Takayasu Arteritis Patients Research Articles

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.

Right Ventricular Function in Takayasu's Arteritis Patients With Pulmonary Artery Involvement Using MRI Feature Tracking.

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. 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. Retrospective. 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. 3 T/Cine imaging sequence with a steady-state free precession readout. 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. 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. 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. 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. 3 TECHNICAL EFFICACY: Stage 3.

Myocardial and Vascular Involvement in Patients with Takayasu Arteritis: A Cardiovascular MRI Study

We aimed to explore the cardiovascular magnetic resonance (CMR) of Takayasu arteritis (TA) and its cardiovascular complications. CMR was conducted on 37 TA patients and 28 healthy individuals. We evaluated the CMR findings and adverse cardiovascular complications at the time of the CMR (ACCCMR). After 8 to 26 months, the major adverse cardiac and cerebrovascular events (MACCEs) were evaluated. The TA included 25 women (67.6%), aged 36 ± 16 years old, and 28 age- and sex-matched healthy controls. Left ventricular (LV) ejection fraction was significantly lower in the TA group than in the control group (51 ± 9% vs. 58 ± 1.7%; p &lt; 0.001). Aortic mural edema was present in 34 patients (92%) and aortic mural hyperenhancement in 36 (97%). Left ventricular global longitudinal strain (LVGLS) was significantly lower in the TA group (median [interquartile range] = 13.70 [3.27] vs. 18.08 [1.35]; p &lt; 0.001). ACCCMR was seen in 13 TA patients (35.1%), with the most common cardiac complication being myocarditis (16.2%). During a median follow-up of 18 months (8–26 months), nine patients developed MACCEs, of which the most common was cerebrovascular accident in five (13.5%). The LVGLS of the CMR had the strongest association with complications. Myocardial strain values, especially LVGLS, can reveal concurrent and future cardiovascular complications in TA patients.

Maternal and fetal outcomes in pregnant women with Takayasu’s arteritis: single center experience over ten years

Background: This study aims to assess pregnant women's maternal and fetal outcomes with Takayasu’s arteritis (TA).&#x0D; 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.&#x0D; 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%).&#x0D; 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.

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

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. 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 60min 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. 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). 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.

EFFICACITY OF COMBINATION ACID ACETYLSALICYLIC AND GLUCOCORTICOID IN TAKAYASU ATERITIS TREATMENT: A CASE CLINIC TAKAYASU ARTERITIS IN VIET NAM

Objective: This brings up the potential of medical management for patients who are diagnosed with Takayasu arteritis, instead of using invasive methods such as aortic stent placement or operations. Design and method: A young female 14 years old was diagnosed with Takayasu arteritis. Initial symptoms include fever, loss of appetite, weight loss, muscle pain, followed by palpitations and abdominal pain, and loose stools. The test showed an increased hypersensitive C-reactive protein level (CRPhs: 28.7 mg/L). Computed tomography angiography (CTA) chest with contrast shows two segments of thoracic aneurysms involving descending aorta, the aortic diameter of the superior and inferior segment aneurysms is #1.6-1.8 cm, #2x 2.1cm, respectively, and the length is #2.5cm, # 3.2cm, respectively. According to the American College Rheumatology 1994, we classified this patient as type III. The patient was treated with corticosteroid therapy consisting of: prednisolone at an initial dose of 20 mg/day, reduced to a dose of 10 mg/day at month 3, and reduced to a dose of 5 mg/day after 6 months until now. In addition, we also added low-dose aspirin (81mg/day), ACE inhibitors, and beta-blockers to increase the effectiveness of treatment Results: After 3 months, Hs-CRP levels returned to normal (from 28.7 mg/L to 1.23 mg/L) and remained below the 3 mg/L thresholds during the next 15 months. After 12 months of combined treatment with corticosteroids and aspirin as described above, the patient's aortic diameter had returned to almost normal on CTA. After that, we have reduced the patient's corticosteroid dose (prednisolone 5 mg/day) and are going to finish the treatment after 18 months. Conclusions: This successful case high value on the role of pharmacological therapy in cases of Takayasu arteritis. It also brings up a debate regarding the concept of surgery required in cases of Takayasu arteritis complicated with dilatation, aneurysm, or stenosis. The combination of glucocorticoid therapy with aspirin can reduce the cost of treatment as well as avoids the side effects of the combination of glucocorticoid with immunosuppressive drugs. This opens up great treatment opportunities for Takayasu arteritis patients in low-economic countries like Vietnam.

