Search

Objectives: To investigate the clinical and image characteristics of primary systemic vasculitis-associated optic neuritis patients. Methods: This is a retrospective study. The patients clinically diagnosed with primary system vasculitis-induced optic neuritis were recruited from March 2013 to December 2023. All cases received orbital magnetic resonance imaging scans were analyzed. The ocular findings, systemic manifestations, laboratory data and prognosis were reviewed retrospectively. In addition, the related literature was reviewed. Results: Fourteen patients (21 eyes), including 10 men and 4 women, were enrolled in this study. The ages ranged from 30 to 86 years in this cohort. Orbits MRI detects the enlargement and/or enhancement of the optic nerve. Cases 1–5 reported a confirmed diagnosis of Takayasu's arteritis, and cases 6–8 had giant cell arteritis. Cases 9–13 were antineutrophil cytoplasmic antibody-associated vasculitis. Case 14 was Cogan's syndrome. Mult organs and tissues, such as the kidneys, heart, paranasal sinuses, meninges, and respiratory system, were involved. In all of the 14 involved patients, the disease onset was either during the fall or winter season. There were no or only slight improvements in visual activity after conventional therapies. Conclusions: The autoantibodies' attack on the optic nerve, ischemic damage, or destruction of the blood–brain barrier may be the potential pathogenesis of vasculitis-associated optic neuritis. Even with prompt and aggressive clinical interventions, the prognosis remains unsatisfactory. [ABSTRACT FROM AUTHOR]

Background Takayasu's arteritis is an infrequent manifestation of vasculitis affecting the aorta and its primary branches with numerous symptoms. This report details a rare case wherein a patient developed interventricular septal dissection following aortic valve replacement. Case summary A middle-aged woman diagnosed with Takayasu's arteritis previously underwent aortic valve replacement with a mechanical valve owing to severe aortic regurgitation. Subsequently, she received a redo aortic valve replacement following an episode of prosthetic valve infective endocarditis with paravalvular leak. Heart failure symptoms emerged during follow-up, revealing aortic root dissection extending into the interventricular septum, causing significant prosthetic valve movement. A Trido Bentall operation and interventricular septum repair were performed, and the patient recovered smoothly. Discussion Interventricular dissection, although uncommon, may be due to factors such as infection, myocardial infarction, congenital anomalies, trauma, or post-surgical shear stress. Timely diagnosis is imperative to prevent life-threatening complications; surgery remains the primary treatment. The present case report describes a rare presentation that was successfully managed through a Bentall operation and underscores the necessity of prompt intervention in treating this condition. [ABSTRACT FROM AUTHOR]

Background: Pulmonary artery involvement (PAI) is not rare in Takayasu arteritis (TA). Persistently elevated pulmonary arterial pressure in TA‐PAI patients leads to pulmonary hypertension (PH), and eventually cardiac death. Thus, the early detection of right ventricular dysfunction before the onset of PH is important. Purpose: To explore the potential of right ventricular global peak longitudinal and circumferential strain (RVGLS and RVGCS, respectively) in detecting right ventricular myocardial damage in TA‐PAI patients without PH. Study Type: Retrospective. Population: One hundred and six TA patients (39.6 ± 13.9 years), of whom 52 were non‐PAI and 54 were PAI patients (36 without PH and 18 with PH), along with 58 sex‐ and age‐matched healthy volunteers (HVs) (36.7 ± 13.2 years). The involved arteries were validated by aorta magnetic resonance (MR) angiography and pulmonary artery computed tomography angiography. Field Strength/Sequence: 3 T/Cine imaging sequence with a steady‐state free precession readout. Assessment: Cardiac MRI‐derived parameters measured by two radiologists independently were compared among HVs, and TA patients with and without PAI. In addition, these indices were further compared among HVs, and TA‐PAI patients with and without PH. Statistical Tests: Student's t test, one‐way ANOVA analysis, Pearson and Spearman correlation analysis, and reproducibility analysis. A P‐value of <0.05 was considered statistically significant. Results: Although the TA‐PAI patients without PH had a similar RV ejection fraction (RVEF) with HV (P = 0.348), RVGLS (non‐PH 20.6 ± 3.7% vs. HV 24.0 ± 3.1%) was significantly lower and RVGCS (non‐PH 14.8 ± 3.9% vs. HV 13.0 ± 2.7%) higher. The TA‐PAI patients with PH had significantly poorer RVGLS (PH 13.5 ± 3.8% vs. non‐PH 20.6 ± 3.7%) and RVGCS (PH 10.9 ± 3.2% vs. non‐PH 14.8 ± 3.9%) than those without PH. Data Conclusion: Right ventricular dysfunction was detected in the TA‐PAI patients without PH. MR‐feature tracking may be an effective method for detecting early cardiac damage in the TA‐PAI patients without PH. Level of Evidence: 3 Technical Efficacy: Stage 3 [ABSTRACT FROM AUTHOR]

Diseases of the carotid arteries can be classified into different categories based on their origin. Atherosclerotic carotid disease remains the most encountered arterial wall pathology. However, other less-common non-atherosclerotic diseases can have detrimental clinical consequences if not appropriately recognized. The underlying histological features of each disease process may result in imaging findings that possess features that are obvious of the disease. However, some carotid disease processes may have histological characteristics that manifest as non-specific radiologic findings. The purpose of this manuscript is to review various non-atherosclerotic causes of carotid artery disease as well as their histologic-radiologic characteristics to aid in the appropriate recognition of these less-commonly encountered pathologies. [ABSTRACT FROM AUTHOR]

