This article reports a male patient，aged 31 years，who were admitted due to sudden-onset speech and language impairment and limb weakness at the right side for more than 7 hours and achieved successful revascularization after endovascular treatment. Etiological screening revealed positive treponema pallidum particle agglutination （1∶1 280） and positive HIV antibodies in serum，as well as an increase in white blood cell count and positive TPPA （1∶640） in cerebrospinal fluid （CSF）. High-resolution magnetic resonance imaging of the vessel wall showed inflammatory changes in the vessel wall of the M1 segment of the left middle cerebral artery. After multidisciplinary consultation，the patient was diagnosed with acute ischemic stroke secondary to cerebral arteritis caused by neurosyphilis and HIV infection and was given antiplatelet therapy，benzathine penicillin for syphilis，and antiviral therapy. After 6 months of follow-up，the patient had a modified Rankin Scale score of 0 and achieved clearance of syphilis-related indicators in serum and CSF，and CT angiography confirmed revascularization without new-onset stenosis. The literature review shows that acute ischemic stroke is a common manifestation of co-infection with HIV and neurosyphilis in young individuals，and early syphilis detection in serum and CSF，HIV screening，and timely combination therapy are of great significance for improving prognosis.
Neurosyphilis ; Arteritis

BACKGROUND

Takayasu Arteritis (TA) is a rare, primary large-vessel vasculitis frequently leading to stenosis and less commonly, aneurysm formation. Saccular aneurysms of the aortic arch in patients with TA are fatal, have rarely been reported and represent a significant technical challenge due to the difficult anatomical location and need for protection of the cerebral circulation. Concomitant intracerebral aneurysms in patients with TA are extremely uncommon and have mostly been documented in very few case reports in literature.

We present a case of a 31 year-old Filipino female with recurrent chest and neck pain radiating to the upper back. Computed tomographic (CT) angiography demonstrated a large saccular aortic arch aneurysm without branch stenosis. CTA of the cerebral circulation likewise demonstrated multiple, saccular, intra-cerebral aneurysms. She underwent hybrid thoracic arch repair with supra-aortic debranching via mini-sternotomy and proximal ligation of the left common carotid artery and staged endovascular aortic arch replacement with coil embolization of the ostial-to-proximal left subclavian artery segment. Post-operative aortogram showed optimal repair with thrombosed aneurysmal sac, optimal graft position, no endoleaks and preservation of cerebral circulation. Patient improved symptomatically post-procedure and remained symptom-free during follow-up after six months. Careful review of local literature suggests that this is the first Philippine TA case with a saccular aortic arch aneurysm successfully managed in this manner.

Saccular aortic arch aneurysms in patients with Takayasu are unusual and presence of concomitant multiple cerebral saccular aneurysms have rarely been reported in literature. This case highlighted that hybrid endovascular arch repair in patients with TA is feasible, minimally invasive and effective.

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.
Humans ; Takayasu Arteritis ; Endothelial Cells ; Transcriptome ; Computational Biology ; Fibroblasts

OBJECTIVE: To compare the diagnostic efficiency of the 1990 American College of Rheumatology (ACR) classification criteria for Takayasu arteritis (TA) and the 2022 ACR classification criteria for TA in Chinese populations. METHODS: The clinical and imaging data of TA patients and patients with arterial stenosis or occlusion caused by atherosclerosis who were admitted to Peking University Third Hospital from May 2012 to May 2022 were retrospectively analyzed. Clinical diagnosis of TA by two rheumatologists were defined as the gold standard. The sensitivity, specificity, positive predictive value, negative predictive value, accuracy and the area under the receiver operating characteristics (ROC) curve (AUC) of the above two classification criteria were compared. In addition, this study also attempted to apply new imaging modalities, such as color Doppler ultrasound (CDUS), computed tomography angiography (CTA), magnetic resonance angiography (MRA) and 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/computed tomography (CT) in the 1990 ACR classification criteria to find whether this approach would improve the diagnostic efficiency. At the same time, the imaging features of the two groups were compared. RESULTS: The sensitivity (91.75%), positive predictive value (94.68%), negative predictive value (92.79%), accuracy (93.66%) and AUC (0.979) of the 2022 ACR TA classification criteria were better than those of the 1990 ACR TA classification criteria (45.36%, 91.67%, 66.24%, 72.20% and 0.855, respectively). In addition, we included new imaging modalities, such as CDUS, CTA, MRA and PET/CT in the 1990 ACR TA classification criteria, and the sensitivity, positive predictive value, negative predictive value, accuracy and AUC were significantly improved, which were 63.92%, 92.54%, 74.64%, 80.49% and 0.959, respectively, but still lower than those of the 2022 ACR classification criteria of TA (P < 0.001). The TA patients had more arterial stenosis (P=0.030), while the atherosclerosis patients had more arterial occlusion (P=0.021). There was no significant difference in arterial aneurysm or dissection (P=0.171). The TA patients had more involvement of ≥3 arteries (P=0.013), while the atherosclerosis patients had more unique artery involvement (P=0.011). CONCLUSION Compared with the 1990 ACR classification criteria for TA, the 2022 ACR classification criteria had higher diagnostic efficiency and might be more sui-table for the Chinese populations. Using more imaging modalities would improve the diagnostic perfor-mance of 1990 ACR classification criteria.
Humans ; Takayasu Arteritis/diagnostic imaging* ; Positron Emission Tomography Computed Tomography ; Retrospective Studies ; Constriction, Pathologic ; East Asian People ; Atherosclerosis

