Diagnosis Of Takayasu Arteritis Research Articles

[Takayasu arteritis after COVID-19 infection in a 26-year-old female patient with Crohn's disease].

Extraintestinal manifestations, e.g. of the skin, joints or liver, are frequently found in Crohn's disease. We report about a 26-year-old female patient with longstanding Crohn's disease, who was admitted to our hospital with suspicion of an acute attack with suggestive symptoms and increased significantly inflammatory parameters. Shortly before, symptomatic COVID-19 disease (SARS-CoV-2 variant omicron) had been made. Comprehensive endoscopic and imaging diagnostics ruled out active Crohn's disease. However, inflammatory thickening of the aortic arch was seen, and a diagnosis of Takayasu arteritis (type II b) was made. Steroid therapy resulted in a rapid and sustained improvement of clinical symptoms. The occurrence of Takayasu arteritis is extremely rare outside Japan. An coincidence has been described in chronic inflammatory bowel diseases and is discussed as a possible extraintestinal manifestation. The occurrence of immune-mediated disease after COVID-19 disease has been described and may be triggered by the infection. Patients with inflammatory bowel disease may represent a special risk population.

Poor obstetric outcomes in women with takayasu arteritis: a retrospective cohort study.

The objective of this study was to assess the pregnancy outcomes in a cohort of patients who experienced pregnancies before and/or after being diagnosed with Takayasu's arteritis (TA). The present investigation encompassed a total of 88 pregnancies seen in a cohort of 35 patients who met the criteria outlined by the American College of Rheumatology in 1990 for the classification of Takayasu arteritis (TA). Pregnancies were classified into two categories. 1. Pregnancies that occurred before the diagnosis (pre-d or pre-TA) 2. Pregnancies that happened following a diagnosis (post-d or post-TA). Fifty-nine pregnancies (67.0%) occurred in 21 TA patients before the diagnosis with and a complication rate of 15.2%, and twenty-nine pregnancies (33.0%) occurred in 14 patients concomitant with or after TA diagnosis and complication rate 100%. Although the hypertension rate was higher in the pre-d group than in the post-d group, it was not significant (32.2% vs. 10.3%, p = 0.160). However, preeclampsia (20.6% vs. 0%, p = 0.001), low birth weight (27.5% vs. 1.6%, p = 0.001), and prematurity (24.1% vs. 1.6%, p = 0.035) were observed more frequently in the post-d group compared to the pre-d group. The frequency of abortions and in-utero deaths were similar in both groups (p > 0.05). Patients with hypertension had significantly higher rates of preeclampsia (p = 0.003), preterm birth (p = 0.036), low birth weight (p = 0.250), abortion (p = 0.018), in utero death (p = 0.128), and cesarean section (p = 0.005) than those without hypertension. Renal artery involvement was detected in 15 (42.8%) patients. All patients with renal artery involvement had hypertension, and they had significantly more pregnancy complications than the other group (p = 0.001). TA negatively affects pregnancy outcomes. A good control of arterial hypertension before conception and during pregnancy is critical to improve both maternal and fetal outcomes. In addition, detecting renal artery stenosis before pregnancy is important in reducing possible negative pregnancy outcomes.

Takayasu arteritis masquerading as recurrent abdominal pain

Takayasu arteritis (TA) is a rare granulomatous inflammation of large arteries, specifically the aorta and its branches. It is more prevalent in young Asian women with the highest incidence in Japan. There is a delay in diagnosis due to nonspecific early symptoms and unfamiliarity with this disease. We present a case of a young female who presented with recurrent abdominal pain suggestive of mesenteric angina and constitutional symptoms over one-year. Her radial pulses were absent, and she had multiple bruits on examination over the right subclavian and carotid arteries and the epigastrium, along with high inflammatory markers. Her CT angiography of large vessels confirmed the diagnosis of TA and she was treated with high-dose steroids with good clinical remission confirmed by a reduction in inflammatory markers and improvement in symptoms.