Takayasu's arteritis (TAK) manifests as an insidiously progressive and debilitating form of granulomatous inflammation including the aorta and its major branches. The precise etiology of TAK remains elusive, with current understanding suggesting an autoimmune origin primarily driven by T cells. Notably, a growing body of evidence bears testimony to the widespread effects of B cells on disease pathogenesis and progression. Distinct alterations in peripheral B cell subsets have been described in individuals with TAK. Advancements in technology have facilitated the identification of novel autoantibodies in TAK. Moreover, emerging data suggest that dysregulated signaling cascades downstream of B cell receptor families, including interactions with innate pattern recognition receptors such as toll-like receptors, as well as co-stimulatory molecules like CD40, CD80 and CD86, may result in the selection and proliferation of autoreactive B cell clones in TAK. Additionally, ectopic lymphoid neogenesis within the aortic wall of TAK patients exhibits functional characteristics. In recent decades, therapeutic interventions targeting B cells, notably utilizing the anti-CD20 monoclonal antibody rituximab, have demonstrated efficacy in TAK. Despite the importance of the humoral immune response, a systematic understanding of how autoreactive B cells contribute to the pathogenic process is still lacking. This review provides a comprehensive overview of the biological significance of B cell-mediated autoimmunity in TAK pathogenesis, as well as insights into therapeutic strategies targeting the humoral response. Furthermore, it examines the roles of T-helper and T follicular helper cells in humoral immunity and their potential contributions to disease mechanisms. We believe that further identification of the pathogenic role of autoimmune B cells and the underlying regulation system will lead to deeper personalized management of TAK patients. We believe that further elucidation of the pathogenic role of autoimmune B cells and the underlying regulatory mechanisms holds promise for the development of personalized approaches to managing TAK patients. [ABSTRACT FROM AUTHOR]

In this study, we aimed to evaluate LIF levels and its possible relationship with disease activity in patients with Takayasu's (TAK) and Giant cell arteritis (GCA) patients. 23 Takayasu's arteritis, 9 Giant cell arteritis patients and 25 healthy volunteers were included in the study. Serum LIF levels were measured ELISA. The mean age of Giant cell arteritis patients was statistically significantly higher than the other groups (p < 0.001). The rate of women was found to be higher in Takayasu's arteritis (p = 0.021). When healthy control, patients with GCA and Takayasu arteritis were compared, there was a difference in LIF values (p = 0.018). In subgroup analyzes, LIF values were found to be higher in GCA patients compared to healthy controls (p < 0.05). There was no statistically significant correlation between LIF and CRP (Rho = −0.038, p = 0.778), ESR (Rho = 0.114, p = 0.399) and ITAS (Rho = −0.357, p = 0.094). While CRP was statistically significantly higher in patients with disease activity (p = 0.003), there was no statistically significant difference between patients in terms of ESR and LIF values. While there was a statistically significant relationship between CRP (OR = 1.19 [1.03–1.37], p = 0.018) and disease activity in univariate analyses, no statistically significant variable was found in multivariable analyses. LIF values were significantly higher in patients with Giant cell arteritis compared to healthy controls. [ABSTRACT FROM AUTHOR]

Background: Takayasu's arteritis (TA) is a rare medical disorder of probable immune aetiology which complicates pregnancy. It affects younger women predominantly and may have a significant impact on maternal and foetal health. Aim: To study the maternal and perinatal outcomes among pregnant women with TA, attending a dedicated obstetric hospital in South India. Method: It is a retrospective observational study done at Fernandez Hospital, Hyderabad, with 10,000 births per annum. All women with TA-complicating pregnancies who were birthed from 2005 to 2022 were included. American College of Rheumatology criteria were used for the diagnosis of TA. The primary outcome of interest was to analyse the type of TA, maternal and foetal outcomes in pregnant women with TA. The secondary outcome of interest was to compare foetal outcomes between pregnancies managed at Fernandez Hospital (group A) versus previous pregnancies managed elsewhere (group B). Results: During the study period, there were 119,053 births of which 38 women had TA, with a total of 92 pregnancies, of which 59 pregnancies were in group A and 33 pregnancies in group B. Type 4 was the commonest seen in 44% of women. Hypertensive disorders of pregnancy were seen in 67.7% of women. The live birth rate was 84%, miscarriages were 10% and 5% of women had stillbirths. Caesarean section rate was 59.3%. There was one maternal mortality. Conclusions: TA should be considered in young women with secondary hypertension. Maternal and foetal outcomes are good with appropriate diagnosis, treatment and management of complications. [ABSTRACT FROM AUTHOR]

Takayasu’s arteritis (TA), also known as Pulseless disease, Aortic arch syndrome, Occlusive thromboaortopathy, or Aortoarteritis, is an inflammatory pan-endarteritis leading to thrombotic occlusion and aneurysm of major arteries. Major challenges for anesthesia in patients with TA involve severe uncontrolled hypertension, end-organ dysfunction, stenosis of major blood vessels, and difficulties in monitoring arterial blood pressure (BP). The cardiovascular complications attributed to the disease can be seriously enhanced during pregnancy. This case series describes the successful anesthetic management of three cases of TA presenting for lower segment cesarean section (LSCS). Each parturient had a unique presentation, necessitating a specific strategy. A sequential combined spinal epidural is an attractive option; however, each case requires thorough evaluation and appropriate management.

