[A case of Takayasu arteritis complicated with cavernous sinus syndrome presenting as unilateral optic nerve DWI hyperintensity].

A 47-year-old male patient with a history of Takayasu arteritis presented with prominent symptoms of left eyeball fixation, protrusion, and visual loss. Orbital magnetic resonance imaging revealed hyperintensity on diffusion-weighted imaging of the left optic nerve, with corresponding low signal on apparent diffusion coefficient maps, suggestive of acute infarction of the left optic nerve. Combined with the patient's cranial magnetic resonance imaging findings, the diagnosis of cavernous sinus syndrome was established.

Comparison of methotrexate and azathioprine as the first-line steroid-sparing immunosuppressive agents in patients with Takayasu's arteritis.

BACKGROUND: Immunosuppressive (IS) agents are recommended for the first-line treatment of patients with active Takayasu's arteritis (TAK) together with glucocorticoids (GCs). However, there is limited data comparing the efficacy and outcomes of different IS agents for this purpose. OBJECTIVES: In this study, we aimed to compare the outcomes of two most frequently used first-line IS agents, namely methotrexate (MTX) and azathioprine (AZA) in TAK patients. METHODS: TAK patients who received any IS agent in addition to GCs as the initial therapy were included in this multicentre, retrospective cohort study. Clinical, laboratory and imaging data of the patients were assessed. In addition, a matched analysis (cc match) using variables 'age', 'gender' and 'diffuse aortic involvement' was performed between patients who received MTX or AZA as the first-line IS treatment. RESULTS: We recruited 301 patients (F/M: 260/41, mean age: 42.2 ± 13.3 years) from 10 tertiary centres. As the first-line IS agent, 204 (67.8 %) patients received MTX, and 77 (25.6 %) received AZA. Less frequently used IS agents included cyclophosphamide in 17 (5.6 %), leflunomide in 2 (0.5 %) and mycophenolate mofetil in one patient. The remission, relapse, radiographic progression and adverse effect rates were similar between patients who received MTX and AZA as the first-line IS agent. Vascular surgery rate was significantly higher in the AZA group (23% vs. 9 %, p = 0.001), whereas the frequency of patients receiving ≤5 mg/day GCs at the end of the follow-up was significantly higher in the MTX group (76% vs 62 %, p = 0.034). Similarly, the rate of vascular surgery was higher in AZA group in matched analysis. Drug survival was similar between MTX and AZA groups (median 48 months, MTX vs AZA: 32% vs 42 %, p = 0.34). IS therapy was discontinued in 18 (12 MTX, 6 AZA) patients during the follow-up period due to remission. Among those patients, two patients had a relapse at 2 and 6 months, while 16 patients were still on remission at the end of a mean 69.4 (±50.9) months of follow-up. CONCLUSIONS: Remission, relapse, radiographic progression and drug survival rates of AZA and MTX were similar for patients with TAK receiving an IS agent as the first-line f therapy. The rate of vascular surgery was higher and the rate of GC dose reduction was lower with AZA compared to MTX at the end of the follow-up.

Coronary artery calcification in Takayasu's arteritis: clinical characteristics and risk factors.

OBJECTIVES: Coronary artery calcification (CAC) is frequently observed in Takayasu's arteritis (TAK). Our objective is to calculate the prevalence and severity of CAC in TAK, while evaluating the influence of traditional cardiovascular risk factors, glucocorticoid exposure, and disease activity on CAC. METHODS: This retrospective study involved 155 TAK patients. We measured the Agatston score by coronary computed tomography angiography (CCTA) and categorised all patients into groups with or without CAC (41 vs. 114) to compare clinical characteristics and ancillary findings between the two groups. RESULTS: Among the TAK patients, a total of 41 TAK patients (26.45%) exhibited CAC. Age of onset, disease duration, history of hypertension, history of hyperlipidaemia, Numano V and glucocorticoid use emerged as the independent risk factors for developing CAC in TAK (OR [95% CI] 1.084[1.028-1.142], p=0.003; 1.005 [1.001-1.010], p=0.020; 4.792 [1.713-13.411], p=0.003; 4.199 [1.087-16.219], p=0.037; 3.287 [1.070-10.100], p=0.038; 3.558[1.269-9.977], p=0.016). Nonetheless, CAC was not associated with disease activity. Moreover, the extent of calcification score in TAK showed a positive correlation with the number of traditional cardiovascular risk factors. CONCLUSIONS: We recommend CCTA screening for Numano V classified TAK patients. Glucocorticoid usage significantly escalates the risk of CAC. Therefore, in cases of effectively controlled disease, the inclusion of immunosuppressants aimed at reducing glucocorticoid dosage is advisable.