Beyond the Usual Suspects: Emerging Insights into Takayasu’s Arteritis and Its Role in Secondary Hypertension

Background: Pulselessness is the main sign of Takayasu's Arteritis (TAK), a granulomatous large artery vasculitis that mostly affects the aorta, major aortic branches, and pulmonary arteries. Renal artery stenosis is the source of arterial hypertension, which is common in TA. It is estimated that there are 2.6 incidences of Takayasu's arteritis per million people worldwide each year.
 Case Details: We report on an 18-year-old girl whose significant arterial hypertension on her left arm, identified during a routine check-up, was the primary manifestation of Takayasu's arteritis (TA). The left arm's systolic blood pressure was noticeably greater than the right's. Additionally, there was a difference in blood pressure between the legs and left arm. Auscultation revealed the presence of vascular bruits over the left subclavian regions. A CT angiography was used to establish the diagnosis of TA. The results indicated a substantial constriction at the right renal artery's origin and characteristics compatible with aortic arteritis of the thoracic and abdominal aorta with active disease given wall enhancement. Due to the disorder's rarity and the varied ways in which it manifests clinically, a delayed diagnosis and treatment are likely.
 Conclusion: Our study emphasizes that this illness can and does affect young people, and as such, patients presenting with suggestive symptoms and signs—especially young patients with unexplained hypertension—should be taken into consideration. Appropriate imaging and clinical suspicion are essential for the accurate diagnosis and treatment of TA patients. Several findings were found during the clinical examination, including signs of claudication, fever symptoms, involvement of major vessels including the aorta and its branches. This illustrates the condition of Takayasu's arteritis and what medical professionals should anticipate when treating it.

Aneurysmal Disease in Patients With Takayasu Arteritis.

Takayasu arteritis (TA) leads to stenotic disease. Aneurysmal lesions are rarer. This study assessed the main characteristics of aneurysmal disease in a Canadian cohort of patients with TA. This monocentric retrospective study included patients with TA followed at the Mount Sinai Hospital Vasculitis Clinic in Toronto. Diagnosis of TA was based on clinical findings and/or satisfied the 1990 American College of Rheumatology classification criteria. Seventy-four patients were included. At any time, aneurysmal disease was found in 23 (31%) patients. Median disease duration was 9.0 (IQR 7.0-19.0) years. Prior hypertension (P = 0.02), fever (P = 0.04), and seizure disorders (P = 0.03) were more common. Limb claudication was less frequent (P = 0.01). Persistent and/or new aneurysms were demonstrated in 22/23 patients at follow-up. Thoracic aorta aneurysm (13/22) was most common, followed by abdominal aorta (8/22), subclavian (7/22), and carotid (6/22) artery disease. Aortic valve regurgitation was more frequent (9/23 vs 3/48; P = 0.001). Twenty-one patients had been treated with glucocorticoids (median 6.1 years [IQR 3.7-8.1]). Methotrexate, azathioprine, and leflunomide were repeatedly used. Infliximab (7/23) was used more often (P = 0.04), whereas tocilizumab was received by only 4 patients with aneurysmal disease (P = 0.01). Patients with aneurysms suffered more frequent relapses (2.0 [IQR 0.0-4.0] vs 1.0 [IQR 0.0-2.0], P = 0.04). Aneurysmal disease was found in a significant proportion of patients with TA. Given that aneurysms may carry a risk of rupture, and are associated with a higher rate of relapse, this finding should be reported systematically in TA studies.

Ischemic injury of colon mucosa as a first manifestation of Takayasu arteritis in a young female patient: challenges in the differential diagnosis

Takayasu arteritis (TA) is a type of chronic large vessel vasculitis, which most often affects the aorta and its main branches as a kind of destructive-productive segmental aortitis and subaortic panarteritis, with possible obliteration of their orifices. Clinical picture of TA may be different depending on the nature and localization of the pathological process.
 We present a clinical case of ischemic injury of the colon mucosa in TA in a young (21 year old) female patient after she had a novel coronavirus infection (COVID-19). Due to ineffectiveness of out-patient treatment of ulcerative colitis, as well as complaints that were not meeting the diagnosis of ulcerative colitis, the patient was referred for additional examination and treatment modification in inpatient settings. Taking into account her diarrheal syndrome at the disease onset, the ileocolonoscopy results (rectal and colonic erosions), the results of histological examination (diffuse leukocytic infiltration of the mucosa, lymphoid infiltration of submucosa of the colon), elevation of proinflammatory markers, involvement of the inferior mesenteric artery at ultrasound dopplerography, the differential diagnosis was made between the TA overlap syndrome with inflammatory bowel diseases and TA-associated ischemic colitis. The assessment results made it possible to exclude inflammatory bowel diseases, to establish the diagnosis of TA and to consider colonic mucosa abnormalities as ischemic with underlying disease.
 The clinical case described is a rare event of intestinal ischemia as a first TA manifestation. Given the increasing worldwide prevalence of TA, especially after the new coronavirus infection pandemic, it is necessary to include this rare cause of gastrointestinal vasculitis to the differential diagnostic search, since mesenteric ischemia is one of the main causes of death in AT patients.