Over the past decades, fundamental insights have been gained to establish the pivotal role of imaging in the diagnosis of large-vessel vasculitis, including giant cell arteritis (GCA) and Takayasu’s arteritis (TAK). A deeper comprehension of imaging modalities has prompted earlier diagnosis leading to expedited treatment for better prognosis. The European Alliance of Associations in Rheumatology (EULAR) recommended in 2023 that ultrasound should be the initial imaging test in suspected GCA, and Magnetic Resonance Imaging (MRI) remains the first-line imaging modality in suspected TAK. We summarize the recent advances in diagnostic imaging in large vessel vasculitis, highlighting use of combination imaging modalities, and discuss progress in newer imaging techniques such as contrast-enhanced ultrasound, shear wave elastography, ocular ultrasound, ultrasound biomicroscopy, integration of Positron Emission Tomography (PET) with MRI, novel tracer in PET, black blood MRI, orbital MRI, and implementation of artificial intelligence (AI) to existing imaging modalities. Our aim is to offer a perspective on ongoing advancements in imaging for the diagnosis of GCA and TAK, particularly innovative technology, which could potentially boost diagnostic precision.

Takayasu’s arteritis (TA) is a granulomatous vasculitis that involves the aortic artery and its branches, resulting in stenosis, occlusion, and aneurysmal dilatation. Cardiovascular involvement is one of the main complications and a major cause of mortality in these patients. Herein, we describe the case of a woman with TA who presented with severe acute heart failure secondary to myocarditis and responded well to immunosuppressive therapy.

Takayasu's arteritis (TAK) is a chronic large vessel vasculitis that involves the aorta and its primary branch arteries, which can result in limb ischemia and organ damage. A clinical practice guideline has been newly developed under the leadership of the National Clinical Medical Research Center for Dermatologic and Immunologic Diseases. This guideline provides evidence-based recommendations on 11 key issues in the management of TAK, covering the diagnosis, clinical evaluation, medical treatment, antithrombotic therapy, surgical intervention as well as pregnancy, and aims to guide the diagnosis and treatment of TAK in clinical practice and improve the outcomes of patients in China. This article interprets the key points of this guideline, in order to promote its application.

Abstract Introduction Takayasu’s arteritis (TAK) patients are at an elevated risk of metabolic syndrome and cardiovascular diseases (CVD). Currently, there are no well-validated biomarkers to assess this risk in this population. Previous research in different cohorts has linked serum levels of osteoprotegerin (OPG) and its polymorphisms to accelerated atherosclerosis and a marker of poor prognosis in CVD. Thus, we assessed this protein as a potential biomarker of CVD in TAK patients. Objectives To evaluate the serum levels of OPG and its SNPs (single nucleotide polymorphisms) in TAK patients and healthy controls, and to associate these parameters with clinical data. Methods This bicentric cross-sectional study included TAK patients who were compared with healthy individuals (control group). The serum levels of OPG and the frequency of OPG SNPs [1181G > C (rs2073618), 245 A > C (rs3134069), 163T > C (rs3102735), and 209 C > T (rs3134070)] were compared between the both groups and associated with clinical data. Results In total, 101 TAK patients and 93 controls were included in the study. The serum levels of OPG (3.8 ± 1.9 vs. 4.3 ± 1.8pmol/L, respectively; P = 0.059), and its four polymorphisms were comparable between both groups. In an additional analysis of only TAK patients, serum OPG levels and its four genes were not associated with any CVD parameters, except for higher OPG levels among patients without dyslipidemia. Conclusion No significant differences were observed in serum OPG levels or in the genotype frequencies of OPG SNPs between the patient and control groups. Similarly, no correlation was found between laboratory parameters and clinical data on CVD risk in TAK patients.

Introduction: Renal artery stenosis (RAS) is a clinical picture that is evaluated as a renovascular disorder and includes many diseases in its etiology. The primary purpose of the present study was to reveal the etiology of cases followed up in the nephrology outpatient clinic due to RAS; to describe their demographic, clinical, and radiological features; and investigate their treatment and prognosis. The second aim of the study was to investigate the relationship between isolated RAS cases that cannot be attributed to any etiological cause and Takayasu’s arteritis. Methods: Patients were included in retrospectively examining all patient files that were registered between January 1996 and 2018. Demographic data of the patients, date of diagnosis, initial physical examination findings, comorbid diseases, imaging findings, interventional and medical treatments, need for kidney replacement therapy, and time to hemodialysis/transplantation were recorded. Results: Out of the 17427 (8800 M/8627 F) patients, a total of 134 (70 M/64 F) patients aged over 18 years with RAS were included in the study; 60 (55%) patients had atherosclerotic RAS, whereas 23 (21.1%) patients were diagnosed with vasculitis. In total, 16 patients (13.5%) died with a mean of 6±3.4 years after admission. It was found that advanced age, low GFR at diagnosis, and small kidneys are significant and poor prognostic factors. Conclusion: A more systematic approach model can be developed in terms of applying “maximal medical treatment”, which is our approach that we can currently describe as incomplete in cases of RAS with an early atherosclerotic process.

