Left ventricular apical aneurysm in Takayasu arteritis and chronic active Epstein-Barr virus infection.

BACKGROUND: Takayasu arteritis (TA) is a chronic inflammatory disease of unknown etiology characterized by a large vessel vasculitis involving the aorta and its branches. Myocardial involvement is extremely unusual in TA and is mostly in the form of myocarditis, ventricular hypertrophy, and ventricular dysfunction secondary to coronary ischemia. Submitral aneurysms have been reported in TA and has been attributed to the chronic inflammatory process in TA. CASE PRESENTATION: We report a novel instance of left ventricular apical aneurysm in a 37-year-old lady with TA and normal epicardial coronaries. She was diagnosed with a left ventricular apical aneurysm, moderate aortic regurgitation, and moderate pericardial effusion. The coronary arteries were normal. The patient had concomitant chronic active Epstein-Barr virus infection complicating patient outcome. CONCLUSIONS: Left ventricular apical aneurysm with normal epicardial coronaries is a rare cause of heart failure in Takayasu arteritis. Concomitant chronic active Epstein-Barr virus infection can potentially accentuate the inflammatory process in Takayasu arteritis and complicate management and patient outcomes.

An Abysmal Quadrad: A Rare Case of Ankylosing Spondylitis, Ulcerative Colitis, and Takayasu Aortoarteritis Complicated by Secondary Renal Amyloidosis.

Ankylosing Spondylitis (AS) is a chronic inflammatory arthritis that typically manifests in young males and may present with extra-articular manifestations. Takayasu aortoarteritis (TA) is a large vessel vasculitis that predominantly affects young and middle-aged females. Despite the limited number of studies examining the potential association between these two diseases, we report a unique case of an individual with ankylosing spondylitis and ulcerative colitis who subsequently developed Takayasu aortoarteritis. This progression ultimately led to the development of secondary renal amyloidosis, attributed to a combination of inflammatory pathologies.

Current Diagnosis and Management of Takayasu Arteritis.

Takayasu arteritis (TA or TAK) is a chronic large vessel vasculitis with predilection to affect the aorta and its branches. The new 2022 ACR/EULAR classification criteria for Takayasu arteritis incorporated imaging characteristics as an absolute requirement. ESR and CRP fails in accuracy as disease activity markers. Pentraxin 3 appears to be a relatively superior biomarker, which correlates with ITAS 2010 as per several studies. PET-CT is also increasingly being studied for assessing disease activity with variable results. The management of TAK involves use of steroids with upfront steroid sparing immunosuppressive agents. MMF is one such conventional DMARD/immunosuppressant with good efficacy and better safety profile, as reported in various cohort studies. Tocilizumab is proved to be a rapid remission inducing agent in refractory Takayasu arteritis in observational studies. TNF inhibitors in many uncontrolled studies showed good responses, and there is a need for good RCTs for confirmation. JAK inhibitors have also been used with success in a few reports.

Role of Cardiac MRI Including LGE, T1 and T2 Mapping in the Assessment of Cardiac Involvement in Patients of Nonspecific Aorto-arteritis: A Prospective Study.