Pathophysiology, Diagnosis, and Management of Takayasu Arteritis: A Review of Current Advances.

Takayasu arteritis (TA) is a rare, chronic, inflammatory vasculitis that primarily affects large arteries, causing significant morbidity and mortality. This review provides an overview of the pathophysiology, diagnosis, and management of TA based on current advances in the field. TA is characterized by autoimmune-mediated inflammation, vascular remodeling, and endothelial dysfunction. The disease progresses through three stages (active, chronic, and healing phase)each presenting distinct clinical features. Diagnosis of TA can be challenging due to non-specific clinical manifestations and the lack of specific diagnostic tests. Various imaging modalities, such as angiography, ultrasound, and Doppler techniques, play a crucial role in the diagnosis of TA by visualizing arterial involvement and assessing disease extent. Management of TA involves a multidisciplinary approach, with disease-modifying anti-rheumatic drugs (DMARDs) as the cornerstone of medical therapy. Synthetic and biologic DMARDs are used to induce remission, control inflammation, and prevent complications. Non-pharmacologic interventions, such as resistance exercises and curcumin supplementation, show potential benefits. Invasive interventions, including endovascular therapy and open surgery, are used for managing vascular lesions. However, challenges remain in disease understanding and management, including the heterogeneity of disease presentation and the lack of standardized treatment guidelines. The future of TA management lies in precision medicine, utilizing biomarkers and molecular profiling to personalize treatment approaches and improve patient outcomes. Further research is needed to unravel the underlying mechanisms of TA and develop targeted therapies.

#5555 TAKAYASU ARTERITIS IN THE TIME OF SARS-COV-2

Abstract Background and Aims SARS CoV 2 infection is characterized by pulmonary, cardiovascular, neurological and other complications. New onset hypertension after SARS Cov 2 infection was observed in various studies with an unclear mechanism. Vessel inflammation is a common finding in these patients. Case A young woman (24 years old) referred to our Unit for high blood pressure levels appeared few days after SARS COVID 2 infection. She had a positive familiar history of hypertension. A 24 hours blood pressure monitoring confirmed hypertension and the patient was treated with a beta-blocker and referred to the nephrologist. Physical examination was negative and peripheral pulses were palpable. Blood pressure was 150/90 mmHg without significative discrepancy (<10 mmHg) between right and left arm and between upper and lower limbs. Carotid-femoral and carotid-radial pulse wave velocity was in the upper limit of the normal range (9.8 and 8.0 m/sec respectively), renal function was preserved (serum creatinine 0.6 mg/dl, eGFR 154 ml/min) and proteinuria was 450 mg/die. C-reactive protein was 13 mg/L. Complement components C3 and C4 as well as IgG and anti-neutrophil cytoplasmic antibody (ANCA) levels were in the normal range. IgA and IgM were slightly elevated and anti-nuclear antibody (ANA) levels were 1:160. TSH was in the normal range. Patient was switched to calcium channel blockers and a screening to exclude secondary hypertension was performed. Hormonal profile showed hypersecretion of cortex and medullary adrenal gland (high renin and aldosterone, plasma and urinary cortisol, and epinephrine levels). Patient was treated with ACE inhibitor and showed an optimal blood pressure profile. Abdominal-Chest CT angiography detected no increase in adrenal gland dimension. Conversely, a left renal artery stenosis and a mild enlargement of the para-aortic tissue, suggestive of a retroperitoneal fibrosis, was described (Figure 1). A Doppler ultrasound examination confirmed a high systolic peak velocity in left renal artery and a low resistive index in the left kidney. According to the diagnosis of renal fibrosis, patient was treated with oral prednisone at a dosage of 1 mg/Kg/BW. Three months later, Doppler ultrasound and CT were materially unchanged. After a case revision and a negative evidence of inflammation at PET-FDG examination, the Takayasu Arteritis diagnosis was formulated and the patient underwent left renal artery angioplasty. Oral prednisone was tapered and methotrexate was started. One month later blood pressure and Doppler ultrasound velocimetric parameters were normalized. Conclusion This case report suggests that Takayasu arteritis may occur after SARS COVID 2 infection.