Background: It has been well documented that Takayasu arteritis (TAK) and ulcerative colitis (UC) coexist in the same patients. HLA-B*52 characterizes the co-occurrence, which is one of the common genetic features between these two diseases, indicating shared underlying pathologic mechanisms. Anti-integrin αvβ6 antibody (Ab) is present in sera of UC patients in a highly specific manner. We investigated if there were any associations between anti-integrin αvβ6 Ab and TAK, considering the risk HLA alleles. Methods: A total of 227 Japanese TAK patients were recruited in the current study and their serum samples were subjected to measurement of anti-integrin αvβ6 Ab by ELISA. The clinical information, including the co-occurrence of UC, was collected. The HLA allele carrier status was determined by Luminex or genotype imputation. Results: The information about the presence of UC was available for 165 patients, among which eight (4.84%) patients had UC. Anti-integrin αvβ6 antibody was identified in 7 out of 8 TAK subjects with UC (87.5%) while only 5 out of 157 (3.18%) TAK subjects without UC had the antibody (OR 121, p=7.46×10-8). A total of 99 out of 218 (45.4%) patients were HLA-B*52 carriers. There was no significant association between the presence of anti-integrin αvβ6 Ab and HLA-B*52 carrier status in those without UC (OR 2.01, 95% CI 0.33-12.4, p = 0.189). Conclusions: The prevalence of anti-integrin αvβ6 Ab was high in TAK patients with UC, but not in the absence of concomitant UC. The effect of HLA-B*52 on anti-integrin αvβ6Ab production would be minimal. [ABSTRACT FROM AUTHOR]

Introduction: Renal artery stenosis (RAS) is a clinical picture that is evaluated as a renovascular disorder and includes many diseases in its etiology. The primary purpose of the present study was to reveal the etiology of cases followed up in the nephrology outpatient clinic due to RAS; to describe their demographic, clinical, and radiological features; and investigate their treatment and prognosis. The second aim of the study was to investigate the relationship between isolated RAS cases that cannot be attributed to any etiological cause and Takayasu's arteritis. Methods: Patients were included in retrospectively examining all patient files that were registered between January 1996 and 2018. Demographic data of the patients, date of diagnosis, initial physical examination findings, comorbid diseases, imaging findings, interventional and medical treatments, need for kidney replacement therapy, and time to hemodialysis/transplantation were recorded. Results: Out of the 17427 (8800 M/8627 F) patients, a total of 134 (70 M/64 F) patients aged over 18 years with RAS were included in the study; 60 (55%) patients had atherosclerotic RAS, whereas 23 (21.1%) patients were diagnosed with vasculitis. In total, 16 patients (13.5%) died with a mean of 6±3.4 years after admission. It was found that advanced age, low GFR at diagnosis, and small kidneys are significant and poor prognostic factors. Conclusion: A more systematic approach model can be developed in terms of applying "maximal medical treatment", which is our approach that we can currently describe as incomplete in cases of RAS with an early atherosclerotic process. [ABSTRACT FROM AUTHOR]

Systemic vasculitides are a rare and complex group of diseases that can affect multiple organ systems. Clinically, presentation may be vague and non-specific and as such, diagnosis and subsequent management are challenging. These entities are typically classified by the size of vessel involved, including large-vessel vasculitis (giant cell arteritis, Takayasu's arteritis, and clinically isolated aortitis), medium-vessel vasculitis (including polyarteritis nodosa and Kawasaki disease), and small-vessel vasculitis (granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis). There are also other systemic vasculitides that do not fit in to these categories, such as Behcet's disease, Cogan syndrome, and IgG4-related disease. Advances in medical imaging modalities have revolutionized the approach to diagnosis of these diseases. Specifically, color Doppler ultrasound, computed tomography and angiography, magnetic resonance imaging, positron emission tomography, or invasive catheterization as indicated have become fundamental in the work up of any patient with suspected systemic or localized vasculitis. This review presents the key diagnostic imaging modalities and their clinical utility in the evaluation of systemic vasculitis. [ABSTRACT FROM AUTHOR]

Chronic primary systemic vasculitis (PSV) comprises a group of heterogeneous diseases that are broadly classified by affected blood vessel size, clinical traits and the presence (or absence) of anti-neutrophil cytoplasmic antibodies (ANCA) against proteinase 3 (PR3) and myeloperoxidase (MPO). In small vessel vasculitis (SVV), ANCA are not present in all patients, and they are rarely detected in patients with vasculitis involving medium (MVV) and large (LVV) blood vessels. Some studies have demonstrated that lysosome-associated membrane protein-2 (LAMP-2/CD107b) is a target of ANCA in SVV, but its presence and prognostic value in childhood MVV and LVV is not known. This study utilized retrospective sera and clinical data obtained from 90 children and adolescents with chronic PSV affecting small (SVV, n = 53), medium (MVV, n = 16), and large (LVV, n = 21) blood vessels. LAMP-2-ANCA were measured in time-of-diagnosis sera using a custom electrochemiluminescence assay. The threshold for seropositivity was established in a comparator cohort of patients with systemic autoinflammatory disease. The proportion of LAMP-2-ANCA-seropositive individuals and sera concentrations of LAMP-2-ANCA were assessed for associations with overall and organ-specific disease activity at diagnosis and one-year follow up. This study demonstrated a greater time-of-diagnosis prevalence and sera concentration of LAMP-2-ANCA in MVV (52.9% seropositive) and LVV (76.2%) compared to SVV (45.3%). Further, LAMP-2-ANCA-seropositive individuals had significantly lower overall, but not organ-specific, disease activity at diagnosis. This did not, however, result in a greater reduction in disease activity or the likelihood of achieving inactive disease one-year after diagnosis. The results of this study demonstrate particularly high prevalence and concentration of LAMP-2-ANCA in chronic PSV that affects large blood vessels and is seronegative for traditional ANCA. Our findings invite reconsideration of roles for autoantigens other than MPO and PR3 in pediatric vasculitis, particularly in medium- and large-sized blood vessels. [ABSTRACT FROM AUTHOR]