IMAGING ASSESSMENT OF INTRACRANIAL VESSELS AND BRAIN PARENCHYMA IN TAKAYASU ARTERITIS AND ITS RELATION WITH MACARONI SIGN IN CERVICAL CAROTID ARTERIES

Objectives - Aim of this study to evaluate the imaging ndings of intracranial vascular and parenchymal involvement in Takayasu arteritis and to correlate these ndings with Macaroni sign in cervical carotid arteries. Materials and Methods: This is a cross-sectional study with total of 19 study subjects. We have evaluated each patient by ultrasonography of cervical carotid vessels. Subsequently each patient underwent MRI brain and MRA of cerebral vessels. We have assessed correlation of Macaroni sign in cervical carotid arteries with intracranial vascular and parenchymal involvement. Result: our study shows Maximum number of patients (63%) belonged to the age group (25-35) years with female predominance. Neurological involvement in the form of history of stroke were present in 31.5% of cases. In our study 19 patients of Takayasu arteritis the neck vessel involvement in the form of macaroni sign, were very common approximately 63.2% of cases. In Macaroni sign the echogenicity of intimamedia thickness were hyperechoic in most of the cases. The maximum thickness of the involved arterial wall was 2.7mm (mean ± SD, 1.14±0.66mm). Two patients of Takayasu arteritis showed calcic plaque with signicant stenosis and one patient also had the reverse ow in vertebral artery. Macaroni sign is a strong predictor for the intracranial cerebral vessels involvement in Takayasu arteritis. Conclusion: CCA involvement and Macaroni sign are very common as compared to cervical part of ICA in Takayasu arteritis. Takayasu arteritis is an unusual etiology for neurological brain parenchymal vascular insult. Ultrasonography (USG) is a simple, portable, bedside, non-invasive, standard realtime, quite sensitive and superior imaging method to detect the early changes in the wall of the arteries, progression of disease and indirect predictor of brain parenchymal vascular insult.

Letter to the Editor Regarding “Natural History of Intracranial Arachnoid Cysts”

This study aims to observe and analyze the clinical efficacy of interventional therapy for patients with Takayasu arteritis (TA) experiencing renovascular hypertension (RH).Eight TA patients with RH underwent percutaneous transluminal renal artery stenting (PTRAS). Patients were followed up 1, 6, 12, and 24 months postoperatively for levels of blood pressure, number of antihypertensive drugs being taken, levels of serum creatinine, and the presence of renal artery restenosis.All 8 patients were successfully followed up 1, 6, and 12 months postoperatively, but 1 was lost to follow-up at 24 months. All patients had significantly lower average blood pressure levels compared with those at baseline (P < 0.05); treatment efficacy rates (recovery or improvement) at 1, 6, 12, and 24 months were 94%, 90%, 80%, and 80%, respectively. The average number of antihypertensive drugs being taken was 3.5 at baseline, 1.0 at 1 month, 0.5 at 6 months, 1.0 at 12 months, and 1.5 at 24 months. Serum creatinine levels during the follow-up period were not significantly different from those at the baseline. No patient developed renal artery restenosis during the follow-up period.PTRAS is a safe and effective treatment for TA-associated RH, with a high technical success rate and a low complication rate. This interventional therapy can effectively control TA-related hypertension and can also preserve and even improve kidney function.

Clinical characteristics and risk factors of coronary artery lesions in chinese pediatric Takayasu arteritis patients: a retrospective study

