Indian Takayasu Activity Score Research Articles

Ocular Manifestations in an Italian Cohort of Patients with Takayasu Arteritis

ABSTRACT Purpose We describe ophthalmic manifestations, therapy, and outcomes in 16 patients with Takayasu arteritis (TA). Methods Takayasu retinopathy was detected in 15 eyes of 9 patients and hypertensive retinopathy in 14 eyes of 7 patients. Results Visual acuity was normal in 7 eyes, 20/40 to 20/200 in 20 eyes, counting fingers in 2 eyes, hand motion in 2 eyes, and no light perception in 1 eye. Glucocorticoids associated with immunosuppressive agents induced a sustained remission in 13 patients. Three relapsing-refractory patients were given the monoclonal antibody tocilizumab, which led to partial and complete response in 1 and 2 patients respectively. Steroid-induced cataracts developed in 4 patients. Restenosis and the consequent recurrence of visual symptoms were detected in 2 of 9 patients who underwent a patency procedure for their stenotic lesions. Conclusions Ocular manifestations were a common feature (37.2%) in our cohort of TA patients and were frequently responsible for severe visual deterioration. Abbreviations BCVA: best-corrected visual acuity; FFA: fundus fluorescein angiography; GC: glucocorticoids; HR: hypertensive retinopathy; ITAS: Indian Takayasu activity score; OCT: optical coherence tomography; TA: Takayasu arteritis; TR: Takayasu retinopathy.

Treatment of childhood-onset Takayasu arteritis: switching between anti-TNF and anti-IL-6 agents.

Biologics are new treatment alternatives in Takayasu arteritis (TA), although data in childhood are limited. The aim of this study was to share our experience in seven childhood-onset TA patients who received a TNF-α inhibitor (adalimumab) or an IL-6 receptor inhibitor (tocilizumab) and the effect of switching therapy. We retrospectively evaluated the medical treatment records of seven patients with TA, followed between August 2005 and January 2021 at the Pediatric Rheumatology Department of Hacettepe University Faculty of Medicine. The median age of patients was 14 (IQR 4) years, and six were female. All of the patients had severe disease and high acute-phase reactants. The patients initially received only steroids or steroids+CYC. Prednisone was decreased, and biologic agents were started once the acute phase reactants decreased, and the Indian Takayasu Activity Score (ITAS) returned to normal. Initially, four patients received tocilizumab (TCZ) [median 25.5 (IQR 41) months] and three patients received adalimumab (ADA) [median 13 (IQR 31) months]. However, due to the progression of MR angiography findings or persistent elevation in acute-phase reactants, the biologic agents were switched from TCZ to ADA in four patients and from ADA to TCZ in three patients. The patients' median follow-up time after changing was 50 (IQR 77) months, and median ITAS was evaluated as '0' after 2 (IQR 4) months. In conclusion, both TNF-α and IL-6 inhibitors are effective alternatives in treating patients with childhood-onset TA. However, prospective randomized controlled trials are needed for the comparison of their effectiveness.

Tocilizumab for faster and safer remission of Takayasu's arteritis.

Takayasu's arteritis (TAK) is a large-vessel vasculitis. Glucocorticoids (GCs) combined with cyclophosphamide (CTX) is a common treatment option, but their efficacy is limited. The aim of this retrospective study was to study the efficacy and safety of tocilizumab (TCZ) in the treatment of TAK. A total of 63 patients with TAK were divided into two groups according to different treatment strategies: 31 patients in the TCZ group and 32 patients in the CTX group. After 6 months of treatment, disease activity and side effects were evaluated and were compared between the two groups. After 3 months of treatment, the National Institutes of Health (NIH) score (p = 0.005), Indian Takayasu Clinical Activity Score (ITAS) 2010 (p = 0.043), and the Indian Takayasu Activity Score with the Acute-Phase Response (ITAS.A; p = 0.036) were lower in patients treated with TCZ compared with those treated with CTX. In the TCZ group, the proportion of patients with NIH scores ⩽1 reached 50% after 1 month, 90% after 3 months, and 96% after 6 months of treatment, whereas in the CTX group, these proportion were 36%, 30%, and 78%, respectively. Thus, TCZ treatment of TAK is accompanied with a faster remission rate than CTX treatment. Moreover, the total cumulative incidence of adverse events was lower in the TCZ group than in the CTX group (p = 0.035). TCZ was faster and safer in alleviating TAK.