Background: Detailed anatomical knowledge of congenital abnormalities of the lungs is essential for proper evaluation and treatment of respiratory diseases, identification of bronchopulmonary segments during lobectomies and surgical resection, and identification and interpretation of their variable radiological imaging. Aim: Accordingly, the aim of this study was designed to evaluate the prevalence of congenital abnormal shapes of lungs in relation to abnormal lobes and fissures. Materials and Methods: This study was carried out on 270 isolated lung specimens (right lungs -- 135 and left lungs -- 135) from 135 formalin-embalmed middle-aged human cadavers irrespective of gender. Results: Out of 270 lung specimens following congenital abnormalities were reported -- (1) three lungs (1.1%) had uunusual different abnormal shapes, such as typical snail shell-shaped right lung with single abnormal circular fissure, bearded axe-shaped left lung with huge cardiac notch, and single-lobed classical pointed hat-shaped right lung, (2) one right lung (0.37%) and 9 left lungs (6.67%) showed complete absence of fissures, (3) among 9 single-lobed left lungs, one had split apex separated by a left subclavian groove with the absence of lingula and cardiac notch, (4) cardiac notch and ligula were noted absently in 3 left lungs (2.36%), and (5) one right lung (0.37%) showed five lobes with complete one oblique and three incomplete horizontal fissures. Conclusion: To the best of our knowledge, such abnormalities of the lungs have not been cited in the recent medical literature. Awareness of these abnormalities is necessary to avoid complications during various radiodiagnostic procedures or cardiothoracic surgeries. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Takayasu's arteritis is a rare type of vasculitis with severe complications like stroke, ischemic heart disease, pulmonary hypertension, secondary hypertension, and aneurysms. Diagnosis is achieved using clinical and angiographic criteria. Treatment is medical and surgical, but unfortunately, the outcome is limited. Case presentation: A 34-year-old Caucasian woman had an ischemic stroke (2009). She was diagnosed with Takayasu's arteritis and received treatment with methotrexate, prednisolone, and antiplatelet agents, with a mild improvement in clinical state. After 6 years (2015), she experienced an ascending aorta aneurysm, pulmonary hypertension, and mild aortic regurgitation. Surgical treatment solved both the ascending aorta aneurysm and left carotid artery stenosis (ultrasound in 2009 and computed tomography angiogram in 2014). Morphopathology revealed a typical case of Takayasu's arteritis. Tumor necrosis factor inhibitors (TNF inhibitors) were prescribed with methotrexate. At 48 years old (2023), she developed coronary heart disease (angina, electrocardiogram); echocardiography revealed severe pulmonary hypertension, and angiography revealed normal coronary arteries, abdominal aorta pseudoaneurysm, and arterial–venous fistula originating in the right coronary artery with drainage in the medium pulmonary artery. The patient refused surgical/interventional treatment. She again received TNF inhibitors, methotrexate, antiplatelet agents, and statins. Conclusions: This case report presented a severe form of Takayasu's arteritis. Our patient had multiple arterial complications, as previously mentioned. She received immunosuppressive treatment, medication targeted to coronary heart disease, and surgical therapy. [ABSTRACT FROM AUTHOR]

Takayasu's arteritis (TAK) is a chronic granulomatous inflammatory disease that involves aorta and its primary branches. It is characterized by wall thickening, stenosis/obliteration or aneurysm formation of the involved arteries. In order to standardize the diagnosis and treatment of TAK in China, a clinical practice guideline with an evidence-based approach is developed under the leadership of National Clinical Medical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID). Eleven recommendations for 11 clinical questions that are important to the diagnosis and treatment of TAK are developed based on the latest evidence and expert opinions combined with real clinical practice in China. [ABSTRACT FROM AUTHOR]

Takayasu's arteritis is a primary systemic vasculitis that affects predominantly large vessels, affecting the aorta and its major branches. We report a case of adolescent female patient who initially experienced numbness and weakness in her limbs, subsequently developing severe hypertension. Physical examination revealed uneven blood pressure in the limbs and a murmur in the auscultation area of the abdominal aorta without decreased pulses. Auxiliary examinations revealed diffuse blood vessel stenosis, leading to the diagnosis of Takayasu's arteritis. One month later, the patient was diagnosed with multiple cerebral hemorrhages following sudden impairment of limb movement. Digital subtraction angiography did not reveal any evident vascular malformations or aneurysms. Following surgery and biologic intervention with tocilizumab, the patient's condition improved, with no new bleeding episodes and stable blood pressure control achieved. We also reviewed the literature that have been previously reported with hypertensive intracerebral hemorrhage complicated by Takayasu's arteritis. We recommend that Takayasu's arteritis be taken into account when considering the hypertension in young patients. Timely vascular imaging and standardized treatment are imperative for diagnosing and managing effectively.

