INTRODUCTION: Takayasu arteritis is the most common large-vessel vasculitis in childhood, but there is a lack of literature regarding childhood-onset Takayasu arteritis (c-TAK) in Southeast Asia. We aim to describe a c-TAK cohort in Singapore and highlight a unique subset that first presents with Kawasaki-like disease (KD). METHOD: A single-centre cohort study in Singapore of consecutive children diagnosed with c-TAK between 2002 and 2023 was performed. Demographic and clinical features, laboratory and angiographic findings, treatment, and outcomes were summarised. Disease activity was evaluated using the Paediatric Vasculitis Disease Activity Score and inflammatory markers. RESULTS: Twenty-three patients, fulfilling both the EULAR/ PRINTO/PReS and ACR/EULAR 2022 criteria, were recruited. The most common clinical features at diagnosis were fever (15, 65%) and neurological symptoms (11, 48%, half of which presented with stroke), while the most prevalent angiographic pattern by Hata's classification was Type V (21, 91%). Eight children (35%) initially presented with refractory KD, and these patients were significantly younger, more male-predominant, and had higher inflammatory markers at diagnosis; all of them had coronary artery involvement, but none had intracranial vascular findings. Of the entire cohort, 16 (70%) achieved inactive disease on medications with a median duration of 6 months (interquartile range [IQR]: 4-11), and 8 (35%) achieved remission off medications with a median duration of 43 months (IQR 35-60). CONCLUSION Our c-TAK cohort has high proportions of neurological involvement and stroke. This is also the first cohort study to describe a distinct group of patients who first presented with refractory KD.
Humans ; Takayasu Arteritis/complications* ; Singapore/epidemiology* ; Male ; Female ; Child ; Adolescent ; Age of Onset ; Mucocutaneous Lymph Node Syndrome/diagnosis* ; Cohort Studies ; Child, Preschool ; Fever/etiology* ; Stroke/epidemiology* ; Retrospective Studies

No abstract available.
Aged ; Antibodies, Antiphospholipid/blood ; Anticoagulants/therapeutic use ; Antiphospholipid Syndrome/blood/*complications/diagnosis/drug therapy ; Biomarkers/blood ; Female ; Fluorodeoxyglucose F18 ; Glucocorticoids/therapeutic use ; Humans ; Multimodal Imaging/methods ; Positron-Emission Tomography ; Radiopharmaceuticals ; Takayasu Arteritis/*complications/diagnosis/drug therapy ; Tomography, X-Ray Computed ; Treatment Outcome

Takayasu arteritis (TA) is a chronic vasculitis that affects the aortic arch and its primary branches. Ulcerative colitis (UC) is an inflammatory bowel disease of unknown etiology. Patients diagnosed with both TA and UC have rarely been reported. The pathogenesis of TA and UC is uncertain, but cell-mediated mechanisms play an important role in both diseases, and a genetic factor is thought to have an effect on the coincidence of these two diseases. We herein report a 38-year-old female with TA who had a history of UC with optic neuritis. We believe that this is the first case of the coexistence of TA and UC in Korea.
Adult ; Anti-Inflammatory Agents/therapeutic use ; Aortography/methods ; Colitis, Ulcerative/*complications/diagnosis/drug therapy ; Colonoscopy ; Female ; Humans ; Immunosuppressive Agents/therapeutic use ; Optic Neuritis/*complications/diagnosis/drug therapy ; Positron-Emission Tomography ; Republic of Korea ; Steroids/therapeutic use ; Takayasu Arteritis/*complications/diagnosis/drug therapy ; Tomography, X-Ray Computed ; Treatment Outcome

Adrenal Cortex Hormones ; therapeutic use ; Adult ; Aorta, Thoracic ; pathology ; Female ; Humans ; Prednisone ; therapeutic use ; Takayasu Arteritis ; complications ; drug therapy

Child ; Humans ; Male ; Moyamoya Disease ; etiology ; Takayasu Arteritis ; complications ; diagnosis

OBJECTIVETo investigate the clinical neurological manifestations of Takayasu arteritis (TA).