Benefits and challenges of 18F-FDG PET/CT in patients with Takayasu arteritis.

AIM: To evaluate the diagnostic performance of 2-[18F]-fluoro-2-deoxy-d-glucose (18F-FDG) positron-emission tomography (PET)/computed tomography (CT) in TA diagnosis and Takayasu arteritis (TA) activity assessment. MATERIALS AND METHODS: This retrospective study included patients with TA diagnosed according to the American College of Rheumatology (ACR) criteria and undergoing 18F-FDG PET/CT imaging from October 2010 to July 2022. TA activity was assessed through 18F-FDG PET/CT (maximum standard uptake value [SUVmax], vascular SUVmax/mean standard uptake value [SUVmean] of liver (SUV ratio), and PET vascular activity score [PETVAS]) using physician global assessment (PGA) as the reference standard, and the results of these assessments were compared against the clinical activity scores (National Institutes of Health [NIH] and Indian Aortitis Disease Activity [ITAS-A] scores), acute-phase reactants (APR), and white blood cell and platelet counts. RESULTS: Twenty 18F-FDG PET/CT examinations from 19 patients were included in the study, nine were performed in the active phase and 11 in the inactive phase. The involved vessels showed segmental and tubular FDG uptake in the active group. The average SUVmax, SUV ratio, and PETVAS was 6.3 ± 2.7 (range 3.4-12), 4.2 ± 1.7 (range 2.1-7.5), and 22.7 ± 11.2 (range 6-39), respectively, in the active group and 1.7 ± 0.9 (0.9-3.1), 1.1 ± 0.6 (range 0.6-2.4), and 3.5 ± 5.5 (range 0-18), respectively, in the inactive group. The sensitivity, specificity of SUVmax, SUV ratio, and PETVAS for TA activity assessment were 100%, 100%; 100%, 90.9%; and 88.9, 90.9%, respectively. After ROC curve analysis, a new SUVmax cut-off was obtained. Based on the new cut-off value, SUVmax 3.3 and SUV ratio 1.9 had a more perfect assessment performance. CONCLUSION: 18F-FDG PET/CT is an alternative imaging technique for TA.

Unusual overlap of systemic sclerosis with Takayasu arteritis.

Overlap syndromes are diseases that meet the criteria of two or more rheumatic diseases. In this case report, a woman in her 20s presented with a constellation of symptoms, including skin thickening, Raynaud's phenomenon, hypertension, absent pulse in both lower limbs with bilateral renal artery bruit. The antinuclear antibody profile revealed Scl-70 positivity. CT thorax identified early interstitial lung disease, and nailfold capillaroscopy showed severe capillary loss. CT angiogram features were suggestive of Takayasu arteritis. Notably, there have been only four documented cases of systemic sclerosis coexisting with Takayasu arteritis, highlighting the rarity of this overlap syndrome. The diagnosis of overlap syndrome was made after a thorough history recording and clinical examination. In the presence of bilateral renal artery stenosis, managing the scleroderma renal crisis may be challenging . This patient received treatment with mycophenolate mofetil and oral corticosteroids, aiming to address both systemic sclerosis and Takayasu arteritis effectively.

Dark-Blood Computed Tomography Angiography Combined With Deep Learning Reconstruction for Cervical Artery Wall Imaging in Takayasu Arteritis.

