ana F Águeda ◽

Sara Monti ◽

Raashid Ahmed Luqmani ◽

Frank Buttgereit ◽

Maria Cid ◽

...

Keyword(s):

Takayasu Arteritis ◽

Meta Analysis ◽

Case Series ◽

Immunosuppressive Drugs ◽

Large Vessel Vasculitis ◽

Large Vessel ◽

Inactive Disease ◽

Controlled Clinical Trial ◽

Level Of Evidence ◽

Randomised Controlled

ObjectiveTo collect available evidence on management of large vessel vasculitis to inform the 2018 update of the EULAR management recommendations.MethodsTwo independent systematic literature reviews were performed, one on diagnosis and monitoring and the other on drugs and surgical treatments. Using a predefined PICO (population, intervention, comparator and outcome) strategy, Medline, Embase and Cochrane databases were accessed. Eligible papers were reviewed and results condensed into a summary of findings table. This paper reports the main results for Takayasu arteritis (TAK).ResultsA total of 287 articles were selected. Relevant heterogeneity precluded meta-analysis. Males appear to have more complications than females. The presence of major complications, older age, a progressive disease course and a weaker inflammatory response are associated with a more unfavourable prognosis. Evidence for details on the best disease monitoring scheme was not found. High-quality evidence to guide the treatment of TAK was not found. Glucocorticoids are widely accepted as first-line treatment. Conventional immunosuppressive drugs and tumour necrosis factor inhibitors were beneficial in case series and uncontrolled studies. Tocilizumab failed the primary endpoint (time to relapse) in a randomised controlled clinical trial; however, results still favoured tocilizumab over placebo. Vascular procedures may be required, and outcome is better when performed during inactive disease.ConclusionsEvidence to guide monitoring and treatment of patients with TAK is predominantly derived from observational studies with low level of evidence. Therefore, higher-quality studies are needed in the future.

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Intervention in Takayasu Aortitis: When, Where and How?

Hearts ◽

10.3390/hearts1020008 ◽

2020 ◽

Vol 1 (2) ◽

pp. 62-74

Author(s):

Andrew Porter ◽

Justin C. Mason

Keyword(s):

Takayasu Arteritis ◽

Vascular Inflammation ◽

Treatment Options ◽

Giant Cells ◽

Arterial Injury ◽

Aortic Disease ◽

Arterial Stenosis ◽

Inactive Disease ◽

Surgical Approaches ◽

Aortic Dilatation

Takayasu arteritis is a large vessel vasculitis which commonly affects the aorta and its major branches. Active arterial inflammation is characterised by the presence of T and B lymphocytes, natural killer cells, macrophages and occasional multinucleate giant cells. Uncontrolled vascular inflammation can progress to cause arterial stenosis, occlusion or aneurysmal dilatation. Medical treatment involves combination immunosuppression and more recently biologic therapies targeting TNF-α and IL-6. Due to the typical delays in diagnosis and accumulation of arterial injury, open and endovascular surgical intervention are important and potentially life-saving treatment options for Takayasu arteritis. Common indications for surgery include aortic coarctation and ascending aortic dilatation ± aortic valve regurgitation, renal artery stenosis, ischaemic heart disease, supra-aortic disease, mesenteric ischaemia, severe limb-threatening claudication and aneurysm repair. Surgical outcomes are markedly improved in patients with clinically inactive disease and those who receive adequate periprocedural immunosuppression. Decisions regarding surgical approaches are best made as part of a multi-disciplinary team.

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A Long-term Survival after Surgical Treatment for Atypical Aortic Coarctation Complicating Takayasu Arteritis with Inactive Disease at the Diagnosis: An Appropriately Treated Autopsy Case

Internal Medicine ◽

10.2169/internalmedicine.2483-18 ◽

2019 ◽

Vol 58 (15) ◽

pp. 2241-2246 ◽

Cited By ~ 2

Author(s):

Misaki Yoshida ◽

Takeshi Zoshima ◽

Satoshi Hara ◽

Ichiro Mizushima ◽

Hiroshi Fujii ◽

...

Keyword(s):

Surgical Treatment ◽

Takayasu Arteritis ◽

Aortic Coarctation ◽

Inactive Disease ◽

Autopsy Case ◽

Term Survival ◽

Long Term Survival ◽

Atypical Aortic Coarctation

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Outcome of Takayasu Arteritis with Inactive Disease at Diagnosis: The Extent of Vascular Involvement As a Predictor of Activation

The Journal of Rheumatology ◽

10.3899/jrheum.140981 ◽

2014 ◽

Vol 42 (3) ◽

pp. 489-494 ◽

Cited By ~ 7

Author(s):

Seokchan Hong ◽

Seung-Hyeon Bae ◽

Soo Min Ahn ◽

Doo-Ho Lim ◽

Yong-Gil Kim ◽

...