Acute non-ST segment elevation myocardial infarction as the first manifestation of Takayasu arteritis in a 16-year-old female patient: a case report and literature review.

Takayasu arteritis (TA) is now recognized worldwide and is a disease that mainly affects the aorta and its main branches. TA rarely involves the small or medium-sized vessels. Certain vascular lesions, such as arterial stenosis, occlusion, and aneurysm are common with TA. However, patients with new-onset TA who present with left main trunk acute non-ST segment elevation myocardial infarction are extremely rare. We report a 16-year-old female patient with non-ST segment elevation myocardial infarction due to severe stenosis of the left main coronary artery that was caused by TA. She was eventually diagnosed with TA and underwent successful coronary artery stenting combined with glucocorticoids and folate reductase inhibitor therapy. Over the 1-year follow-up, she experienced two episodes of chest pain and was admitted to the hospital. During the second hospitalization, coronary angiography (CAG) revealed 90% stenosis of the original left main trunk (LM) stent. Following percutaneous coronary angiography (PTCA), drug-coated balloon (DCB) angioplasty was performed. Fortunately, a clear diagnosis of TA was made, and treatment was initiated with an interleukin-6 (IL-6) receptor inhibitor. Early diagnosis and therapy for TA are emphasized.

PS-R05-3: SEVERE GLOMERULAR MICROANGIOPATHY IN A PATIENT WITH NEPHROTIC SYNDROME FOLLOWING TAKAYASU ARTERITIS

Introduction: Takayasu arteritis (TA) is a large vessel inflammation that predominantly involves aorta and its main arteries. TA-caused kidney injury is known to be mainly due to renal artery stenosis. However, glomerulonephritis including membranous proliferative glomerulonephritis, focal segmental sclerosis, or crescentic nephritis has been histologically identified in some TA cases. Case description: A 69-year-old female presented with leg edema and refractory hypertension with nephrotic range proteinuria (17.0 g/gCr). She exhibited > 10 mmHg blood pressure discrepancy between left and right upper limbs and severe aortic regurgitation. Lab test showed high level of C-reactive protein and high erythrocyte sedimentation rate. Contrast-enhanced computed tomography identified wall thickening of the left subclavian artery, which led to the diagnosis of TA. Renal biopsy demonstrated that double basement membrane, enlarged subendothelial space, and mesangiolysis with no immunoglobulin deposition. Although azilsartan 40 mg QD and nifedipine 30 mg BID had been prescribed, systolic blood pressure (sBP) was still > 160 mmHg. Thus, while oral prednisolone was started to suppress TA-induced inflammation, azilsartan was replaced with sacubitril valsartan, an angiotensin receptor-neprilysin inhibitor (ARNI), for treating refractory hypertension. After a few weeks, sBP was decreased to 130 mmHg with sacubitril valsartan 200 mg QD and proteinuria was dramatically improved to 0.5 g/gCr. Discussion: TA commonly involves the renal artery, leading to stenosis and subsequent ischemic nephropathy. However, no stenosis and occlusion were observed in renal artery of this case. In addition, small vessel inflammation in the kidney such as vessel wall necrosis and immune cells infiltration around arterioles was not found. Serum levels of pro-inflammatory cytokines including vascular endothelial growth factor and interleukin-6, a well-known contributors for progression of TA-related inflammation, were within the normal range. The results indicate that TA-caused hypertension, but not vessel inflammation induced by TA, seems to play a pathological role for development of glomerular microangiopathy in this case. A previous study showed that 70% of TA patients presenting with glomerular lesion has severe hypertension and intimal thickening in renal arterioles was observed more frequently in TA patients presenting with glomerular lesion when compared to those without glomerulonephritis, which supports our hypothesis. In the present case, ARNI significantly reduced sBP which might directly contribute to the improvement of proteinuria. ARNI can be potent to control hypertension more effectively than conventional hypertensive agents in TA patients.