BackgroudTo summarize the clinical characteristics and identify the risk factors for pediatric Takayasu arteritis (TAK) with coronary artery lesions (CALs).MethodsClinical data of pediatric TAK patients in our center were retrospectively assessed. Independent risk factors for CALs were identified using multivariate logistic regression analysis. Survival analysis was used to compare differences in survival rates between the groups.ResultsAmong the 66 pediatric TAK cases, the incidence of accompanying CALs was 39.4%. In the CAL group, 19 (73.1%) cases started within 36 months. None of the patients had symptoms of angina or ischemia on electrocardiogram (ECG), the CALs were detected using coronary ultrasound. The CALs most commonly were the left main and right coronary arteries. The lesions were mostly small or middle coronary artery aneurysms; some children may have giant coronary aneurysmal dilations, thrombosis and heart failure. The age of onset and symptom onset to diagnosis in TAK patients with CAL were lower than those in TAK patients without CAL(P < 0.005). TAK patients with CAL had significantly higher CRP,WBC, PLT,TNF-α and IL-2R levels (P < 0.05), lower HGB (P = 0.01), lower rate of renal artery stenosis (RAS) (P = 0.009). In multivariate logistic regression, the risk factors for pediatric TAK combined with CAL included the age of TAK onset (OR = 0.9835, 95% CI: 0.9710–0.9946, P = 0.006) and RAS (OR = 0.1901, 95% CI: 0.0386–0.7503, P = 0.03). In addition, there was no significant difference in survival rates between the two groups after regular treatment.ConclusionThis study showed that the occurrence of CAL in pediatric TAK patients has a relatively more rapid clinical course, and a stronger inflammatory state at the time of diagnosis. The earlier the age of TAK onset and without RAS are more likely to cause CAL.

Acute left heart failure caused by Takayasu arteritis: a case report and literature review.

Takayasu arteritis is a rare disease. Coronary involvement may appear in patients with Takayasu arteritis. With delayed diagnosis and the difficulty of treatment, Takayasu arteritis patients complicated with coronary abnormalities usually have poor prognosis. We present a rare case of acute left heart failure caused by total occlusion of the left main coronary artery due to Takayasu arteritis. A 30-year-old Chinese woman presented at our hospital with recurrent chest tightness accompanied by dyspnoea. A series of modern imaging methods were used for diagnosis and evaluation of Takayasu arteritis, including invasive angiography, CT angiography, and vascular ultrasound. The patient received drugs therapy including glucocorticoids, immunosuppressants, and cardiovascular drugs, without a reperfusion therapy. Cardiac events, inflammatory marks, and cardiac function were observed during 2-year follow-up period. In this paper, we briefly disscuss the diagnosis and treatment for young women with cardiac complication caused by Takayasu arteritis.

Clinical and vascular features of stroke in Takayasu's arteritis: A 24-year retrospective study.

To investigate the clinical characteristics, vascular imaging features, and prognosis of Takayasu's arteritis (TA) patients with stroke in China. Medical charts of 411 in-patients who fulfilled the classification criteria of modified 1990 American College of Rheumatology (ACR) criteria for TA and with complete data from 1990 to 2014 were reviewed retrospectively. The demographic data, symptoms and signs, laboratory test results, radiological features, treatment, and interventional or surgical procedures were collected and analyzed. Patients with radiological confirmed stroke were identified. Chi-square test or Fisher exact test was used to compare the differences between patients with and without stroke. Twenty-two patients with ischemic stroke (IS) and 4 patients with hemorrhagic stroke were identified. The incidence of stroke in TA patients was 6.3% (26/411), of which 11 patients were considered to be the initial manifestation. Stroke patients had more visual acuity loss (15.4% vs. 4.7%, P = 0.042). Systemic inflammatory symptoms and inflammatory markers were less common in patients with stroke than in those without stroke [fever P = 0.007; erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP), P < 0.001]. Cranial angiography showed that common carotid artery (CCA) (73.0%, 19/26) and subclavian artery (SCA) (73.0%, 19/26) were the most involved, followed by internal carotid artery (ICA) (57.7%, 15/26) in stroke patients. The intracranial vascular involvement rate of stroke patients was 38.5% (10/26); the middle cerebral artery (MCA) was the most common artery involved. The most common site of stroke was the basal ganglia region. The occurrence of intracranial vascular involvement was much higher in patients with stroke when compared to patients without stroke (38.5% vs. 5.5%, P < 0.001). Among all patients with intracranial vascular involvement, patients without stroke received more aggressive treatment than patients with stroke (90.4% vs. 20.0%, P < 0.001). There was no significant increase in in-hospital mortality in patients with stroke compared with patients without stroke (3.8% vs. 2.3%, P = 0.629). Stroke is the initial presentation in 50% of TA patients with stroke. The intracranial vascular involvement rate is significantly increased in stroke patients than in patients without stroke. The artery invloved in patients with stroke are cervical artery and intracranial involvement. Systemic inflammation is less in patients with stroke. Aggressive treatment for TA with glucosteroid (GC) and immunosuppressive agents combined with anti-stroke therapy is needed to improve the prognosis of TA complicated stroke.