Objective Nonspecific aorto-arteritis (NSAA) may involve the myocardium in the form of edema and fibrosis. We conducted this study to investigate role of cardiac MRI including late gadolinium enhancement (LGE), T1 and T2 mapping in the assessment of cardiac involvement in NSAA. Methods and Materials Over the period between 2016 and 2019, 36 patients with NSAA presenting with uncontrolled hypertension, left ventricular dysfunction, congestive cardiac failure, or tachyarrhythmia were included in the study. We also had 16 voluntary control patients for providing normal T1 and T2 mapping values. Results The average age of patients was 27.1 years and the majority were females. MRI is more sensitive than echocardiography in the detection of LV dysfunction and RWMA. Out of 36 patients, 10 (27.8%) had LGE. The most common pattern of midmyocardial enhancement was present in 5 out of 10 patients. Five (13.8%) patients show mid-myocardial enhancement, followed by epicardial enhancement, which was seen in four (11.11%) patients. The values of post-gad T1 mapping values were significantly lower than pre-gad T1 mapping values. At a cut-off global native T1 mapping value of 1019 milliseconds had the sensitivity of 83.3% and specificity of 81.2% in detecting an abnormal T1 map. No significant association of MRI contrast enhancement with elevated ESR and CRP levels. There was no significant relation of myocardial T2 mapping values between NSAA and control groups. Conclusion Quantitative tissue characterization in the myocardium with native T1 mapping values help in the detection of cardiac involvement in patients with NSAA. T1 mapping may provide incremental value in the assessment of myocardial involvement in NSAA in addition to LGE imaging.

[Pathohistological characteristics of muscles in patients with chronic lower limb ischemia]. / Patogistologicheskaya kharakteristika myshts u patsientov s khronicheskoi arterial'noi nedostatochnost'yu nizhnikh konechnostei.

Despite the widespread occurrence of ischemic diseases of the lower extremities, including atherosclerosis and diseases with an autoimmune component of their pathogenesis, the pathohistological signs of damage and concomitant chronic ischemia, compensatory tissue responses as intracellular and cellular regeneration remain out of the field of vision in researchers. OBJECTIVE: To assess the signs of damage (the extent of necrosis and apoptosis, capillary density (CD)) and regeneration (the cross-sectional muscle fiber area (CSMFA), the proportion of centrinucleated muscle fibers (CNMFs), and that of connective tissue), by using the gastrocnemius medial head biopsy specimens obtained from patients with heterogeneous forms of chronic lower limb obliterating diseases (CLLODs). SUBJECTS AND METHODS: The investigation included the biopsy specimens obtained from 44 men under 65 years of age (their mean age was 54±9.8 years) with Stage IIB-IV chronic limb ischemia (according to the A.V. Pokrovsky-Fontaine classification) with its history of at least six months. The nosological entities were atherosclerotic lesion in 33 patients (distal atherosclerosis n=13), multistage lesion (n=8), and Leriche's syndrome (n=12); autoimmune-mediated vascular injury in 11 patients (Buerger's disease (n=7) and nonspecific aortoarteritis (n=4)). The similar muscle fragments obtained during autopsy from the deceased without obvious signs of cardiovascular system diseases were examined as a control. RESULTS: It was found that there was a statistically significant difference between the nosological entities, as compared to the control in terms of CD and CSMFA (a decrease), the proportion of CNMFs and that of connective tissue (an increase). No substantial differences were found in the studied parameters between the nosological entities. CONCLUSION: The findings may suggest the universal mechanism for damage to striated muscle tissue because of circulatory hypoxia, regardless of its etiology and the common character of tissue compensatory-adaptive responses (regeneration).

Ortner's Syndrome Associated with Takayasu's Aortoarteritis Identified on Fluorodeoxyglucose Positron-Emission Tomography/Computed Tomography.

Cardiovocal syndrome or Ortner's syndrome is hoarseness voice due to left recurrent laryngeal nerve palsy secondary to nerve compression caused by enlarged cardiovascular structures in the mediastinum. We present here an interesting positron-emission tomography/computed tomography image of a patient suspected to have Takayasu's aortoarteritis and presenting with hoarseness of voice.

Disease-modifying anti-rheumatic drugs for the management of Takayasu arteritis-a systematic review and meta-analysis.