149: INCIDENCE, PREVALENCE, AND ASSOCIATION OF VASCULAR DISORDERS AMONG TAKAYASU ARTERITIS

Introduction: Studies have shown that patients with systemic vasculitis such as takayasu arteritis (TA) experience a higher incidence of hypertension, primary myocardial infarction (MI), and other vascular disorders. The aim of our study is to assess the incidence, prevalence, and association of vascular disorders amongst TA in the US population. Methods: We performed a cross-sectional retrospective study of the NIS database (2016-2018) using the ICD-10-CM codes. Hospitalizations with a diagnosis of TA and other comorbidities were identified. Weighted univariate analysis was performed using an unpaired t-test and chi-square test and multivariate analysis were performed using multivariate regression models to evaluate incidence, prevalence, and association of vascular disorders among TA. Results: We found 3,560 patients with TA. The incidence of MI (3.09% vs 2.15%), transient ischemic attack (1.4% vs 0.36%), and intracerebral hemorrhage (0.56% vs 0.24%) were higher in TA compared to no TA. (p <.0001) Concurrent prevalence of hypertension (67.7% vs 55.72%), dyslipidemia (35.96% vs 32.42%), ischemic heart disease (32.3% vs 22.21%), and congestive heart failure (23.17% vs 16.15%) higher amongst TA in comparison without TA. (p <.0001) In regression analysis, we found that patients with hypertension (aOR 4.49, 95%CI 3.26-6.19), dyslipidemia (1.34, 1.10-1.63), renal failure (1.30, 1.04-1.62), congestive heart failure (1.95, 1.56-2.45), MI (1.63, 1.16-2.27), angina (2.72, 1.01-7.32), acute ischemic stroke (1.86, 1.25-2.77), transient ischemic stroke (5.06, 3.09-8.28), subarachnoid hemorrhage (2.84, 1.17-6.93) (c=0.501) had significantly higher odds of takayasu’s arteritis. Conclusions: In our data, we identified a higher incidence, prevalence, and association of some vascular events amongst TA. Hence, more prospective studies are needed to evaluate the early identification to reduce the burden of vascular events amongst TA populations. Our study is confounded by the absence of severity and outcomes of disease, causality, and long-term follow-up of the patients.

84 Methotrexate toxicity in a patient with Takayasu arteritis; a case report

BackgroundMethotrexate is an antimetabolite commonly used as a chemotherapeutic and as a steroid sparing agent in multiple rheumatologic conditions. The common side effects are well documented and usually seen in patients receiving high dose therapy and hence more commonly seen in oncology. Here, we discuss a case on methotrexate toxicity in a patient being treated for Takayasu arteritis.MethodsThis was a retrospective case reviewResultsA seventeen-year-old was seen in the rheumatology service with a diagnosis of Takayasu arteritis after being initially worked up due to hypertension. Methotrexate therapy was started at an initial dose of 25 mg to be taken weekly. She was later seen in the pediatric out-patient unit 10 days post initiation of therapy with a history of inability to feed due to oral sores and severe abdominal pain. She also complained of odynophagia and hence had poor oral intake. On further history, it was noted that the patient was taking the medication on a daily basis as opposed to the prescribed weekly regimen. On exam, she was noted to have extensive ulceration of her buccal mucosa, gingiva and tongue. She was also found to have severe epigastric tenderness. Her genital regions were however spared. A diagnosis of methotrexate toxicity was made and she was admitted to the high dependency unit for management. Methotrexate was immediately discontinued and a hyper hydration regimen was initiated with intravenous fluids (125 ml/m2/h). Her pain was managed with an intravenous morphine infusion. Particular care was taken to avoid drugs such as proton pump inhibitors (PPIs), non-steroidal anti-inflammatory drugs (NSAIDs) and Sulphur containing drugs. A rescue was initiated with 15 mg/m2 of folinic acid given 6 hourly. The patient made a full recovery and was discharged 4 days later.Discussion and ConclusionsMethotrexate is a drug with varied and severe adverse effects. Toxicity with methotrexate affects multiple systems and can cause end organ damage. It is therefore of paramount importance to recognize toxicity early and manage appropriately before onset of end organ damage. The above patient was risk of hepatotoxicity, renal toxicity, Neurologic toxicity, methotrexate induced lung injury, hematologic toxicity and hypersensitivity. She developed mucositis as a result of the medication.Enhancing renal elimination of the drug is at the core of management. This can be done with hyperhydration, alkalinisation of urine and avoiding drugs that slow elimination of methotrexate such as PPIs, NSAIDs, tyrosine kinase inhibitors, sulfa drugs and penicillin like drugs. Once end organ damage is established, therapy is supportive and highly dependent on the organ systems involved and the extent of injury.It is necessary for clinicians to be well versed with the adverse effects expected with methotrexate as evidenced by this case for prompt diagnosis and sound management. It is also vital to counsel patients and parents about the medication- correct dosage and timing, expected adverse effects and when to seek medical attention.