Fecal S100A12 in Takayasu arteritis predicts disease activity and intestinal involvement.

Gastrointestinal involvement in Takayasu Arteritis (TA) requires invasive or expensive diagnostic studies. Instead a fecal biomarker can be used as an initial screening test. In this context, S100A12 is promising as an established biomarker in intestinal inflammation and its role in TA pathogenesis. As such we aimed to test the feasibility of fecal S100A12 as a means of the fecal biomarker in gastrointestinal involvement in TA in this pilot study. Our study population consisted of 30 TA patients and 14 control patients with non-inflammatory arthralgia. Patients with inflammatory or infectious gastrointestinal tract diseases, or used oral antibiotics or NSAIDs for the 3weeks were excluded. Vasculitis involvements were determined with cross-sectional radiologic studies. TA disease activity was evaluated per Indian Takayasu's Activity Score (2010) criteria and vascular involvements were classified according to Numano classification. ELISA test was used to determine fecal S100A12 levels. Fecal S100A12 levels were higher in TA patients when compared to the controls (37.9ng/ml vs. 12.5ng/ml p = 0.038). ESR and CRP levels were also higher in the TA group, however not correlated with fecal S100A12. Among TA patients, fecal S100A12 levels were higher inactive ones with ITAS2010 > 1 (72.9ng/ml vs. 16.7ng/ml p = 0.016) correlated with total ITAS2010 scores. (R = 0.52 p = 0.003). TA patients with abdominal symptoms had higher fecal S100A12 levels when compared to the remaining TA population (327.8ng/ml vs. 28.0ng/ml p = 0.003). However, fecal S100A12 levels in patients with or without mesenteric vessel involvement did not differ. Fecal S100A12 shows promise as a fecal biomarker to screen intestinal ischemia and inflammatory bowel disease in TA patients.

Efficacy and safety of tocilizumab in patients with Takayasu arteritis

Objective: To assess the efficacy and safety of tocilizumab and cyclophosphamide in patients with Takayasu arteritis (TA). Methods: Twenty-seven TA patients treated with tocilizumab (TCZ group) and 22 treated with cyclophosphamide (CTX group) were enrolled and retrospectively analyzed. The duration of treatment was 6 months. Disease activity and side effects were compared between the two groups. Results: After treatment, the median C-reactive protein (CRP), erythrocyte sedimentation rate (ESR) and disease activity scores in TCZ group were significantly lower than those in CTX group respectively [ESR 3 mm/1h vs. 8 mm/1h; CRP 0.13 mg/L vs. 1.09 mg/L; National Institutes of Health (NIH) score 0(0,1) vs. 0(1,1); the Indian Takayasu clinical activity score (ITAS 2010) 0(0,2) vs. 2(0,3.5), and the Indian Takayasu activity score with the acute phase response (ITAS-A) 0(0,2) vs. 2.5(0,3.5); all P<0.05]. The daily prednisone doses before treatment and after treatment in TCZ group were significantly lower than those in CTX group [(20.1±15.9) mg/d vs. (39.3±16.7) mg/d;(5.1±4.2)mg/d vs. (12.1±4.6) mg/d,both P<0.05)].The incidence of drug-related side effects in TCZ group was significantly lower than that in CTX group, which was 22.2% vs. 54.5% (P<0.05). Conclusion: Compared with CTX treatment, TCZ treatment for TA with less prednisone has better efficacy and safety.