OBJECTIVE: To evaluate the image quality of novel dark-blood computed tomography angiography (CTA) imaging combined with deep learning reconstruction (DLR) compared to delayed-phase CTA images with hybrid iterative reconstruction (HIR), to visualize the cervical artery wall in patients with Takayasu arteritis (TAK). MATERIALS AND METHODS: This prospective study continuously recruited 53 patients with TAK (mean age: 33.8 ± 10.2 years; 49 females) between January and July 2022 who underwent head-neck CTA scans. The arterial- and delayed-phase images were reconstructed using HIR and DLR. Subtracted images of the arterial-phase from the delayed-phase were then added to the original delayed-phase using a denoising filter to generate the final-dark-blood images. Qualitative image quality scores and quantitative parameters were obtained and compared among the three groups of images: Delayed-HIR, Dark-blood-HIR, and Dark-blood-DLR. RESULTS: Compared to Delayed-HIR, Dark-blood-HIR images demonstrated higher qualitative scores in terms of vascular wall visualization and diagnostic confidence index (all P < 0.001). These qualitative scores further improved after applying DLR (Dark-blood-DLR compared to Dark-blood-HIR, all P < 0.001). Dark-blood DLR also showed higher scores for overall image noise than Dark-blood-HIR (P < 0.001). In the quantitative analysis, the contrast-to-noise ratio (CNR) values between the vessel wall and lumen for the bilateral common carotid arteries and brachiocephalic trunk were significantly higher on Dark-blood-HIR images than on Delayed-HIR images (all P < 0.05). The CNR values were significantly higher for Dark-blood-DLR than for Dark-blood-HIR in all cervical arteries (all P < 0.001). CONCLUSION: Compared with Delayed-HIR CTA, the dark-blood method combined with DLR improved CTA image quality and enhanced visualization of the cervical artery wall in patients with TAK.

Outcome of Open Repair of Thoracoabdominal Aortic Aneurysm in Takayasu Arteritis: A Retrospective Analysis.

BACKGROUND: Takayasu Arteritis (TA) is an immune mediated arteritis causing inflammation of the aorta and its branches, which can result in aortic aneurysms. Our aim is to describe the outcome of surgical management in these patients who presented with Thoracoabdominal aortic aneurysm (TAAA). METHODS: Between 2003 and 2023, 40 TA patients with TAAA underwent operative repair. RESULTS: There were 24 females and 16 males, in the age group of 19-53 years, with hypertension in 20 patients. Raised Erythrocyte sedimentation Rate was present in 13 patients. According to Crawford classification, there were 2 patients with type I, 2 with type II, 17 with type III, 12 patients with type IV and 7 with type V aneurysm. Multiple steno-occlusive lesions of aortic branches were present in 21 patients, with majority affecting the renal artery. Femoral Artery Femoral Vein Partial cardiopulmonary bypass was used for types I, II, III and V. Separate bypass to visceral branches was done in eight patients, of whom five had multiple bypasses and three patients only had renal bypass. Twelve patients underwent reimplantation of branches, out of which nine had multiple vessel reimplantation. Four patients underwent staged repair of the aneurysm, which included visceral debranching in the first day, followed by repair of the aneurysm in the next day. In the immediate postoperative period, ten patients developed acute kidney injury and two required dialysis. Other morbidities included acute respiratory distress syndrome (ARDS), spinal cord dysfunction, bleeding, and wound complications. Three patients expired in the immediate postoperative period. Mean duration of intensive care unit stay was 4.1 days and hospital stay was 12.7 days. Comparison of disease activity with morbidity and mortality was statistically insignificant. Patients were on follow-up for a range of 6 months to 14 years and median follow-up of 25 months. Over this time period four patients expired and four developed anastomotic pseudoaneurysm requiring intervention. On comparing the disease activity at the time of surgery with the long-term arteritis related complications that required intervention, the P value was 0.653 and hence statistically not significant. The 10-year survival rate is 84.4%. CONCLUSIONS: Surgical repair has good and satisfactory outcome, with low early and late mortality rates. Progression of disease can occur at any stage of the disease, hence indicating the need for long term follow-up and frequent imaging.

Takayasu's arteritis associated with Crohn's disease treated with infliximab.

