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.

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.

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.

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.

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.

Enfermedad de Takayasu: serie de casos / Takayasu's disease: case series

Introducción: La arteritis de Takayasu se trata de una vasculitis de grandes vasos, crónica y granulomatosa caracterizada por la presencia de estenosis y aneurismas en la aorta y sus ramas principales. Se produce inflamación. vascular que causa dolor. Las pruebas de imagen como el PET-TC han ganado importancia en el diagnóstico. El tratamiento se realiza con inmunosupresores, pudiendo usar fármacos biológicos en casos refractarios. Material y métodos: Estudio descriptivo retrospectivo de 3 casos de enfermedad de Takayasu. Resultados: Presentamos 3 casos atípicos de vasculitis de Takayasu que fueron diagnosticados mediante PET-TC y que evolucionaron de forma diferente, requiriendo 2 de ellos tratamiento con fármacos biológicos. Conclusión: Pruebas de imagen como el PET-TC permiten un diagnóstico temprano y un seguimiento de la enfermedad. Con el uso de fármacos biológicos podemos controlar los casos refractarios. (AU)

Introduction: Takayasu's arteritis is a chronic granulomatous vasculitis of large vessels characterized by the presence of stenosis and aneurysms in the aorta and its mainbranches. Vascular inflammation occurs that causes pain. Imaging tests such as PET-CT have gained importance in diagnosis. The treatment is carried out with immunosuppressants, being able to use biological drugs in refractory cases. Material and methods: Retrospective descriptive study of 3 cases of Takayasu's disease. Results: We present 3 atypical cases of Takayasu vasculitis that were diagnosed by PET-CT and that evolved differently, requiring 2 of them treatment with biological drugs. Conclusion: Imaging tests such as PET-CT allow early diagnosis and monitoring of the disease. With the use of biological drugs we can control refractory cases. (AU)

Takayasu arteritis, an atypical presentation as chronic kidney disease.

Takayasu arteritis, also known as "pulseless disease" usually affects major vessels like aorta and its branches, pulmonary arteries, and renal arteries. Hypertension is the common presentation. Chronic kidney disease involvement is less common. Only a few chronic kidney disease cases are reported so far in Takayasu arteritis. We discuss a case of a young female who presented with accelerated hypertension with chronic kidney disease with preserved peripheral pulses. The diagnosis was confirmed by Computed tomography aortic angiogram, which showed diffuse circumferential thickening, multifocal ectasia, and aneurysmal dilatation with few saccular outpouchings of the aortic arch, descending thoracic and abdominal aorta (Type V). The patient was treated with steroids, antihypertensives, antiplatelet, and hemodialysis.

A case of aortic root replacement for annuloaortic ectasia due to Takayasu's arteritis in active inflammatory phase.

A 39-year-old man was admitted with acute heart failure due to severe aortic regurgitation induced by annuloaortic ectasia associated with Takayasu's arteritis. Because of the active inflammatory phase associated with Takayasu's arteritis, surgery is typically performed following immune suppression by steroid therapy. Herein, we report a favorable recovery in the active inflammatory phase. Steroid therapy was initiated shortly following surgery. The decision to perform aortic root replacement without prior steroid therapy was made because the patient's risk of subsequent heart failures was deemed high and was complicated by other comorbidities.

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.

The performance of duplex ultrasonography for the assessment of renal artery stenosis in Takayasu's arteritis patients.

BACKGROUND: This study intends to analyze the hemodynamic parameters of the renal artery in patients with Takayasu's arteritis (TAK) to explore the diagnostic efficacy of duplex ultrasonography in assessing the involved renal artery in TAK patients. METHODS: One hundred fifteen TAK patients with 314 renal arteries were retrospectively analyzed, who were admitted to Peking Union Medical College Hospital between 2017 and 2022. These patients underwent both renal artery ultrasonography and angiography within a 4-week period. Specifically, the study compared seven ultrasonic parameters across groups categorized by the severity of renal artery stenosis (RAS), including noninvolvement, < 50% stenosis, 50-69% stenosis, and 70-99% stenosis. Receiver operating characteristic (ROC) curves were employed to determine the optimal threshold values for renal artery peak systolic velocity (RPSV), renal-aortic PSV ratio (RAR), and renal-interlobar PSV ratio (RIR) in order to diagnose various degrees of RAS in TAK patients. RESULTS: Statistically significant differences were observed in RAR and RIR among the four groups (all P < 0.05). However, no statistically significant differences were found in RPSV and AT between the moderate stenosis group (50-69% stenosis) and the severe stenosis group (70-99% stenosis). The discrimination of interlobar PSV (IPSV) and interlobar RI (IRI) was not significant, and IEDV did not show statistical significance among the four groups. For TAK patients, the recommended thresholds of RPSV for the diagnosis of renal artery involvement (RAI), ≥ 50% RAS, and ≥ 70% RAS were determined to be 143 cm/s, 152 cm/s, and 183 cm/s, respectively. The sensitivities, specificities, and accuracies of these thresholds were all found to be greater than 80%. Additionally, the optimal thresholds of RIR for detecting RAI, ≥ 50% RAS, and ≥ 70% RAS were determined to be 4.6, 5.6, and 6.4, respectively, with satisfactory diagnostic efficiencies. The areas under the curve (AUCs) for RPSV and RIR were calculated to be 0.908 and 0.910, respectively, for the diagnosis of ≥ 50% RAS, and 0.876 and 0.882 for the diagnosis of ≥ 70% RAS. When the aortic PSV is greater than or equal to 140 cm/s, the RAR exhibits inadequate diagnostic efficacy. Conversely, when the aortic PSV is less than 140 cm/s, a RAR value of 2.2 or higher can be employed as the diagnostic threshold for identifying RAS of 70% or greater, with a sensitivity of 84.00%, specificity of 89.93%, and an overall accuracy of 89.08%. CONCLUSION: In the present study, it has been demonstrated that RPSV and RIR possess substantial diagnostic value as ultrasonic parameters for diagnosing RAS in TAK patients. Furthermore, when assessing the diagnostic efficacy of RAR, it is crucial to consider the severity of aortic stenosis as a determining factor.