METHODSA retrospective study was conducted with 63 consecutive TA cases admitted to Peking Union Medical College Hospital from January 2009 to May 2010. All the patients fulfilled the diagnostic criteria of TA by the American College of Rheumatology. Among the 63 TA patients, 27 with neurological manifestations were included in the present study. All the patients were evaluated using standardized neurological examination, sonography, computed tomography (CT) angiography, and cerebral CT or magnetic resonance imaging.

RESULTSDizziness and visual disturbance were the most common symptoms, which occurred in 20 (74.1%) and 16 (59.3%) patients respectively. Another common symptom was headache, observed in 15 (55.6%) patients. Six (22.2%) patients had suffered from ischemic stroke; 7 (25.9%) patients had epileptic seizures. Two (7.4%) patients were diagnosed as reversible posterior encephalopathy syndrome (RPES) based on typical clinical and imaging manifestations.

CONCLUSIONSNeurological manifestations are common symptoms in TA patients in the chronic phase, including dizziness, visual disturbance, headache, ischemic stroke, seizures, and some unusual ones such as RPES. We suggested RPES be included into the differential diagnosis of acute neurological changes in TA.

Adolescent ; Adult ; Child ; Dizziness ; etiology ; Female ; Headache ; etiology ; Humans ; Male ; Middle Aged ; Nervous System Diseases ; etiology ; Retrospective Studies ; Seizures ; etiology ; Stroke ; etiology ; Takayasu Arteritis ; complications

OBJECTIVETo clarify the outcome of surgical reconstruction for renal artery in Takayasu arteritis-induced renal artery stenosis (TARAS).

METHODSA retrospective chart review was conducted on 33 consecutive patients with TARAS, who underwent aortorenal bypass (ARB) with autologous saphenous vein graft. There were 9 male and 24 female patients, with a mean age of (25 ± 11) years. The effects on blood pressure and renal function were analyzed. Primary, primary assisted, and secondary patency rates were determined. The effects of various factors on primary patency rate were analyzed. All patients showed hypertension. The mean blood pressure was (175 ± 26)/(100 ± 19) mmHg (1 mmHg = 0.133 kPa). The mean antihypertensive dosage was (2.1 ± 0.6) defined daily dose (DDD). Seventeen patients showed intractable hypertension. Mean estimated glomerular filtration rate was (78 ± 5) ml/min. One patient was dialysis-dependent, and 3 patients were combined with congestive heart failure.

RESULTSARB was performed for the 39 renal arteries, including 27 unilateral and 6 bilateral bypasses. Postoperative morbidity was 15.2%. All patients survived. During follow-up of mean (56 ± 18) months, two graft occlusions and four graft restenoses occurred. All graft restenoses were eliminated successfully with percutaneous angioplasty, but one patient experienced restenosis again six months later. At 1, 3, and 5 years of follow-up, primary patency was 92%, 89%, and 79%, respectively, primary assisted patency was 95%, 95%, and 91%, respectively, and secondary patency was 95%, 95%, and 91%, respectively. ARB resulted in a decrease in mean blood pressure to 139/85 mmHg (one month post-ARB, P = 0.000) and 136/80 mmHg (last follow-up, P = 0.000), and a reduction in mean antihypertensive dosage to 1.4 DDD (one month post-ARB, P = 0.084) and 0.6 DDD (last follow-up, P = 0.000). Mean estimated glomerular filtration rate increased to 82 ml/min (P = 0.458) one month post-ARB, and 91 ml/min (P = 0.044) at last follow-up, respectively. The dialysis-dependent patient no longer required hemodialysis, and left ventricular dysfunction resolved in all of the three patients.

CONCLUSIONARB using the autologous saphenous vein graft is safe, effective and durable for treating TARAS.

Adolescent ; Adult ; Aorta ; surgery ; Child ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Renal Artery ; surgery ; Renal Artery Obstruction ; etiology ; surgery ; Retrospective Studies ; Saphenous Vein ; transplantation ; Takayasu Arteritis ; complications ; Treatment Outcome ; Young Adult

BACKGROUNDTakayasu's arteritis (TA) is a chronic idiopathic inflammatory disease that affects large and medium size arteries. The brachiocephalic trunk is the most frequently involved site in TA, and multi-vessel lesions are common. Surgical treatment includes vessel reconstruction surgery and percutaneous transluminal angioplasty (PTA). Herein, we report our preliminary experience with surgical treatment of cerebral ischemia caused by cervical arterial lesions due to TA.