ПРОБЛЕМЫ ХИРУРГИЧЕСКОГО ЛЕЧЕНИЯ ДЕТЕЙ С АРТЕРИИТОМ ТАКАЯСУ С ПОЗИЦИИ ПЕДИАТРА

The purposes of the study were as follows: to perform a comparative analysis of demographic and clinical data in children with Takayasu's arteritis (TA) who underwent surgical treatment of the underlying disease, to determine approaches to surgical interventions and to evaluate its outcomes. Materials and methods of the research: 51 children (boys:girls ratio 1:4.7) aged from 8 months till 17 years old (11 [9; 14] years as median) with a reliable TA diagnosis were observed from February, 2001 till January, 2022. The median age of the TA debut was 11 [9; 14] years. The single-center (I.M. Sechenov University Children's Clinical Hospital with the N.F. Filatov Clinical Institute of Children's Health of the I.M. Sechenov First Moscow State Medical University located in Moscow, Russia) case-control study included 40 patients: Group 1 (n=10) consisted of patients who underwent the surgical intervention, and Group 2 (n=30) consisted of children who have not been indicated for surgical treatment at the end of the follow-up. Patients did not differ statistically significantly in terms of gender (p=1.0), age (p=0.729), disease duration (p=0.542) and duration of follow-up at the hospital (p=0.708). The data that the authors have evaluated included gender, age, duration of the disease, including before the diagnosis was verified, the clinical picture at the onset and the type of TA, data from laboratory and instrumental studies before diagnosis and during follow-up, treatment options, assessment of the TA activity according to the ITAS-A score. Results: the median duration of TA before diagnosis was statistically significantly higher in the Group 1 (p=0.013) and amounted to 23.5 [13; 36] months (9.5 [2; 20] months in the Group 2). The median systolic blood pressure (SBP) and diastolic blood pressure (DBP) at the onset on the TA diagnosis in patients from the Group 1 (165.0 and 110.0 mm Hg, respectively) were statistically significantly higher (p&lt;0.001) than in the Group 2 (116.5 and 65.0 mm Hg, respectively). The arterial hypertension (p&lt;0.001) and headaches (p=0.006) were statically significantly more often in patients from the Group 1, while in patients from the Group 2 the general inflammatory reactions were more typical, such as episodes of fever (80%, p=0.003) and weight loss (33.3%). The median amount of vessels involved in the pathological process at the onset of TA in the Group 1 patients (6.5; [4; 8]) was also statistically significantly higher (p=0.043) than in the Group 2 patients (4; [3 ; 6]). In patients of the 1st Group, the abdominal aorta (90%, p=0.006), descending thoracic aorta (70%, p=0.007) and renal arteries (70%, p&lt;0.001) were affected statistically significantly more often. The Group 1 patients had 14 surgical interventions in total. The median SBP decreased after the 1st surgical intervention from 170 [160; 172] to 130 [105; 145] mm Hg, and the median DBP dropped from 110 [100; 114] to 70 [65; 85] mm Hg. The early postoperative period proceeded favorably after 13 out of total of 14 surgical interventions: in a single patient the neck phlegmon was developed on the same side a week after subclavian-carotid prosthesis on the right side. During the observation period in the hospital there were no deaths recorded in the Group 2. In a single female patient from Group 1 the aneurysm of the anastomosis of the infrarenal aorta ruptured on the 42nd month after the surgical intervention, which has led to her death. Conclusion: the duration of TA before the start of therapy, the extensive damage to the arterial bed, including involvement of the abdominal aorta and renal arteries, the absence or mild severity of the general inflammatory syndrome and arterial hypertension at the onset of the disease increase the likelihood of surgical treatment. The surgical intervention for TA should be carried out against the background of immunosuppressive therapy, according to the strict indications and considering the risk/benefit ratio for each patient.

Amaurosis as an initial presentation of Takayasu arteritis in children.