Keyword(s):

Takayasu Arteritis ◽

National Institutes Of Health ◽

Inactive Disease ◽

Vascular Involvement ◽

Stable Group ◽

Group 12 ◽

Increased Risk ◽

Risk Of Disease ◽

Group 5 ◽

Type V

Objective.Some patients with Takayasu arteritis (TA) have inactive disease at the time of diagnosis. The objective of our study was to investigate the clinical outcomes and factors that predict disease activation in patients with clinically inactive TA.Methods.The medical records of patients diagnosed with TA between 1990 and 2012 were reviewed. At the time of diagnosis, patients were identified as having inactive disease according to the National Institutes of Health definition. Patients who went on to develop active disease during followup were classified as the “activation group”. The pattern of vascular involvement was classified according to the International Conference on TA, 1994.Results.A total of 59 patients with TA were classified as having inactive disease at the time of diagnosis. During the followup, 13 (22.0%) of these experienced TA activation (median followup, 37.0 mos; activation group). The remaining 46 (78.0%) did not experience disease activation (stable group). Renovascular hypertension was more common in the activation group than in the stable group (5/13, 38.5% vs 4/46, 8.7%, p = 0.019). Further, type V, which is the most extensive, was more common in the activation group (12/13, 92.3%) than in the stable group (18/46, 39.1%, p = 0.008). Multivariate analysis identified type V disease (OR 10.969, 95% CI 1.144–105.182, p = 0.038) as being significantly associated with an increased risk of disease activation.Conclusion.Substantial portions of patients with clinically inactive TA at the time of diagnosis experienced disease activation during followup. Type V disease may be an important predictive factor for disease activation in patients with clinically inactive TA.

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Assessment of Patients with Takayasu Arteritis in Routine Practice with Indian Takayasu Clinical Activity Score

The Journal of Rheumatology ◽

10.3899/jrheum.140817 ◽

2015 ◽

Vol 42 (8) ◽

pp. 1443-1447 ◽

Cited By ~ 12

Author(s):

Fatma Alibaz-Oner ◽

Sibel Z. Aydin ◽

Servet Akar ◽

Kenan Aksu ◽

Sevil Kamali ◽

...

Keyword(s):

Takayasu Arteritis ◽

Global Assessment ◽

Vascular Imaging ◽

Activity Score ◽

Inactive Disease ◽

Clinical Activity ◽

Routine Practice ◽

Clinical Activity Score ◽

Future Work ◽

Total Agreement

Objective.To assess the Indian Takayasu Clinical Activity Score (ITAS2010) in followup of Takayasu arteritis (TA).Methods.ITAS2010 forms were filled in prospectively (n = 144). Clinical activity was assessed with physician’s global assessment (PGA) and criteria defined by Kerr,et al.Results.ITAS2010 was significantly higher in patients with active disease. Total agreement between ITAS2010 and PGA was 66.4%, and between ITAS2010 and Kerr,et alwas 82.8%. During followup, 14 of 15 patients showing vascular progression with imaging were categorized as having inactive disease according to ITAS2010.Conclusion.ITAS2010 was discriminatory for activity during the followup, but the agreement between PGA and ITAS2010 was moderate. Future work should include the incorporation of advanced vascular imaging and demonstration of ITAS2010 as a scalable measure and not simply a dichotomous measure of activity/flare versus remission.

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Contrast-enhanced Ultrasonography for Monitoring Arterial Inflammation in Takayasu Arteritis

The Journal of Rheumatology ◽

10.3899/jrheum.180701 ◽

2019 ◽

Vol 46 (6) ◽

pp. 616-622 ◽

Cited By ~ 2

Author(s):

ZhiQin Li ◽

ZhaoHui Zheng ◽

Jin Ding ◽

XiaoFeng Li ◽

YongFeng Zhao ◽

...

Keyword(s):

Disease Activity ◽

Takayasu Arteritis ◽

Fdg Pet ◽

Vascular Inflammation ◽

Inactive Disease ◽

Clinical Activity ◽

Visual Grade ◽

Fdg Uptake ◽

Contrast Enhanced ◽

Bilateral Carotid

Objective.To evaluate the utility of contrast-enhanced ultrasound (CEUS) compared with 18F-fluorodeoxyglucose–positron emission tomography (FDG-PET) in assessing vessel inflammation of Takayasu arteritis (TA).Methods.This is a retrospective analysis of 71 patients with TA who had undergone carotid CEUS. Twenty-two of 71 patients underwent FDG-PET after CEUS. Clinical disease activity was assessed by Kerr criteria and the Indian Takayasu Clinical Activity Score 2010 (ITAS2010). We investigated the correlation between carotid vascularization on CEUS and clinical data. The consistency of carotid CEUS and PET data has been analyzed for TA disease activity.Results.There was a statistically significant correlation between the results of CEUS and ITAS2010 (p = 0.004) or Kerr criteria (p < 0.001). According to ITAS2010, thirty-four of 71 patients with TA were clinically inactive. Assessment of 34 TA patients with clinically inactive disease yielded 11 CEUS scans that showed active lesions (visual grade ≥ 2) in the left or right carotid artery. In 22 cases that underwent CEUS and FDG-PET, 12 were active and 10 were inactive on the basis of ITAS2010. Moreover, bilateral carotid CEUS vascularization score positively correlated with vascular FDG uptake in these patients with TA (p = 0.004). When vascular inflammation was defined as FDG uptake with visual grade ≥ 2, carotid CEUS showed sensitivity of 100% and specificity of 80%.Conclusion.For TA patients with clinically inactive disease, CEUS could help clinicians to identify active lesions in the carotid vascular region. Carotid CEUS may be a rapid and cost-effective imaging tool in the followup of patients with TA.

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