The pharmacotherapy of Takayasu arteritis (TAK) with disease-modifying anti-rheumatic drugs (DMARDs) is an evolving area. A systematic review of Scopus, Web of Science, Pubmed Central, clinical trial databases and recent international rheumatology conferences for interventional and observational studies reporting the effectiveness of DMARDs in TAK identified four randomized controlled trials (RCTs, with another longer-term follow-up of one RCT) and 63 observational studies. The identified trials had some concern or high risk of bias. Most observational studies were downgraded on the Newcastle-Ottawa scale due to lack of appropriate comparator groups. Studies used heterogenous outcomes of clinical responses, angiographic stabilization, normalization of inflammatory markers, reduction in vascular uptake on positron emission tomography, reduction in prednisolone doses and relapses. Tocilizumab showed benefit in a RCT compared to placebo in a secondary per-protocol analysis but not the primary intention-to-treat analysis. Abatacept failed to demonstrate benefit compared to placebo for preventing relapses in another RCT. Pooled data from uncontrolled observational studies demonstrated beneficial clinical responses and angiographic stabilization in nearly 80% patients treated with tumour necrosis factor alpha inhibitors, tocilizumab or leflunomide. Certainty of evidence for outcomes from RCTs ranged from moderate to very low and was low to very low for all observational studies. There is a paucity of high-quality evidence to guide the pharmacotherapy of TAK. Future observational studies should attempt to include appropriate comparator arms. Multicentric, adequately powered RCTs assessing both clinical and angiographic responses are necessary in TAK.

Comparison Between Time-Resolved Magnetic Resonance Angiography and Diagnostic Digital Subtraction Angiography in the Vascular Assessment of Nonspecific Aorto-Arteritis Patients: A Prospective Study.

PURPOSE: Nonspecific aortoarteritis (NSAA) is a chronic inflammatory vasculitis involving aorta and its branches. We conducted a study prospectively to compare time resolved magnetic resonance angiography (MRA) with diagnostic digital subtraction angiography (DSA) for the vascular assessment in the patients of NSAA. MATERIALS AND METHODS: Seventeen patients of NSAA were recruited in the study over the period of 3 years. Contrast enhanced MRA using Time-resolved angiography With Interleaved Stochastic Trajectories (TWIST) sequence and diagnostic DSA were performed in these 17 patients. RESULTS: The majority of the patients were young (median age was 25 years, range 8 to 46 years) and 11 patients were females. Erythrocyte sedimentation rate (ESR) was elevated in 9 patients and C-reactive protein (CRP) was elevated in 6 patients. Most commonly involved vessels in our patients were right renal artery (14 patients), abdominal aorta (12 patients) and left renal artery (11 patients). Left and right subclavian arteres were involved in 10 and 6 patients respectively. The sensitivity and specificity of time-resolved MRA using TWIST sequence is 100% as compared to DSA in the assessment of major vessels such as aorta, arch vessels, celiac artery and superior mesenteric artery. However, the sensitivity and specificity of time resolved MRA in the evaluation of renal arteries and vertebral arteries were 100%, 71.4% and 85.7%, 33.3% respectively. No significant association of MRI contrast enhancement with erythrocyte sedimentation rate (p = 1.00) and C-reactive protein (p = 0.600). CONCLUSION: Time resolved MRA images obtained using TWIST sequence were as qualitative as DSA images and can noninvasively evaluate the vascular involvement in NSAA patients.

Update on pregnancy in Takayasu arteritis-A narrative review.

Takayasu arteritis (TA) is a chronic, idiopathic large-vessel vasculitis that affects women of reproductive age, and has significant maternal and fetal implications. Although there are contrasting data on the effect of TA on fertility, most studies have shown that fertility outcomes remain unaffected. The disease activity of TA usually either remains stable or decreases during pregnancy. The important fetomaternal complications are maternal hypertension, pre-eclampsia, prematurity, and intrauterine growth restriction. To reduce maternal and fetal morbidity, controlling the disease before conception is important. This review article discusses the various implications, challenges, and medical and endovascular management of TA during pregnancy.

Rare association of aortoarteritis and pheochromocytoma: A case report.