A 23-year-old woman presented with fever, diarrhea, bloody stools, and arthralgia that did not improve despite previous treatments and was diagnosed with Crohn's disease. Remission was achieved after the introduction of infliximab, nutritional therapy, and 5-aminosalicylic acid treatment. However, the patient's blood sedimentation rate remained elevated without symptom recurrence, except for abdominal pain in the following year. Aortic wall thickening in the thoracic descending aorta was also observed on computed tomography. Accumulation in the thoracic descending aorta and abdominal aorta was confirmed using positron emission tomography-computed tomography. The patient was diagnosed with Takayasu's arteritis. The patient's abdominal symptoms resolved, and her blood sedimentation rate normalized after steroid administration.

Development of the Takayasu Arteritis Integrated Disease Activity Index.

OBJECTIVE: Accurate clinical assessment of disease activity in Takayasu arteritis (TAK) can be challenging. 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET) can directly measure vascular inflammation. This study details the development of a new type of disease activity index called the Takayasu's Arteritis Integrated Disease Activity Index (TAIDAI). METHODS: Clinical symptoms for TAIDAI were identified from a literature review. Each symptom was paired with FDG-PET findings in corresponding arterial territories. Constitutional symptoms were paired with acute phase reactant levels. One point was given for each clinical symptom paired with supporting FDG-PET or laboratory abnormalities and summed into the TAIDAI score. A TAIDAI of ≥1 defined active disease. To assess performance of TAIDAI, face validity, content validity, and sensitivity to change were evaluated within a prospective observational cohort study of patients with TAK. RESULTS: Seventeen clinical symptoms were paired with imaging or laboratory abnormalities. In a cohort of 96 patients contributing 204 study visits, TAIDAI showed excellent sensitivity (96.3%) and good specificity (79.2%) compared to physician's clinical assessment. TAIDAI significantly correlated with physician global assessment, PET Vascular Activity Score, patient global assessment, and acute phase reactant levels. In patients treated with either tumor necrosis factor inhibitors or tocilizumab, a TAIDAI of 0 was achieved in 21 (91%) of 23 patients who met a predefined definition of clinical response. CONCLUSION: TAIDAI is new type of disease activity index in TAK in which clinical symptoms are integrated with specific laboratory and imaging findings. TAIDAI should be validated in future randomized controlled trials in TAK.

Phlebitis in Takayasu arteritis: A case-based literature review.

Takayasu arteritis (TAK) is the main type of large vessel arteritis in young adults, which mainly affects the aorta and its main branches, leading to clinical manifestations such as syncope, intermittent limb claudication, hypertension, and abdominal pain. Among them, venous involvement is rarely reported. Here we show a case of TAK presenting as phlebitis. This was a 27-year-old woman, who initially admitted to our hospital with myalgia of the upper and lower extremities and night sweats. She was diagnosed as TAK according to the 1990 American College of Rheumatology TAK criteria. Surprisingly, vascular ultrasonography revealed wall thickening as indicated by macaroni sign of the multiple veins. TAK phlebitis appeared at the active phase, while disappearing rapidly at remission. Phlebitis might have a close relationship with disease activity. By retrospective study in our department, the estimated incidence rate of phlebitis might be 9.1% in TAK. With the literature review, it revealed that phlebitis might be an ignored manifestation in active TAK. However, due to the smaller sample size, it should be noted that a direct cause-effect relationship cannot be established.

Does Bronchial Artery Hypertrophies After Descending Thoracic Aorta Stenting in Takayasu Arteritis? - A Clinical Dilemma.

41-year-old lady, known case of Takayasu arteritis with pulmonary arterial involvement, presented with multiple episodes of haemoptysis (maximum 50 mL) in a week. She had undergone descending thoracic aorta angioplasty and stenting 3 years ago due to uncontrolled hypertension, left ventricular systolic dysfunction and approximately 70% stenosis of descending thoracic aorta. This lady was treated with embolization of hypertrophied bronchial artery as well as left internal mammary artery branch for management of haemoptysis. Embolization of hypertrophied bronchial artery in the setting of Takayasu arteritis with pulmonary arterial involvement presenting with haemoptysis is rare. Hypertrophied bronchial artery detected in post stenting computed tomography raises suspicion whether descending thoracic aorta stenting promotes the hypertrophy of bronchial artery. Literature of bronchial artery embolization in the setting of Takayasu with post DTA stenting is scarce.