Leflunomide in Takayasu arteritis – A long term observational study

ObjectiveTo evaluate the extended follow-up data on efficacy and toxicity of leflunomide therapy in Takayasu arteritis (TA) patients previously enrolled in the original open-label study of short-term effects of leflunomide in TA. MethodsAn open-label long-term longitudinal study was performed in TA patients who fulfilled the 1990 American College of Rheumatology criteria for TA and had participated in a previous study that evaluated short-term efficacy of leflunomide in TA. Complete follow-up information could be retrieved from 12 out of 15 patients enrolled in the original study. Disease activity was evaluated by Kerr's criteria and by the Indian Takayasu Activity Score 2010 (ITAS2010). ResultsThe mean follow up time was 43.0±7.6 months and 5 (41.6%) TA patients remained on leflunomide therapy while 7 (58.3%) TA patients had to change to another therapy due to failure to prevent relapses in 6 patients and toxicity in one patient. No significant differences were found between patients who remained on leflunomide therapy and those who changed to another agent regarding age at study entry, time since diagnosis, prednisone daily dose at study entry, baseline ITAS2010, mean or maximum ESR and CRP, and cumulative prednisone dose at study end. Among TA patients who had changed leflunomide to another agent, two had an additional clinical relapse and needed to change therapy. ConclusionLeflunomide led to sustained remission in approximately half of patients at a mean time of 12 months and was well tolerated by TA patients.

Leflunomida na arterite de Takayasu – Estudo observacional de longo prazo

ObjectiveTo evaluate the extended follow-up data on efficacy and toxicity of leflunomide therapy in Takayasu arteritis (TA) patients previously enrolled in the original open-label study of short-term effects of leflunomide in TA. MethodsAn open-label long-term longitudinal study was performed in TA patients who fulfilled the 1990 American College of Rheumatology criteria for TA and had participated in a previous study that evaluated short-term efficacy of leflunomide in TA. Complete follow-up information could be retrieved from 12 out of 15 patients enrolled in the original study. Disease activity was evaluated by Kerr's criteria and by the Indian Takayasu Activity Score 2010 (ITAS2010). ResultsThe mean follow up time was 43.0±7.6 months and 5 (41.6%) TA patients remained on leflunomide therapy while 7 (58.3%) TA patients had to change to another therapy due to failure to prevent relapses in 6 patients and toxicity in one patient. No significant differences were found between patients who remained on leflunomide therapy and those who changed to another agent regarding age at study entry, time since diagnosis, prednisone daily dose at study entry, baseline ITAS2010, mean or maximum ESR and CRP, and cumulative prednisone dose at study end. Among two TA patients who had changed laflunomide to another agent, two had a clinical relapse and needed to change therapy. ConclusionLeflunomide led to sustained remission in approximately half of patients at a mean time of 12 months and was well tolerated by TA patients.

Development of a colour Doppler ultrasound scoring system in patients of Takayasu’s arteritis and its correlation with clinical activity score (ITAS 2010)

The objectives of this study were to develop a scoring system with colour Doppler ultrasound (CDUS) in patients with Takayasu's arteritis, to correlate the CDUS score with the Indian Takayasu's Activity Score (ITAS) and to assess the degree of agreement between CDUS and angiogram in the diagnosis of Takayasu's arteritis. Nineteen angiographically confirmed Takayasu's arteritis patients fulfilling three or more of the 1990 ACR criteria were evaluated. Their ITASs were recorded. A CDUS scoring system, CDUS-Kolkata (CDUS-K), was devised based on the presence of stenosis and altered flow patterns. It was then correlated with the ITAS. An inter-rater agreement analysis was done between the CDUS-K scores and angiographic scores in selected arterial sites. We found a significant degree of correlation between the ITAS and the CDUS-K score (r = 0.7144, 95% CI 0.3852, 0.8823, P = 0.0006). A high degree of correlation was found between the CDUS-K and angiographic scores in the selected arterial sites (κ-value = 0.725 on inter-rater agreement analysis). CDUS imaging may be used as an objective tool for assessing disease severity in Takayasu's arteritis. Development of a CDUS-K scoring system would be a supplementary tool to clinical scoring and the ITAS. CDUS imaging could be a cost-effective, non-invasive and reliable substitute for angiogram, especially for follow-up in Takayasu's arteritis patients.

AB0778 Role of pet scan in the management of patients with takayasu arteritis

BackgroundTakayasu’s arteritis (TA) is the most common large vessel vasculitis in India1. The rarity of the disorder and the heterogeneous nature of its clinical presentation predisposes to late diagnosis and...