INTRODUCTION: Pheochromocytoma is an uncommon but treatable cause of secondary hypertension, it may present with a wide variety of manifestations. The coexistence of pheochromocytoma and vascular abnormalities is described but rarely reported entity. PRESENTATION OF CASE: A 36-year-old man was admitted to our hospital for severe hypertension, examination revealed absent femoral pulses with notion of intermittent claudication. Abdominal computed tomography revealed the presence of a right adrenal pheochromocytoma. CT angiogram showed thickening of the thoracoabdominal aortic wall and the proximal portions of some of its branches with stenosis of more than 50% of the origin of the celiac trunk, bilateral occlusion of the external iliac arteries and trunk stenosis of the right renal artery. The Pheochromocytoma was surgically removed. DISCUSSION: Coexistence of pheyochromocytoma and vascular abnormalities especially renal artery stenosis and aortoarteritis seems to be an association rather than a coincidence. CONCLUSION: To the best of our knowledge, the coexistence of pheochromocytoma along with both aortoarteritis and renal artery stenosis has not been reported thus far. The diagnosis, management and potential mechanisms underlying such an association will be discussed in this case.

Takayasu's aorto-arteritis: Not your regular lesion for angioplasty.

We report a case of a 6-year-old female child with Takayasu's aorto-arteritis (TA) with severe coarctation of the aorta which resulted in an aortic dissection post-ballooning. This happened despite ensuring that markers for disease activity were negative, with appropriate corticosteroid therapy started before the procedure, and using a low-profile, low-pressure, and slightly undersized balloon for dilating the stenotic segment. It required immediate endovascular stenting to tide over the crisis. Following the procedure, she became normotensive with well-palpable lower limb pulses.

Takayasu's arteritis: a rare disease in Poland.

INTRODUCTION: Takayasu's arteritis (TA) is a rare and potentially life-threatening granulomatous large-vessel vasculitis that involves mostly in the aorta and its proximal branches, and occurs most commonly in young females. This study measures the incidence and prevalence of TA, and assesses the gender distribution and territorial differences in the occurrences of this disease in Poland over a five-year period. To the best of our knowledge, this is the first evaluation of this rare disease in Poland based on a hospital morbidity database. MATERIAL AND METHODS: Analyses were performed with population-based administrative data obtained from a national hospital morbidity study carried out between January 2011 - December 2015 by the Polish National Institute of Public Health. Yearly incidence rates and prevalence of TA were calculated using the number of TA patients and corresponding census data for the overall Polish population. RESULTS: Data included 660 hospitalization records. The final study sample comprised 177 patients: 154 female (87%) and 23 male (13%) with first-time hospitalization for TA. The mean age was 45.4years (95% CI: 42.9-47.8; SD 16.8; range 4-81 years), median 47. The incidence rate of TA was estimated at 0.92 per million per year (95% CI: 0.68-1.16). Five-year TA prevalence was estimated to be 4,6 per million. Incidence rates of TA did not vary significantly between more urban and more rural regions. CONCLUSIONS: The incidence of TA in Poland was similar or lower to data reported by other European countries. The study provides epidemiological data on TA in Poland that may be useful while comparing it with other geographical regions.

18F-Fluorodeoxyglucose Positron Emission Tomography-computed Tomography in Evaluation of Large Vessel Vasculitis.

18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET-CT) is a rapidly evolving hybrid imaging technique in evaluation of infection and inflammation. Usually, functional changes often precede anatomical changes. 18F-FDG PET-CT, a noninvasive diagnostic test and it is useful for the early detection of inflammation. Most of the large vessel vasculitis patients present with nonspecific signs and symptoms, which are difficult to diagnose clinically. Here, we discuss three cases of large vessel vasculitis with different clinical presentations, identified by 18F-FDG PET-CT scan.

Aortoarteritis with tuberculosis.

Aortoarteritis is an inflammatory condition of the aorta, which has been rarely reported due to tuberculous infection. We report two cases of children who had aortoarteritis along with tuberculosis (TB), of which one had collapse consolidation and the other had latent TB. Both patients were treated with anti-TB therapy and steroids.