The feasibility of quantitative assessment of dynamic 18F-fluorodeoxyglucose PET in Takayasu's arteritis: a pilot study.

PURPOSE: PET has been demonstrated to be sensitive for detecting active inflammation in Takayasu's arteritis (TAK) patients, but semi-quantitative-based assessment may be susceptible to various biological and technical factors. Absolute quantification via dynamic PET (dPET) may provide a more reliable and quantitative assessment of TAK-active arteries. The purpose of this study was to investigate the feasibility and efficacy of dPET in quantifying TAK-active arteries compared to static PET. MATERIALS AND METHODS: This prospective study enrolled 10 TAK-active patients (fulfilled the NIH criteria) and 5 control participants from March to October 2022. One-hour dPET scan (all TAK and control participants) and delayed static PET scan at 2-h (all TAK patients) were acquired. For 1-h static PET, summed images from 50 to 60 min of the dPET were extracted. PET parameters derived from 1- and 2-h static PET including SUV (SUV1H and SUV2H), target-to-background ratio (TBR) (TBR1H and TBR2H), net influx rate (Ki), and TBRKi extracted from dPET were obtained. The detectability of TAK-active arteries was compared among different scanning methods using the generalized estimating equation (GEE) with a logistic regression with repeated measures, and the GEE with gamma distribution and log link function was used to evaluate the different study groups or scanning methods. RESULTS: Based on the disease states, 5 cases of TAK were classified as untreated and relapsed, respectively. The SUVmax on 2-h PET was higher than that on 1-h PET in the untreated patients (P < 0.05). However, no significant differences were observed in the median SUVmax between 1-h PET and 2-h PET in the relapsed patients (P > 0.05). The TBRKi was significantly higher than both TBR1H and TBR2H (all P < 0.001). Moreover, the detectability of TAK-active arteries by dPET-derived Ki was significantly higher than 1-h and 2-h PET (all P < 0.001). Significant differences were observed in Kimax, SUVmax-1H, TBR1H, and TBRKi among untreated, relapsed, and control groups (all P < 0.05). CONCLUSIONS: Absolute quantitative assessment by dPET provides an improved sensitivity and detectability in both visualization and quantification of TAK-active arteries. This elucidates the clinical significance of dPET in the early detection of active inflammation and monitoring recurrence.

Prevalence of subclinical systolic dysfunction in Takayasu's arteritis and its association with disease activity: a cross-sectional study.

BACKGROUND: Takayasu's arteritis (TA) is a vasculitis that affects the aorta and its branches and causes stenosis, occlusion, and aneurysms. Up to 60% of TA patients are associated with cardiac involvement which confers a poor prognosis. Global longitudinal strain (GLS) analysis is an echocardiographic technique that can detect the presence of subclinical systolic dysfunction. Hence, this study aimed to describe the prevalence of subclinical systolic dysfunction in patients with TA using the GLS method and to correlate this finding with disease activity using the ITAS-2010 (Indian Takayasu Activity Score). METHODS: Thirty patients over 18 years of age who met the American College of Rheumatology (ACR) 1990 criteria for TA were included. The sample was submitted for medical record review, clinical and echocardiographic evaluation, and application of ITAS-2010. The cutoff for systolic dysfunction was GLS > - 20%. RESULTS: Of the 30 patients analyzed, 25 (83.3%) were female, and the mean age was 42.6 years (± 13.2). The median time since diagnosis was 7.5 years [range, 3-16.6 years], and the type V angiographic classification was the most prevalent (56.7%). Regarding echocardiographic findings, the median ejection fraction (EF) was 66% [61-71%] and the GLS was - 19.5% [-21.3 to -15.8%]. Although half of the participants had reduced GLS, only two had reduced EF. Eleven patients (33.%) met the criteria for activity. An association was found between disease activity and reduced GLS in eight patients (P = 0.02) using the chi-square test. CONCLUSION: GLS seems to be an instrument capable of the early detection of systolic dysfunction in TA. The association between GLS and disease activity in this study should be confirmed in a study with a larger sample size.