Visual disturbances in Takayasu arteritis (TA) are common but tend to be late manifestations of the disease. However, its presence at diagnosis must alert TA to avoid sight disabilities. Herein, we present two children with TA that debuted with vision loss, and the results of the literature review displayed 58 subjects with vision loss before the diagnosis of TA. The world English literature was reviewed by searching the PubMed database of the National Library of Medicine for the terms "Takayasu Arteritis" and "Blindness" or "Amaurosis fugax", from 190 to 2021. Cases eligible must present vision loss before or at TA diagnosis. Our two patients who presented with amaurosis fulfilled the criteria for TA diagnosis. The first patient had a bilateral and transient visual loss, whereas the second had monocular and permanent amaurosis. Both patients were cursed with hypertension and demonstrated large vessel compromise; their clinical picture improved with corticosteroids and immunosuppressant therapy. We identified in the literature review sixteen patients with TA in case reports and 42 in case series, plus our two cases presented herein with monocular or bilateral vision loss at the time of diagnosis. Previous literature indicated that amaurosis represents a severely advanced disease. Herein, we reported two children with amaurosis as their pivotal symptom; they had significant head and neck vascular alterations, so prompt and aggressive treatment is needed to prevent disease progression and disability. Transient or permanent vision loss must alert the physician to include Takayasu arteritis in the differential diagnosis.

Cerebrovascular Ischemic Events in Patients With Takayasu Arteritis.

Takayasu arteritis (TA) is a large vessel vasculitis that may complicate with cerebrovascular ischemic events. The objective was to describe clinical and vascular features of TA patients with cerebrovascular ischemic events and to identify risk factors for these events. We analyzed the prevalence and type of stroke/transient ischemic attack (TIA), factors associated with cerebrovascular ischemic events, and stroke-free survival in a large cohort fulfilling the American College of Rheumatology or Ishikawa criteria of TA. Among 320 patients with TA (median age at diagnosis, 36 [25-47] years; 261 [86%] women), 63 (20%) had a stroke (n=41; 65%) or TIA (n=22; 35%). Ischemic event localized in the carotid territory for 55 (87%) patients and the vertebral artery territory in 8 (13%) patients. Multiple stenosis were observed in 33 (52%) patients with a median number of stenosis of 2 (minimum, 0 to maximum, 11), and aneurysms were observed in 10 (16%) patients. A history of stroke or TIA before TA diagnosis (hazard ratio [HR], 4.50 [2.45-8.17]; P<0.0001), smoking (HR, 1.75 [1.01-3.02]; P=0.05), myocardial infarction history (HR, 0.21 [0.05-0.89]; P=0.039), thoracic aorta involvement (HR, 2.05 [1.30-3.75]; P=0.023), time from first symptoms to diagnosis >1 year (HR, 2.22 [1.30-3.80]; P=0.005), and aspirin treatment (HR, 1.82 [1.04-3.19]; P=0.035) were associated with cerebrovascular ischemic event. In multivariate analysis, time from first symptoms to TA diagnosis >1 year (HR, 2.16 [1.27-3.70]; P=0.007) was independently associated with cerebrovascular ischemic events in patients with TA. The HR for cerebrovascular ischemic event in patients who already experienced a stroke/TIA was 5.11 (2.91-8.99; P<0.0001), compared with those who had not. Carotid stroke/TIA is frequent in TA. We identified factors associated with cerebrovascular ischemic events.

A rare cause of hemoptysis in a young female

Takayasu's arteritis (TA) is also termed pulseless disease owing to the frequent occlusion of large arteries originating from the aorta and its major branches. It is an uncommon chronic inflammatory progressive idiopathic large vessel vasculitis with a predilection for young women of childbearing age group. Fatigue, malaise, and fever are the frequently encountered early symptoms of TA. Hemoptysis is not commonly seen as an initial presentation of TA. The subclavian artery is most commonly involved, and the disease is usually discovered accidentally owing to pulse asymmetry. In addition, pulmonary artery involvement is not common in TA. Here, we present a case of a young female presented to us with hemoptysis with pulmonary artery involvement with a de novo diagnosis of TA.

A case of middle aortic syndrome in takayasu arteritis with left ventricle dysfunction and heart failure

Left ventricular dysfunction is an uncommon complication of Takayasu arteritis (TA) with a prevalence of about 10%–15%. We report a case of a 27-year-old girl who presented with dyspnea, bipedal edema, loss of weight, and easy fatigability for 3 months. Patient also developed right hemiparesis 3 months ago. Computed tomography of the brain was suggestive of the left temporal infarct, on evaluation she was diagnosed as left ventricle (LV) dysfunction and she was treated for CVA and heart failure. Clinical examination revealed the absence of the left upper limb and bilateral lower limb pulses with right upper limb hypertension. Echocardiography revealed severe LV dysfunction (ejection function ~20%). Computed tomography angiogram and other inflammatory markers confirmed the diagnosis of TA and revealed the presence of coaraction of the thoracic and abdominal aorta. Coaractoplasty was done, and immunosuppressant therapy with oral prednisolone and weekly oral methotrexate was started. On follow-up, patient is asymptomatic with improved LV function. Any patient with LV dysfunction or dilated cardiomyopathy, reversible causes have to be ruled out and TA has to be thought of as a differential diagnosis particularly in young females. Checking of all four limbs pulses and blood pressure is strictly recommended. TA is a systemic vasculopathy that can progress to cause vital organ ischemia. Therefore, early diagnosis and management as well as long-term follow-up is recommended.