METHODSFrom January 2000 to December 2007, 38 patients with cerebral ischemia caused by cervical arterial occlusive lesions due to TA were treated surgically. There were three males and 35 females, with an age range of 15 - 42 years (mean 26.5 years). All patients had operative repairs undertaken. Twenty eight patients received bypass operation and 10 patients received percutaneous transluminal angioplasty. One case with coronary stenosis received coronary artery bypass simultaneously. Patients were followed up for 11 months to eight years.

RESULTSThere were no peri-operative deaths in cerebrovascular reconstruction patients. Symptoms of cerebral ischemia were improved or cured in 25 of 38 patients. There was a low incidence of cerebral reperfusion syndrome. Two patients died at five and seven years after surgery due to heart failure. Another 8 patients (20%) required further surgery for stenosis (5 patients) or anastomotic aneurysms (3 patients). Percutaneous transluminal angioplasty was performed successfully for treatment of aortic and renal lesions. Repeated angioplasty for revascularization was performed in six PTA cases with restenosis after 5 - 24 months.

CONCLUSIONSWhen cerebral perfusion has potential to be affected by TA, a definitive corrective procedure is advised when the patient is relatively stable. Although the recurrence rate is very high, percutaneous transluminal angioplasty is the first choice procedure. Bypass operation is optimal for brachiocephalic-vessel involvement in TA. Cerebral reperfusion syndrome can be avoided by careful selection of the operation method and improved post-operative treatment.

Adolescent ; Adult ; Arteritis ; drug therapy ; Brachiocephalic Trunk ; pathology ; surgery ; Brain Ischemia ; etiology ; pathology ; surgery ; Cerebral Revascularization ; methods ; Female ; Humans ; Male ; Methylprednisolone ; therapeutic use ; Takayasu Arteritis ; complications ; pathology ; surgery ; Treatment Outcome ; Young Adult

OBJECTIVETo evaluate the effect of surgical bypass and to explore the role of transcranial doppler (TCD) during the bypass operation.

METHODSFrom March 2003 to February 2008, sixteen patients (4 male and 12 female) with mean age of 32 years old and mean disease course of 7.5 years were treated by surgical procedures. The main clinical presentations were dizziness, headache, vertigo, and visional dysfunction. Variated degree of artery stenoses in the 4 arteries (bilateral carotid and vertebral arteries) were revealed by color doppler ultrasonography and DSA. Eight patients underwent aorto-bi-subclavian arteries prosthetic graft bypass. Three patients underwent aorto-bi-subclavian artery prosthetic graft bypass and graft-unilateral carotid artery bypass with autologous great saphenous vein. Three patients underwent aorta-unilateral subclavian artery-unilateral carotid artery prosthetic graft bypass. Two patients underwent aorta carotid artery bypass with autologous great saphenous vein, of which one patient underwent aortocoronary artery bypass simultaneously. Cerebral blood supply was monitored in 14 patients with transcranial doppler. Unilateral subclavian carotid and femoral carotid artery shunt was used respectively to avoid cerebral ischemia during operation in 2 patients.

RESULTSSymptoms and signs of cerebral ischemia improved in all patients with effective rate of 100% apart from deflected tongue-protrusion in 3 patients which recovered in 2 weeks after operation. All patients survived and no symptoms recurred at the end of a 2.2 year's follow-up. Unfortunately, two patients developed aneurysm at the anastomosis within 4 years after operation.

CONCLUSIONSArterial reconstruction is an effective method for Takayasu's disease with severe cerebral ischemia. TCD monitoring plays an important role during the bypass operation. It can help to determine the revision of blood pressure and prevent postoperative brain reperfusion injury.

Adult ; Brain ; blood supply ; Brain Ischemia ; etiology ; surgery ; Cerebral Revascularization ; methods ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Monitoring, Intraoperative ; Regional Blood Flow ; Takayasu Arteritis ; complications ; surgery ; Treatment Outcome ; Ultrasonography, Doppler, Transcranial ; Young Adult

Carbazoles ; therapeutic use ; Child, Preschool ; Heart Failure ; complications ; drug therapy ; Humans ; Male ; Propanolamines ; therapeutic use ; Takayasu Arteritis ; complications ; drug therapy ; Vasodilator Agents ; therapeutic use