Ascending Aortic Coarctation - an Atypical Location in a Non-Takayasu Arteritis Female Patient.

Coarctation of the aorta is a well-known congenital cardiovascular disorder that typically occurs within proximity to the ductus arteriosus. The ascending aorta, distal descending aorta, and abdominal aorta are segments which are prone to development of an atypical coarctation. The etiologies of atypical cases are usually associated with various types of vasculitis syndromes or underlying genetic disorders. In this report, we present a 24-year-old female patient with an ascending aortic coarctation which developed secondary to an atherosclerotic process.

Percutaneous transluminal pulmonary angioplasty for Takayasu arteritis-associated pulmonary hypertension: A single-arm meta-analysis.

BACKGROUND: The efficacy and safety of percutaneous transluminal pulmonary angioplasty (PTPA) for Takayasu arteritis-associated pulmonary hypertension (TA-PH) remain unclear. OBJECTIVES: To examine the efficacy and safety of PTPA in TA-PH. METHODS: PubMed, Embase, and the Cochrane Central Register of Controlled Trials Library were searched from inception to August 18, 2022, for articles investigating the efficacy and safety of PTPA for TA-PH. The primary efficacy outcomes were pulmonary vascular resistance (PVR) changes from baseline to re-evaluation and 6-minute walking distance (6MWD). The safety outcome was procedure-related complications. RESULTS: Five articles comprising 104 patients with TA-PH who underwent PTPA were included. The scores of article quality, as assessed using the methodological index for nonrandomized studies tool, were high, ranging from 13 to 15 points. The pooled treatment effects of PVR (weighted mean difference [WMD]: -4.8 WU; 95% confidence interval [CI]: -6.0 to -3.5 WU; I2 = 0.0%), 6MWD (WMD: 101.9 m; 95% CI: 60.3-143.6 m; I2 = 70.4%) significantly improved. Procedure-related complications, which predominantly present as pulmonary artery injury and pulmonary injury, occurred in 32.0% of the included patients. Periprocedural death occurred in one patient (1.0%, 1/100). CONCLUSIONS: Patients with TA-PH could benefit from PTPA in terms of hemodynamics and exercise tolerance, at the expense of procedure-related complications. PTPA should be encouraged to enhance the treatment response in TA-PH. These findings need to be confirmed by further studies, ideally, randomized controlled trials. REGISTRATION: PROSPERO CRD42022354087.

Takayasu's arteritis as an incidental finding in a patient with celiac disease: the importance of positron emission computed tomography.

BACKGROUND: Takayasu arteritis is a large-vessel vasculitis which affects large-caliber elastic arteries, primarily the aorta and its main branches. It mainly affects women between 20-30 years, so it is rare in children. CASE REPORT: We describe the case of a 15-year-old female who was followed up since she was 9 years old due to celiac disease. At the age of 13, anaemia of chronic disorders associated to elevated C-reactive protein and erythrocyte sedimentation were detected. The patient remained asymptomatic. After excluding other diseases, we requested a positron emission computed tomography (PET-CT); lesions compatible with large-vessel vasculitis were detected. Cardiology evaluation showed an aneurysm in the right coronary artery. Angio-CT suggested Takayasu type III arteritis. CONCLUSIONS: The delay in the diagnosis of Takayasu arteritis in pediatric patients is quite common. In this case, we have found phase II lesions, with no previous phase I symptoms. However, PET-CT allowed the diagnosis of vasculitis, key to the diagnosis of the patient.

INTRODUCCIÓN: La arteritis de Takayasu es una vasculitis de grandes vasos que afecta a las arterias elásticas de gran calibre, fundamentalmente la aorta y sus ramas principales. Ocurre frecuentemente en mujeres entre los 20-30 años, por lo que es muy infrecuente en la edad pediátrica. CASO CLÍNICO: Se presenta el caso de una paciente de 15 años en seguimiento desde los 9 años por enfermedad celiaca. A los 13 años se detectó anemia de trastornos crónicos, elevación de proteína C reactiva y velocidad de sedimentación globular. La paciente permaneció en todo momento asintomática. Tras descartar otros procesos, se solicitó tomografía computarizada por emisión de positrones (PET-TC), donde se detectaron lesiones compatibles con vasculitis de grandes vasos. La valoración por Cardiología evidenció un aneurisma en coronaria derecha. Se realizó angio-TC, que sugirió arteritis de Takayasu tipo III. CONCLUSIONES: Es frecuente un retraso en el diagnóstico de la arteritis de Takayasu en los pacientes pediátricos. En este caso se encontraron lesiones de la fase II sin la presencia de síntomas correspondientes a la fase I. El PET-TC permitió el diagnóstico de vasculitis, clave para el diagnóstico de la paciente.

The Role of Imaging in Diagnosis and Monitoring of Large Vessel Vasculitis.

Technological advances and increased recognition of the prevalence and implications of large vessel vasculitis have led to robust research into various imaging techniques. Although there is still debate about which modality to choose in specific clinical scenarios, Ultrasound, PET/CT, MRI/A, and CT/A offer complementary information regarding diagnosis, disease activity, and vascular complication monitoring. Recognition of the strengths and limitations of each technique is important for appropriate application in clinical practice.