Large-vessel vasculitis (LVV) is a group of diseases characterized by inflammation of the aorta and its main branches, which includes giant cell arteritis (GCA), polymyalgia rheumatica (PMR), and Takayasu’s arteritis (TAK). These conditions pose significant diagnostic and management challenges due to their diverse clinical presentations and potential for serious complications. 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG-PET-CT) has emerged as a valuable imaging modality for the diagnosis and monitoring of LVV, offering insights into disease activity, extent, and response to treatment. 18F-FDG-PET-CT plays a crucial role in the diagnosis and management of LVV by allowing to visualize vessel involvement, assess disease activity, and guide treatment decisions. Studies have demonstrated the utility of 18F-FDG-PET-CT in distinguishing between LVV subtypes, evaluating disease distribution, and detecting extracranial involvement in patients with cranial GCA or PMR phenotypes. Additionally, 18F-FDG-PET-CT has shown promising utility in predicting clinical outcomes and assessing treatment response, based on the correlation between reductions in FDG uptake and improved disease control. Future research should focus on further refining PET-CT techniques, exploring their utility in monitoring treatment response, and investigating novel imaging modalities such as PET-MRI for enhanced diagnostic accuracy in LVV. Overall, 18F-FDG-PET-CT represents a valuable tool in the multidisciplinary management of LVV, facilitating timely diagnosis and personalized treatment strategies to improve patient outcomes.

Ankylosing spondylitis (AS) is a chronic inflammatory disease that mainly affects the sacroiliac joints, spine, and enthesis. Typical symptoms include stiffness and gradual functional restriction of the axial skeleton. Takayasu's arteritis (TA) is a condition characterized by chronic inflammation of blood vessels, usually affecting major vessels like the aorta and its branches. It leads to constriction, obstruction, and the development of aneurysms in the systemic and pulmonary arteries. Both AS and TA are rare inflammatory conditions, and their occurrence together in one person is even rarer. Autoimmune mechanisms are likely to have a substantial impact on the onset of TA, similar to AS. Hence, therapy involving steroids and biological medications like infliximab and tocilizumab is expected to be advantageous. In this case report, we present a 24-year-old female diagnosed with TA and AS. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To study the prevalence, risk and clinical associations of hypothyroidism among several forms of vasculitis. METHODS: Patients with GCA, Takayasus arteritis (TAK), PAN and the three forms of ANCA-associated vasculitis [AAV; granulomatosis with polyangiitis (GPA), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (EGPA)] enrolled in a prospective, multicentre, longitudinal study were included. RESULTS: The study included data on 2085 patients [63% female, 90% White] with a mean age of 54.6 years (s.d. 17.2). Diagnoses were GCA (20%), TAK (11%), PAN (5%), GPA (42%), microscopic polyangiitis (8%) and EGPA (14%). Hypothyroidism was present in 217 patients (10%) (83% female), with a mean age 59.8 years (s.d. 14.5). Age- and sex-adjusted risk of hypothyroidism was GCA, odds ratio (OR) 0.61 (95% CI 0.41, 0.90); TAK, OR 0.57 (95% CI 0.31, 1.03); PAN, OR 0.59 (95% CI 0.25, 1.38); GPA, OR 1.51 (95% CI 1.12, 2.05); microscopic polyangiitis, OR 1.81 (95% CI 1.18, 2.80) and EGPA, OR 0.82 (95% CI 0.52, 1.30). Among patients with AAV, age- and sex-adjusted risk of hypothyroidism was higher with positive MPO-ANCA [OR 1.89 (95% CI 1.39, 2.76)]. The clinical manifestations of vasculitis were similar in patients with and without hypothyroidism, except transient ischaemic attacks, which were more frequently observed in patients with GCA and hypothyroidism (12% vs 2%; P = 0.001). CONCLUSIONS: Differences in the risk of hypothyroidism among vasculitides may be due to genetic susceptibilities or immune responses. This study confirms an association of hypothyroidism with MPO-ANCA.

Autoimmune vasculitides affect the cerebral vasculature significantly in a considerable number of cases. When immunosuppressive treatments fail to prevent stenosis in cerebral vessels, treatment options for affected patients become limited. In this case series, we present four cases of pharmacoresistant vasculitis with recurrent transient ischemic attacks (TIAs) or stroke successfully treated with either extracranial-intracranial (EC-IC) bypass surgery or endovascular stenting. Both rescue treatments were effective and safe in the selected cases. Our experience suggests that cases of pharmacoresistant cerebral vasculitis with recurrent stroke may benefit from rescue revascularization in combination with maximum medical management. [ABSTRACT FROM AUTHOR]

Background Aortic aneurysm as a presenting feature in Takayasu's arteritis is very rare. Here, we report three cases of extensive thoracoabdominal aortic aneurysm in Takayasu's arteritis as initial presentation. Case summary All three cases were males and presented with complaints of abdominal pain and refractory hypertension. The diagnosis was made from the finding of thickened and calcified aortic wall, stenosis of visceral arteries, and age < 40 years at diagnosis. Case 1 was a 34 years male with aortic aneurysm extending from left subclavian artery to infrarenal aorta. He underwent endovascular repair of aneurysm by sandwich chimney technique in view of impending aneurysm rupture. Case 2, a 37 years male had aortic aneurysm from descending thoracic aorta (D4 vertebral body) to infrarenal aorta (L4 level). While being evaluated for repair, he had sudden death probably due to ruptured aneurysm. Case three, a 40 years male had aortic aneurysm extending from left subclavian artery to aortic bifurcation and stenosis of visceral arteries. He did not consent for repair and died one year later due to chronic kidney disease and related complications. Discussion Thoracoabdominal aortic aneurysm is a very rare manifestation in Takayasu's arteritis; more common in males. Endovascular repair is challenging but feasible. Long-term monitoring and repeat intervention may be needed due to young age of patients and disease progression. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