Percutaneous Angioplasty of the Sole Patent Cerebral Artery in Two Patients with Takayasu's Aortoarteritis.

We report two female patients with Takayasu's aortoarteritis, who presented with symptoms of cerebral ischemia due to critical stenosis of the sole patent cerebral artery. Both had occlusion of both vertebral arteries and one carotid artery with critical stenosis of the other carotid artery and presented with hemiparesis contralateral to the patent but stenosed cerebral artery. They also had transient ischemic attacks attributable to the culprit vessel. In the first patient, balloon angioplasty alone was not successful, and hence, a self-expanding stent was deployed in the right common carotid artery. In the second patient, successful balloon angioplasty was performed for the left common carotid artery. Distal protection devices were not used, and neither patient experienced any periprocedural neurological event. Clinical follow-up at six months revealed no significant cerebral events.

[Medicinal laws and application examples of traditional Chinese medicine prescriptions for multiple aorto-arteritis].

To collect the literature on traditional Chinese medicine treatment for multiple aorto-arteritis from China National Knowledge Infrastructure(CNKI), establish prescriptions database after screening and normalizing the prescriptions reported in these literature, and analyze their medicinal rules by using traditional Chinese medicine inheritance support system. A total of 126 prescriptions for multiple aorto-arteritis were screened, containing 212 kinds of Chinese herbs. 26 core herb combinations were obtained by analysis of the commonly used herbs and their use frequencies. The treatment for multiple aorto-arteritis was manly of tonifying qi to nourish blood, promoting blood circulation to remove blood stasis, warming yang to dredge collaterals, and four new prescriptions were obtained. On this basis, two clinical cases were taken as the examples by analyzing the medicinal rules and the features of multiple aorto-arteritis. The first case showed that the herb combination of this study conformed to the basic core drug application mode and the core pathogenesis of multiple aorto-arteritis. The second case reflected the characteristics of the new prescriptions' herb combinations based on entropy hierarchical clustering. The practical analysis of the two clinical cases further indicated the reliability of the results. This study has certain guiding significance and reference value on new medicine research and development as well as clinical traditional Chinese medicine treatment for multiple aorto-arteritis.

Spectrum of Abdominal Aortic Disease in a Tertiary Health Care Setup: MDCT Based Observational Study.

INTRODUCTION: Abdominal aortic disease is an important cause of clinical disability that requires early detection by imaging methods for prompt and effective management. Understanding regional disease pattern and prevalence has a bearing on healthcare management and resource planning. Non-invasive, conclusive imaging strategy plays an important role in the detection of disease. Multi-Detector Computed Tomography (MDCT) with its technological developments provides affordable, accurate and comprehensive imaging solution. AIM: To evaluate regional demography of abdominal aortic disease spectrum detected using MDCT imaging data in a tertiary hospital. MATERIALS AND METHODS: A descriptive study was conducted based on MDCT imaging data of patients who were investigated with clinical diagnosis of abdominal aortic disease, from March 2008-2010, over a period of 24 months. Patients were examined with the contrast-enhanced MDCT examination. Morphological diagnosis of the aortic disease was based on changes in relative aortic caliber, luminal irregularity, presence of wall calcification, dissection or thrombus and evidence of major branch occlusion. Patients were categorized into four groups based on imaging findings. MDCT information and associated clinical parameters were examined and correlated to management of patient. Descriptive statistical data, namely mean, standard deviation and frequency of disease were evaluated. RESULTS: A total of 90 out of 210 patients (43%) were detected with the abdominal aortic abnormality defined by imaging criteria. Group I, comprising of patients with atherosclerosis -including those with complications, constituted 65.5% of the patients. Group II represented patients with aneurysms (45.5%). Group III, consisting of 32.2% of the patients, contained those with dissections. The rest of the patients, including patients with aorto-arteritis, were classified as group IV. Eight patients with aneurysm and one patient with aorto-arteritis were considered for surgical treatment. Ten patients with dissection underwent endovascular procedure. Rest of the patients was managed conservatively. CONCLUSION: Aortic disease was observed in 43% of investigated patients. Atherosclerosis with and without aortic aneurysm constituted the largest group. MDCT provided comprehensive information about the lesion and associated complications. In view of the wider availability and desired imaging qualities, MDCT provided optimal information for diagnosis and management of aortic pathology. Majority of our patients (90%) were treated conservatively.