Takayasu arteritis in young female a case report

Takayasu arteritis (TA) is a systemic inflammatory large vessel vasculitis. Here, we report a rare case of 18-year-old female with complaints of upper and lower limb pain with blackish discoloration along the veins. During hospital stay, she developed breathlessness and limb pain limiting her daily activities. As her right lower limb peripheral pulses were feeble lower limb and abdominal Doppler was performed which showed thrombosis of right common femoral and superficial artery thrombosis and concentric thickening of theaorta, celiac trunk, and superior mesenteric artery, respectively. Computed tomography aortogram supported Doppler findings and two-dimensional echo showed pulmonary artery hypertension and dilated right chambers of the heart. She was started on systemic steroids and anticoagulation to which she fairly responded and she was discharged with maintenance steroids. Hence, we suggest early and prompt diagnosis of TA in young females can improve the condition and reduce the sufferings.

A Case of Takayasu Arteritis in a Male Patient

Takayasu arteritis (TA), otherwise called “pulseless disease”, is a category of primary systemic vasculitis involving medium and large arteries, including the aorta, its branches and the pulmonary and the coronary arteries. The presence of TA is often observed in women and the main population of patients develops their first symptoms between 15 years to 30 years. If left untreated, this disease may lead to the development of many comorbid renal and pulmonary obstructions causing lethal consequences. Here we present a case report of the diagnosis of TA in a 25-year-old male, treatment and following procedures. A patient with no anamnesis was hospitalized with paralysis of the right half of body and altered consciousness. He had been experiencing symptoms like fatigue, general weakness and respiratory distress for the past year. After extensive medical analysis patient was treated with oral prednisone and later endarterectomy was performed. After the operation, the patient improved and was discharged. Till date, no new complaints were reported.

The effect of late diagnosis on survival and risk of vascular complications in Takayasu's arteritis patients

Aim. To assess the effect of late diagnosis on survival and risk of vascular complications in Takayasu's arteritis (TA) patients.  Methods. 183 patients with TA who were treated at the Sverdlovsk Regional Clinical Hospital No. 1 during the period from 01.01.1979 to 31.04.2018 were examined. There were 139 women and 44 men included, median age at the time of diagnosis being 35 [24; 44] years in females and 34 [26.5; 42] years in males and a median interval from the first symptoms onset to the diagnosis of 3 [1; 7] and 4 [1.5; 8] years respectively. The diagnosis of TA met the ACR criteria (1990).Results. During the follow-up period, 31 deaths were observed (18 in males and 13 in females). The median age of death for women was 36 [32-44] years, for men - 50 [40-57] years. Cardiovascular complications were recorded in 72 patients (27 men and 45 women). Vascular complications and namely acute cerebrovascular accident observed in 31 (43%) patients, including ischemic stroke in 24 (35%), transient ischemic attack in 3 (3%) and hemorrhagic stroke in 4 (6%) were the most frequent. Arterial thromboses were registered in 32 (44%) cases. In 162 (88.5%) patients, the diagnosis was established more than six months after the appearance of the first symptoms.At the primary physician visit in 71 (39%) patients, the initial diagnosis was erroneous. The most common symptoms they had were regarded as a manifestation of arterial hypertension (in 18% cases). 4 years or more interval from the onset of TA symptoms to diagnosis was associated with a significant cardiovascular events odds increase (OR=1.8; 95% CI: 1.07-3.34) and premature death by the 5th year of follow-up (OR=2.9; 95% CI: 1,27-6,55).Conclusion. In the retrospective TA cohort setting, late diagnosis verification and delayed treatment initiation were associated with an increased risk of premature death and severe vascular complications, described earlier in other samplings. Timely diagnosis and as early as possible appropriate treatment administration can prevent the disease progression and disabling complications occurrence.