In this study, we studied the performance of the 2022 American College of Rheumatology (ACR)/ European Alliance of Associations for Rheumatology (EULAR) classification criteria for Takayasu's arteritis (TAK) as compared to the 1990 ACR classification criteria in a Chinese population. The sensitivity, specificity, positive predictive value, negative predictive value, accuracy and the area under the receiver operating characteristics curve (AUC) of the above two criteria were compared. The sensitivity (92.6%), positive predictive value (95.6%), negative predictive value (94.6%), accuracy (95.0%) and AUC (0.981) of the 2022 criteria were superior to those of the 1990 criteria (45.7%, 91.5%, 70.5%, 75.0% and 0.874, respectively), and the difference of AUC was statistically significant (Z = 5.362, P < 0.001). In addition, we included new imaging modalities in the 1990 criteria, whose sensitivity, positive predictive value, negative predictive value, accuracy and AUC were significantly improved, but still lower than those of the 2022 criteria, the difference in AUC was also statistically significant (Z = 2.023, P = 0.043). The 2022 criteria for TAK exhibited superior performance compared with the 1990 criteria and may be more appropriate for the Chinese population. Incorporating additional imaging modalities could enhance the classification performance of the 1990 criteria even further. [ABSTRACT FROM AUTHOR]

The role of 18F-fluorodeoxyglucose (FDG) positron emission tomography (18F-FDG PET) in the diagnosis of large vessel vasculitis (LVV) is well established. It permits us to assess the extent and the grade of vascular involvement and to rule out the other causes in clinical scenarios characterized by less specific symptoms. The advantages of 18F-FDG PET are far less clear in monitoring disease activity over time. Studies looking for the role of 18F-FDG PET as a potential biomarker had conflicting results and whether and when to repeat it during follow-up is based on clinical experience. A comprehensive assessment, including clinical, laboratory and morphological imaging is still required to monitor patients with large-vessel vasculitis over time. The aim of this review is to present more recent data about the utility of 18 F-FDG PET in the diagnosis and follow-up of LVV. [ABSTRACT FROM AUTHOR]

Takayasu's arteritis is a chronic, idiopathic, inflammatory disease that mainly affects large blood vessels such as the aorta and its branches, and is mostly prevalent among women younger than 40 years old. Although involvement of the central nervous system and stroke may occur during the course of the disease, it is uncommon as the initial manifestation of the patient. Here, a 58-year-old patient is presented with stroke symptoms and occlusion of the left and right carotid and left vertebral arteries since 7 years ago. The patient was subjected to laboratory examination, imaging, and additional investigations, and with Takayasu's diagnosis, she underwent treatment with corticosteroids, cyclophosphamide (and then methotrexate), and antiplatelet therapy. In addition to the occurrence of stroke as the first manifestation of the disease, the development of symptoms at the age of 51 indicated that the inflammatory process had probably started more than a decade before the symptoms.

Pulmonary artery sarcomas are rare, high-grade malignancies, primarily affecting the proximal elastic pulmonary artery and usually manifesting as tumoral impaction on imaging. Due to similar clinical and imaging findings, pulmonary artery sarcomas are frequently misdiagnosed as pulmonary thromboembolism or, occasionally, as vasculitis. Herein, we reported a case of pulmonary artery intimal sarcoma initially misdiagnosed as pulmonary thromboembolism and vasculitis due to its relatively atypical location and morphology, along with a literature review.

Takayasu’s disease (nonspecific aortoarteritis) is a granulomatous inflammation of the aorta and its main branches with a progressive course and development of severe ischemic disorders. The difficulty of diagnosis and the possibility of applying various methods of pathogenetic anti-inflammatory treatment of Takayasu’s arteritis make it expedient to study a clinical case. The analysis of a clinical case of a patient with Takayasu’s arteritis with manifestation of the disease in the form of general inflammatory syndrome and manifestations of severe cerebral ischemia due to bilateral stenotic carotid artery lesion was performed. The patient has been under observation since September 2017 up to the present time, various methods of pharmacotherapy and surgical correction were used in her therapy. The dynamics of clinical symptomatology of Takayasu’s arteritis and clinical results of step therapy with high doses of methylprednisolone, bolus administration of cyclophosphan followed by long-term oral cyclophosphan administration were analyzed. In the course of treatment, the patient underwent carotid angioplasty. Due to the unstable effect of the therapy, the patient was administered intravenous infusions of IL-6 blocker tocilizumab, which led to remission of the disease. The presented clinical case demonstrates the important diagnostic value of vascular imaging methods in early diagnosis and control of the disease course and the effectiveness of IL-6 inhibitors in achieving and maintaining remission of Takayasu’s arteritis.

The role of 18F-fluorodeoxyglucose (FDG) positron emission tomography (18F-FDG PET) in the diagnosis of large vessel vasculitis (LVV) is well established. It permits us to assess the extent and the grade of vascular involvement and to rule out the other causes in clinical scenarios characterized by less specific symptoms. The advantages of 18F-FDG PET are far less clear in monitoring disease activity over time. Studies looking for the role of 18F-FDG PET as a potential biomarker had conflicting results and whether and when to repeat it during follow-up is based on clinical experience. A comprehensive assessment, including clinical, laboratory and morphological imaging is still required to monitor patients with large-vessel vasculitis over time. The aim of this review is to present more recent data about the utility of 18 F-FDG PET in the diagnosis and follow-up of LVV.