Medicinal laws and application examples of traditional Chinese medicine prescriptions for multiple aorto-arteritis / 中国中药杂志

To collect the literature on traditional Chinese medicine treatment for multiple aorto-arteritis from China National Knowledge Infrastructure(CNKI), establish prescriptions database after screening and normalizing the prescriptions reported in these literature, and analyze their medicinal rules by using traditional Chinese medicine inheritance support system. A total of 126 prescriptions for multiple aorto-arteritis were screened, containing 212 kinds of Chinese herbs. 26 core herb combinations were obtained by analysis of the commonly used herbs and their use frequencies. The treatment for multiple aorto-arteritis was manly of tonifying qi to nourish blood, promoting blood circulation to remove blood stasis, warming yang to dredge collaterals, and four new prescriptions were obtained. On this basis, two clinical cases were taken as the examples by analyzing the medicinal rules and the features of multiple aorto-arteritis. The first case showed that the herb combination of this study conformed to the basic core drug application mode and the core pathogenesis of multiple aorto-arteritis. The second case reflected the characteristics of the new prescriptions' herb combinations based on entropy hierarchical clustering. The practical analysis of the two clinical cases further indicated the reliability of the results. This study has certain guiding significance and reference value on new medicine research and development as well as clinical traditional Chinese medicine treatment for multiple aorto-arteritis.

Maternal and fetal outcomes in pregnant women with Takayasu aortoarteritis: Does optimally timed intervention in women with renal artery involvement improve pregnancy outcome?

OBJECTIVE: Takayasu aortoarteritis (TA) is common in the Southeast Asian and Indian subcontinent regions with a female-to-male ratio of 8:1. Age at diagnosis is < 30 years in 90% of the cases. Because the disease is common in women of child-bearing age, management of pregnancy in these patients becomes an important issue. The purpose of this study is to evaluate the maternal and fetal outcomes in pregnancies with TA and also to evaluate whether early intervention for renal artery involvement is associated with improved outcomes. MATERIALS AND METHODS: We collected data of 12 patients with 18 pregnancies prospectively from 2006 to 2012. The patients were divided into three groups and their outcomes were noted: (1) without renal artery involvement; (2) with renal artery involvement without intervention; and (3) with renal artery involvement for which intervention has been done. RESULTS: Body mass index of patients was between 18.5 kg/m(2) and 23.2 kg/m(2). Renal artery involvement and hypertension were seen in four patients. One patient had percutaneous transluminal balloon angioplasty and another had renal artery stenting. In patients without renal artery involvement, gestational hypertension was seen in 50%, pre-eclampsia in 10%, abortion in 10%, and intrauterine growth restriction (IUGR) in 40% of pregnancies. In patients with renal artery involvement without intervention, gestational hypertension was seen in 90%, pre-eclampsia in 20%, abortion in 60%, preterm in 20%, IUGR in 20%, fetal demise in 20%, and neonatal death in 20% of pregnancies. In patients with renal artery involvement for which intervention has been carried out, gestational hypertension was seen in 66%, and abortion and IUGR were seen in 33% of pregnancies. CONCLUSION: Patients with renovascular involvement without intervention are at high risk of having maternal and fetal complications. Early intervention prior to conception in these women is recommended to prevent pregnancy complications.