Objective: To evaluate the impact of interventional therapy on top of drug therapy on cardiac function and structure in heart failure with reduced ejection fraction (HFrEF) patients complicating with middle aortic syndrome caused by Takayasu arteritis (TA-MAS). Methods: It was a retrospective longitudinal study. The data of patients with TA-MAS and HFrEF, who received interventional therapy on top of drug therapy in Fuwai Hospital from January 2010 to September 2020, were collected and analyzed. Baseline clinical data (including demographic data, basic treatment, etc.) were collected through the electronic medical record system. Changes of indexes such as New York Heart Association (NYHA) classification, N-terminal pro-brain natriuretic peptide (NT-proBNP), left ventricular end diastolic diameter (LVEDD), left ventricular ejection fraction (LVEF), left ventricular mass index (LVMI) before and after therapy were analyzed. Results: A total of 10 patients were collected. There were 8 females in this patient cohort, age was (18.4±5.0) years and onset age was (15.3±5.0) years. All 10 patients received standard heart failure medication therapy in addition to hormone and/or immunosuppressive anti-inflammatory therapy, but cardiac function was not improved, so aortic balloon dilatation and/or aortic stenting were performed in these patients. The median follow-up was 3.3(1.3, 5.6) years. On the third day after interventional therapy, the clinical symptoms of the 10 patients were significantly improved, NYHA classfication was restored from preoperative Ⅲ/Ⅳ to Ⅱ at 6 months post intervention(P<0.05). Compared with preoperation, NT-proBNP (P=0.028), LVEDD (P=0.011) and LVMI (P=0.019) were significantly decreased, LVEF was significantly increased (P<0.001) at 6 months after operation. Compared with preoperation, NT-proBNP (P=0.016), LVEDD (P=0.023) and LVMI (P=0.043) remained decreased, LVEF remained increased (P<0.001) at 1 year after operation. Conclusion: Results from short and medium term follow-up show that interventional therapy on top of heart failure drug therpay can effectively improve left cardiac function and attenuate cardiac remodeling in patients with TA-MAS comorbid with HFrEF.
Adolescent ; Child ; Female ; Humans ; Young Adult ; Heart Failure/surgery* ; Longitudinal Studies ; Natriuretic Peptide, Brain ; Peptide Fragments ; Retrospective Studies ; Stroke Volume ; Takayasu Arteritis/surgery* ; Ventricular Function, Left/drug effects* ; Heart Ventricles/drug effects* ; Male ; Cardiovascular Agents/therapeutic use* ; Angioplasty, Balloon ; Stents ; Blood Vessel Prosthesis Implantation

Aorta ; Aortitis ; Cardiac Surgical Procedures ; Humans ; Immunosuppression ; Takayasu Arteritis/drug therapy*

Most of temporal arteritis occurs in the older patient over 50 years old, and the histopathologic finding shows a granulomatous inflammation, so this called giant cell arteritis. However, the young patients also present with a nodular lesion in their temple, and juvenile temporal arteritis (JTA) should be considered as one of the differential diagnosis, although it is very rare. For both diagnosis and treatment of JTA, excisional biopsy is essential. The pathologic finding of the temporal artery shows panarteritis with lymphoeosinophilic infiltrates, but no giant cell or granulomatous lesion. JTA is a localized disease with low level of systemic inflammatory marker, so the symptom is usually relieved by excision of affected lesion. Peripheral blood eosinophilia present in some cases of JTA, but its relation with clinical course and prognosis is not yet been known. Herein, we report the case of a 24-year-old man diagnosed with concurrent JTA and hypereosinophilic syndrome. We also reviewed the literature of JTA focusing on the impact of combined peripheral eosinophilia on the course of the disease. Combined peripheral eosinophilia may increase the risk of recurrence of JTA after local treatment such as excision only.
Biopsy ; Diagnosis ; Diagnosis, Differential ; Eosinophilia ; Giant Cell Arteritis ; Giant Cells ; Humans ; Hypereosinophilic Syndrome ; Inflammation ; Prognosis ; Recurrence ; Temporal Arteries ; Young Adult