Association of Killer Cell Immunoglobulin-like Receptor (KIR) Genes and their HLA Ligands with Susceptibility to Takayasu Arteritis in the Iranian Population.

Takayasu arteritis (TA) is a chronic inflammatory disorder characterized by vascular damage and fibrosis in the intima that commonly occurs in the aorta. In many damaged sites in TA patients, natural killer (NK) cells have been shown to be hyperactivated and produce inflammatory cytokines and toxic components. Killer cell immunoglobulin-like receptors (KIRs) are found on NK cells and interact with human leukocyte antigen (HLA) class I ligands to activate or suppress NK cells. The present study assessed the possible role of KIR and their HLA ligand genes in susceptibility to TA in Iranian patients. This case-control study included 50 TA patients and 50 healthy subjects. DNA was extracted from whole peripheral blood samples, and polymerase chain reaction with sequence-specific primers (PCR-SSP) was performed to recognize the presence or absence of polymorphism in 17 KIR genes and 5 HLA class I ligands in each participant. Among the KIR and HLA genes, a significant decrease was detected in the frequency of2DS4 (full allele) in TA patients (38%) compared with healthy controls (82%) (OR=0.13, 95% CI=0.05-0.34). However, none of the KIR and HLA genotypes or the interactions between these genes were associated with susceptibility to TA. The KIR2DS4 gene might be involved in the regulation of activation as well as the production of cytotoxic mediators of NK cells in patients with TA.

Preliminary Results of Single-cell Transcriptome Sequencing in Renal Arterial Lesions of Takayasu Arteritis

Objective To explore the preliminary application of single-cell RNA sequencing (scRNA-seq) in the renal arterial lesions in Takayasu arteritis (TA) patients. Methods This study included 2 TA patients with renal artery stenosis treated by bypass surgery in the Department of Vascular Surgery,Beijing Hospital.The obtained 2 renal artery samples were digested with two different protocols (GEXSCOPE kit and self-made digestion liquid) before scRNA-seq and bioinformatics analysis. Results A total of 2920 cells were obtained for further analysis.After unbiased cluster analysis,2 endothelial cell subsets,2 smooth muscle cell subsets,1 fibroblast subset,2 mononuclear macrophage subsets,1 T cell subset,and 1 undefined cell subset were identified.Among them,the two subsets of smooth muscle cells were contractile and secretory,respectively.The results of scRNA-seq indicated that enzymatic hydrolysis with GEXSCOPE kit produced a large number of endothelial cells (57.46%) and a small number of immune cells (13.21%).However,immune cells (34.64%) were dominant in the cells obtained by enzymatic hydrolysis with self-made digestive liquid. Conclusion scRNA-seq can be employed to explore the cellular heterogeneity of diseased vessels in TA patients.Different enzymatic digestion protocols may impact the proportion of different cells.

Clinical and vascular lesion characteristics of the patients with takayasu arteritis manifested firstly as acute myocardial infarction at onset

ObjectiveTo explore clinical and vascular lesion characteristics of the patients with Takayasu arteritis (TA) manifested firstly as acute myocardial infarction (AMI) at onset and to improve the diagnostic rate of TA. MethodsThe clinical and angiographic data of six patients with TA manifested firstly as AMI at onset were retrospectively analyzed. ResultsOf six patients (16–25 years old), 83.33% (five cases) was female, three patients had a history of hypertension and three patients did not have any medical history. One patient had intermittent effort chest tightness. On admission patients all presented with chest pain, dyspnea, hypotension, cardiogenic shock, abnormal electrocardiogram, and elevated cardiac troponin I. The vessel involvement was left coronary main trunk 83.33%, left anterior descending artery 33.33% and left circumflex branch 16.67%, right coronary artery 66.67%, subclavian artery 83.33%, and renal artery 50%. Five patients received the emergency PCI. One patient died of heart failure. During follow-up 3 patients received again PCI treatment. ConclusionClinical and vascular lesion characteristics of those patients were no discomfort before admission, and the suddenly typical manifestation of AMI. Severe stenosis or occlusion occurred in main coronary artery ostia and peripheral large artery. For the TA patients with hemodynamic instability the effectiveness of emergency PCI is positive.