OP0306 Evaluation of Disease Activity Using FDG PET-CT in Patients with Large Vessel Vasculitis

Background18F-Fluorodeoxyglucose (FDG) positron emission tomography (PET)/computerized tomography (CT) [PET/CT] has been proposed as a useful tool to diagnose and monitor activity of large vessel vasculitis (LVV), but its precise role...

SAT0167 Vascular Standardized Uptake Value of FDG-PET/CT Correlates with Indices of LVV Activity.

Background18F-Fluorodeoxyglucose (FDG) positron emission tomography/computerized tomography [PET/CT] is a useful tool to diagnose large vessel vasculitis (LVV) but its role in distinguishing between active and inactive disease is still debated.ObjectivesTo...

Vasculitis assessment and Takayasu aorto-arteritis

Assessment of disease activity using clinical instruments like Birmingham Vasculitis Activity Score and Vasculitis Damage index has been extensively used both in trial and clinic setting. It has revolutionised outcome assessment for small size vasculitis as evidenced from studies carried out by the European vasculitis group. There is a need for clinical assessment tools for large vessel disease like Takayasu arteritis, which is seen more frequently in India. An attempt has been made by the Indian Rheumatology Association Vasculitis core group to validate instruments, like Disease Extent Index for Takayasu Arteritis (DEI.Tak), Indian Takayasu Activity Score (ITAS) and Takayasu Arteritis Damage Scores. Both DEI.Tak and ITAS have received interests from international investigators. Widespread usage of these instruments will pave the way to controlled clinical trials for TA.

Tocilizumab: a novel therapy for patients with large-vessel vasculitis

Treatment of large-vessel vasculitis (LVV) remains challenging. Patients usually respond to glucocorticoid (GC) therapy, but often relapse on tapering of the GC dose or after GC withdrawal. In addition, GCs are fraught with numerous adverse events. The aim of this study was to assess the efficacy and safety of the anti-IL-6 receptor (IL-6R) antibody tocilizumab (TCZ) in patients with LVV. Four patients with active LVV (two with GCA and two with Takayasu arteritis) received monthly TCZ infusions (8 mg/kg bodyweight) for 6 consecutive months. Two patients were treatment naïve, while two had relapsing disease. Disease activity and drug tolerability were assessed clinically and by laboratory tests at study entry and subsequently every month for 6 months of TCZ treatment, while an [(18)F]fluorodeoxyglucose PET (PET/CT) scan was performed before and after treatment. In addition, a semi-quantitative clinical evaluation was performed at baseline and at 3 and 6 months using the Indian Takayasu activity score and the Kerr indices. After TCZ, MTX was used as maintenance therapy. All patients treated with TCZ therapy had a satisfactory clinical and laboratory response, while PET/CT findings significantly improved in all cases. No serious adverse events were noted. Only one patient had a transient increase in liver enzymes. In this small group of patients with LVV, treatment with TCZ was effective and well tolerated. Further, larger studies are required to confirm our findings.

P48 Low Indian Takayasu activity score aortoarteritis presenting as pyrexia of unknown origin: a case series

P48 Low Indian Takayasu activity score aortoarteritis presenting as pyrexia of unknown origin: a case series

Disease activity assessment in large vessel vasculitis

Disease activity assessment in large vessel vasculitis (LVV) is often challenging for physicians. In this study, we compared the assessment of disease activity based on inflammatory markers, clinical indices (Indian Takayasu Activity Score [ITAS] and the Kerr/National Institute of Health indices [Kerr/NIH]), and 18F-Fluorodesossiglucose (FGD) vascular uptake at positron emission tomography (Pet). We found that Pet results did not statistically correlate with the clinical indices ITAS and Kerr/NIH, because FDG uptake was increased (grade>2 on a 0-3 scale in at least one evaluated vascular segment) in many patients with inactive disease according to clinical and laboratory parameters (i.e., negative ITAS and Kerr/NIH indices as well as normal erythrocyte sedimentation rate (ESR) and C-reactive protein (PCR)). Similarly, interleukin- 6 and its soluble receptor did not statistically correlate with disease activity. In contrast, clinical indices showed a significant correlation between each other and with inflammatory markers (VES and PCR). These data suggest that while clinical indices and inflammatory markers may be useful to assess disease activity, Pet may be more sensitive.