Noninfectious aortitis, inflammatory abdominal periaortitis, and idiopathic retroperitoneal fibrosis are chronic inflammatory diseases with unclear causes. Recent studies have shown that some cases of aortitis are associated with immunoglobulin G4 (IgG4)-related systemic disease. Herein, we report a case of IgG4-related aortitis (IgG4-RA) that was diagnosed after surgery. Our patient was a 46-year-old man who had experienced abdominal pain for several weeks. Preoperative evaluations revealed an area of aortitis on the infrarenal aorta. He underwent surgery, and histological examination resulted in a diagnosis of IgG4-RA.
Abdominal Pain ; Aorta ; Aorta, Abdominal ; Aortitis ; Arteritis ; Diagnosis ; Humans ; Immunoglobulins ; Middle Aged ; Retroperitoneal Fibrosis

BACKGROUND AND PURPOSE: The aim of this study is to report the relative incidence of arteritic anterior ischemic optic neuropathy (AAION) associated with giant-cell arteritis (GCA) in a single-center and evaluate the clinical features of AAION in Korean patients. METHODS: The medical records of patients with presumed AION who visited our hospital from January 2013 to August 2018 were examined retrospectively. The patients were divided into two groups: AAION associated with GCA, and non AION (NAION). We additionally reviewed the literature and identified all cases of AAION in Korean and Caucasian patients. We evaluated the clinical data including the initial and final best-corrected visual acuities, fundus photographs, visual field tests, fluorescein angiography, and contrast-enhanced MRI, and compared the data with those for Caucasian patients in the literature. RESULTS: Of the 142 patients with presumed AION, 3 (2.1%) were diagnosed with AAION and 139 (97.9%) were diagnosed with NAION. Seven Korean patients with AAION associated with GCA were identified in our data and the literature review. We found no difference in any clinical features other than laterality: four of the seven Korean patients had bilateral involvement. Moreover, the optic nerve sheath was enhanced in two of our Korean patients. CONCLUSIONS: AAION associated with GCA is a very rare condition compared to NAION in Korea. However, GCA should be considered in all cases of ischemic optic neuropathy because AAION is associated with poor visual outcome, and sometimes presents bilaterally.
Arteritis ; Fluorescein Angiography ; Humans ; Incidence ; Korea ; Magnetic Resonance Imaging ; Medical Records ; Optic Nerve ; Optic Neuropathy, Ischemic ; Retrospective Studies ; Visual Acuity ; Visual Field Tests

BACKGROUND: Polymyalgia rheumatica (PMR) is a common systemic inflammatory disease of the elderly; however, the clinical characteristics and therapeutic response of PMR in Korea have been rarely studied. METHODS: We reviewed the medical records of 54 Korean patients diagnosed with PMR between January 2009 and February 2017 in a locomotive pain clinic of one tertiary referral hospital. We analyzed epidemiologic and clinical characteristics, therapeutic responses, and prognostic factors for remission-failure at one-year after oral prednisolone treatment. RESULTS: In 54 patients with PMR, 32 (59.3%) were female. The average age at diagnosis was 65.0 ± 10.5 years. Duration of symptoms before diagnosis was 8.1 ± 8.6 months. All patients had shoulder pain (54 patients, 100.0%); 49 patients (90.7%) had hip girdle pain, while 19 patients (35.2%) had peripheral joint pain. Four patients (7.4%) were accompanied by the giant cell arteritis (GCA). There was no seasonal preference for symptom development. Only 19 patients were diagnosed with PMR at initial symptom presentation. At one-year follow-up after oral prednisolone treatment, the remission rate was 35.3% (12/34). Multivariate analysis showed that history of relapse (odds ratio, 6.81; 95% confidence interval, 1.035–44.804) was a significant predictor of remission-failure. CONCLUSION: The rate of remission (35.3%) after oral prednisolone treatment was similar to previous reports in western countries; and GCA is not a rare condition in Korean PMR patients. Misdiagnosis of PMR is common, and heightened consideration for PMR is needed in elderly patients who present inflammatory features of bilateral shoulder pain.
Aged ; Arthralgia ; Cohort Studies* ; Diagnosis ; Diagnostic Errors ; Female ; Follow-Up Studies ; Giant Cell Arteritis ; Hip ; Humans ; Korea ; Medical Records ; Multivariate Analysis ; Pain Clinics* ; Polymyalgia Rheumatica* ; Prednisolone ; Recurrence ; Seasons ; Shoulder Pain ; Tertiary Care Centers ; Treatment Outcome