Locked-in syndrome (LiS) is a condition of motor paralysis involving all the voluntary muscles of 4 extremities with retained consciousness and alertness. Meanwhile, Takayasu arteritis (TA) is a systemic inflammatory disease affecting large arteries, including the aorta and the branches. This disease often affects the extracranial arteries but rarely occurs in the intracranial arteries. An 18-year-old male presented with a sudden onset of decreased consciousness and bilateral weakness. The patient exhibited normal horizontal and vertical eye movements but presented with impairment of the trigeminal nerve, peripheral-type facial palsy, absence of gag reflex and tongue movement, and neck weakness. Physiological reflexes were increased bilaterally, while pathological reflexes were present on both sides. Autonomic function was impaired, and communication was impossible due to paralysis. Additionally, the patient displayed varying blood pressure readings between the right and left arms. The antinuclear antibody (ANA) and antismooth muscle (ASMA) antibody tests yielded negative results, while the electroencephalogram (EEG) showed normal readings. The cerebral angiogram indicated multiple internal and external stenoses, with total stenosis evident in the basilar artery. The patient was diagnosed with multiple extracranial and intracranial stenoses due to TA. Total stenosis of the basilar artery resulted in brainstem infarction, which led to the development of LiS. Meanwhile, the disturbance of the motor tracts in the ventral brainstem was the underlying cause of the LiS. This case report demonstrated a variety of atypical presentations of TA. The involvement of multiple extracranial and intracranial arterial was attributed to LiS.

Takayasu’s arteritis (TAK) manifests as an insidiously progressive and debilitating form of granulomatous inflammation including the aorta and its major branches. The precise etiology of TAK remains elusive, with current understanding suggesting an autoimmune origin primarily driven by T cells. Notably, a growing body of evidence bears testimony to the widespread effects of B cells on disease pathogenesis and progression. Distinct alterations in peripheral B cell subsets have been described in individuals with TAK. Advancements in technology have facilitated the identification of novel autoantibodies in TAK. Moreover, emerging data suggest that dysregulated signaling cascades downstream of B cell receptor families, including interactions with innate pattern recognition receptors such as toll-like receptors, as well as co-stimulatory molecules like CD40, CD80 and CD86, may result in the selection and proliferation of autoreactive B cell clones in TAK. Additionally, ectopic lymphoid neogenesis within the aortic wall of TAK patients exhibits functional characteristics. In recent decades, therapeutic interventions targeting B cells, notably utilizing the anti-CD20 monoclonal antibody rituximab, have demonstrated efficacy in TAK. Despite the importance of the humoral immune response, a systematic understanding of how autoreactive B cells contribute to the pathogenic process is still lacking. This review provides a comprehensive overview of the biological significance of B cell-mediated autoimmunity in TAK pathogenesis, as well as insights into therapeutic strategies targeting the humoral response. Furthermore, it examines the roles of T-helper and T follicular helper cells in humoral immunity and their potential contributions to disease mechanisms. We believe that further identification of the pathogenic role of autoimmune B cells and the underlying regulation system will lead to deeper personalized management of TAK patients. We believe that further elucidation of the pathogenic role of autoimmune B cells and the underlying regulatory mechanisms holds promise for the development of personalized approaches to managing TAK patients.

Abstract 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.

Abstract Takayasu’s arteritis (TAK) is a rare chronic granulomatous arteritis that mainly affects the aorta and its major branches. Coronary artery (CA) involvement can be observed in 10–25% of TAK patients. We report a 21-year-old young female who was previously diagnosed with TAK and severe left main coronary artery (LMCA) stenosis and underwent numerous percutaneous coronary interventions (PCIs) in our hospital due to in-stent restenosis (ISR). This time, an excimer laser coronary atherectomy (ELCA) and drug-coated balloon (DCB) dilation was taken at the LMCA for the ISR. The blood flow was smooth after the operation, and she was symptom-free after discharge. Unfortunately, 5 months later, severe intimal hyperplasia was still seen in the stent of LMCA and left anterior descending (LAD) coronary artery. A coronary artery bypass graft surgery (CABG) was performed, and she has been symptom-free ever since. ELCA plus DCB is one of the novel ways we first reported. However, ensuring long-term inflammation control is equally important to restore blood flow. The combination of revascularization and anti-inflammation/immunosuppression is recommended to improve the outcomes of TAK patients with CA involvements.

Abstract 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,

Background This study aims to assess pregnant women’s maternal and fetal outcomes with Takayasu’s arteritis (TA). Material and Methods The study comprised ten pregnant women at the time of diagnosis or afterwards among the 50 patients diagnosed with TA between 2003 and 2021. Twenty-one pregnancy outcomes of 10 patients were obtained from hospital records and telephonic interviews. Two pregnancies were excluded due to timing before diagnosis. Results Based on the angiographic classification, six patients had type 1, two had type 2b, and two had type 1+4 TA. 63.15% of pregnancies were planned, and the rheumatologist approved 42.10%. Live birth occurred in 16 (84.2%) of 19 pregnancies, three pregnancies (15.7%) resulted in abortion and two (10.5 %) of 19 pregnancies ended in neonatal death. In five (26.3%) of the 19 pregnancies, the disease was activated during pregnancy. Two neonatal deaths were from the two patients diagnosed with preeclampsia during pregnancy. Pre-existing hypertension and active disease are shared features of these two patients. After one year of follow-up, six pregnancies (31.5%) had active disease, and four (66.6%) had active disease both before and during pregnancy. While fetal data analysis revealed no congenital anomalies, four pregnancies resulted in low birth weight and intrauterine growth retardation (21.05%). Conclusions The risk of developing preeclampsia and neonatal death should be considered, especially in TA patients with pre-existing hypertension who become pregnant during active disease. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]