Predictors for pulmonary artery involvement in Takayasu arteritis and its cluster analysis

ObjectiveTo investigate the clinical characteristics and the site of pulmonary involvement in Takayasu arteritis (TAK) patients with pulmonary artery involvement (PAI).MethodsWe retrospectively investigated data of 141 TAK patients. The clinical and image data of the patients with and without PAI were analyzed and compared. The patients were followed up. The major outcome was all-cause mortality. The minor outcome was exacerbation or new occurrence of PAI, which leads to disease progression events.ResultsFor the 141 TAK patients considered, PAI was detected in 65 (46.1%) patients. TAK patients with PAI had a significantly higher cumulative incidence of events than those without PAI (P < 0.001). The frequencies of the following were significantly higher in TAK with PAI than those in TAK without PAI: disease duration [median 96 months (IQR: 24–174) vs. median 42 months (IQR: 6–120); P = 0.012], hemoptysis (10.8% vs. 1.32%; P = 0.040), oppression in the chest (40.0% vs. 21.1%; P = 0.014), fever (23.1% vs. 9.21%; P = 0.024), Mycobacterium tuberculosis infection (21.5% vs. 6.57%; P = 0.010), pulmonary hypertension (PAH) (21.5% vs. 2.6%; P < 0.001), pulmonary infarction (41.5% vs. 0%; P < 0.001), and hypoxemia (18.5% vs. 1.3%; P < 0.001). Multivariate logistic regression analysis of data of TAK patients with symptom presentation showed that oppression in the chest (OR: 2.304; 95% CI: 1.024–5.183; P = 0.044) and thoracic aorta involvement (OR: 2.819; 95% CI: 1.165–6.833; P = 0.022) were associated with PAI. The cluster analysis performed for data of TAK patients with PAI revealed that the cluster characterized as the upper lobe of the right lung (Cluster1) had the worst prognosis.ConclusionIn TAK, PAI is associated with thoracic aorta involvement. In TAK patients with PAI, the involvement of the upper lobe of the right lung is characterized with the worst prognosis.

Pregnancy outcomes in Takayasu arteritis patients: a systematic review and meta-analysis

Takayasu arteritis (TA) is a systemic disease affecting women of reproductive age. Similarly to other systemic autoimmune diseases, pregnancies in patients suffering from TA are at high risk for adverse outcomes; however, the precise incidence of adverse events has not been assessed in a systematic approach. The aim of this study was to evaluate the prevalence of adverse pregnancy outcomes in TA. Searches were conducted on PubMed, Cochrane Library, Scopus and Cinahl databases from inception to 25 May 2022. Three independent investigators extracted data and assessed the risk of bias using ROBINS-1 tool. We used a random effects model to calculate the prevalence of the adverse pregnancy outcomes in TA, namely miscarriage, hypertension and pre-eclampsia. We calculated the prevalence of the adverse outcomes in pregnancy for TA. We included 27 studies, with 825 pregnancies. The occurrence of miscarriage, hypertension and pre-eclampsia in patients with TA was 16% (CI 12–21%, p < 0.01), 37% (CI 30–45%, p < 0.01) and 14% (CI 8–23%, p < 0.01), respectively. The results of our meta-analysis indicate that pregnancies in patients with TA are at increased risk for adverse pregnancy outcomes compared to the general population, suggesting that pregnant women with TA should be closely monitored.Trial registration: There was no registration for this systematic review.

The impact of antiphospholipid antibodies in Takayasu arteritis.

The significance of antiphospholipid antibodies (aPL) is controversial in Takayasu arteritis (TA). This study was conducted to explore the frequency of aPL and their association with disease-related complications in TA. : This cross-sectional study was conducted to investigate the presence of anti-cardiolipin (aCL), anti-beta 2 glycoprotein- 1(aβ2G1) antibodies, and lupus anticoagulant (LA) in TA patients. TA patients admitted to the Department of Rheumatology of Hacettepe University Faculty of Medicine between December 2015 and September 2016 who fulfilled the American College of Rheumatology (ACR) classification criteria for TA were consecutively enrolled in the study. Patients were grouped according to aPL positivity and compared in terms of disease manifestations, type of vascular involvement at diagnosis, and vascular complications/interventions attributable to TA. Fifty-three TA (49 female) patients were enrolled in the study. We detected 9 (16.9%) patients with IgM and/or IgG aβ2G1 and/or LA positivity. There were no patients with positive aCL. All aβ2G1 titers were low. There were no differences in terms of symptoms, signs, type of vascular involvement, the number of patients with disease-related complications or vascular interventions/surgery between aPL (+) and aPL(-) groups (p > 0.05 for all). The number of patients with thrombotic lesions was similar between the groups (p > 0.05). There were no patients with a history of venous thrombosis or on anticoagulant treatment in the aPL(+) group. Only 1 patient with IgM aβ2G1 (+) had a history of pregnancy loss. Our results indicate that aPL positivity is not rare in TA. On the other hand, all aPL titers were low and no differences were found in the frequency of disease-related complications between aPL(+) and aPL(-) patient groups. Only TA patients with atypical manifestations with high suspicion of aPL-related complications should be considered to be investigated for aPL.

Anaesthesia for Thoracic Aortic Disease: Series of Four Cases

Thoracic aortic diseases involving the thoracic aorta, from the ascending aorta, the aortic arch to the descending aorta, can present in the form of aneurysms, dissection, tear and coarctation which usually lead to various complications, requiring surgical intervention. The same can be addressed surgically by ascending aorta replacement or reduction aortoplasty with/without Aortic Valve Replacement (AVR). The anaesthetic implications might vary depending on the pathology of the thoracic aortic disease which can be acute or chronic and, silent or symptomatic. Anaesthetic management of four patients (1 female and 3 males) with varied thoracic aortic diseases have been described in the series, including bicuspid aortic valve with severe aortic stenosis, Ischaemic Heart Disease (IHD) with severe Aortic Regurgitation (AR) with ascending aortic aneurysm, ascending aortic dilatation in a known Takayasu arteritis patient and coarctation of aorta with atrial septal defect. These patients underwent aortoplasty with or without aortic root replacement. Full cardiopulmonary bypass with Deep Hypothermic Circulatory Arrest (DHCA) at 16-20°C was the technique used for these procedures as it prevents stroke and ensures cognitive function. This technique had no additional cannulas, less chances of intimal injury or embolisation and clear surgical fields. During the process of rewarming, Inj. nitroglycerine was started which reduced preload, conserving the myocardium against ischaemic injuries. These patients were successfully managed perioperatively and were discharged with good outcomes postoperatively

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 &gt; 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 &gt; 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.

Prevalence and pattern of myocardial involvement on cardiac magnetic resonance imaging in Takayasu arteritis and its relationship with disease activity

BackgroundCardiac involvement in Takayasu arteritis (TA) is the major cause of morbidity and mortality. Cardiovascular magnetic resonance (CMR) is an excellent modality for the assessment of myocardial involvement. Studies have shown myocardial involvement in 25%–27% of patients. ObjectivesTo evaluate the prevalence and pattern of myocardial involvement in TA on CMR. We also evaluated any correlation between CMR changes and disease activity score (ITAS 2010 and ITAS-A) assessed at the time of CMR. MethodsPatients classified as Takayasu arteritis according to Sharma et al. criteria were enrolled in the study. Demographic, clinical, and laboratory data were documented in the predesigned proforma. CMR was performed on a dedicated cardiac 3Tesla MR machine. Disease activity was recorded by ITAS2010 and ITAS-A. ResultsA total of 37 TA patients were included. Mean (±SD) age was 29±11 years. Female to male ratio was 3:1. Five patients (14%) had myocardial involvement on CMR. Two (2/5) had myocarditis and three (3/5) patients had features of ischaemic myocardial fibrosis. ConclusionThe myocardium is affected in TA, however the prevalence of subclinical myocardial involvement in our study was less (8% vs. 25%–27%) compared to the previous studies. Myocardial involvement trends towards early age of onset, less disease duration, lack of classical risk factors, and more with disease activity.