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

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

Escherichia coli (E coli) O157 may cause abdominal pain and diarrhea followed by hematochezia. Most of cases resolve spontaneously after several days. Takayasu's arteritis affects medium- and large-sized arteries, aortic arch and its branch, and rarely affects inferior mesenteric artery. In case of Takayasu's arteritis with hematochezia, we must distinguish among ulcerative colitis, ischemic colitis, and infectious colitis with Takayasu's arteritis. We report a case of 17-year-old woman who suffered from hemorrhagic colitis by E. coli O157, and combined with leg claudication and abdominal pain by Takayasu's arteritis that affected abdominal aorta and inferior mesenteric artery. Sigmoidoscopy showed edematous, hyperemic mucosa and superficial ulcerations in the sigmoid colon. Abdominal CT scan showed diffuse submucosal edema, narrowing of distal abdominal aorta and inferior mesenteric artery. Hematochezia disappeared after the conservative treatment and leg claudication and abdominal pain disappeared after the aortic angioplasty.
Adolescent ; Angiography ; Colitis/*diagnosis/etiology/pathology ; Escherichia coli Infections/complications/*diagnosis ; *Escherichia coli O157 ; Female ; Gastrointestinal Hemorrhage/*diagnosis/etiology ; Humans ; Imaging, Three-Dimensional ; Sigmoidoscopy ; Takayasu Arteritis/*radiography/therapy ; Tomography, Spiral Computed

Ischemic colitis is one of the most common intestinal ischemic injury in which more than 90% of patient are over 60 year-old. It results from impaired perfusion of blood to the bowel and is rarely caused by vasculitis such as systemic lupus erythematosus, polyarteritis nodosa, and Takayasu's arteritis. Takayasu's arteritis affects the aortic arch, medium-sized and large arteries but rarely involves inferior mesenteric artery. We report a case of Takayasu's arteritis involving inferior mesenteric artery which developed ischemic colitis in a 70 year old female. To the author's knowledge this is the first case report in Korea. A 70 year old woman who had suffered from Takayasu's arteritis for 5 years was admitted for sudden abominal pain and hematochezia. On sigmoidoscopy, there were multiple segmental longitudinal ulcerations around splenic flexure and diffuse hemorrhagic edematous mucosa from descending colon to sigmoid colon. On abdominal CT angiography, inferior mesenteric artery was not traced. We diagnosed it as ischemic colitis combined with Takayasu's arteritis. After the conservative treatment, abdominal pain and hematochezia disappeared. She was followed up to 2 years without recurrence of symptoms.
Abdominal Pain/diagnosis ; Aged ; Colitis, Ischemic/*diagnosis/etiology/pathology ; Female ; Gastrointestinal Hemorrhage ; Humans ; Imaging, Three-Dimensional ; Sigmoidoscopy ; Takayasu Arteritis/complications/*diagnosis/pathology ; Tomography, Spiral Computed

Takayasu's arteritis is a chronic inflammatory disease that produces a narrowing of the aorta and its major branches. Fibrosis and thickening of the arterial wall often occur in later stages, resulting in a cerebrovascular accident. The authors report two young women patients who presented with subarachnoid hemorrhage (SAH) and occlusive cerebrovasular disease associated with Takayasu's arteritis. Both patients had sudden headache and hemiparesis. Physical examination showed weak radial pulse, carotid bruit, and asymmetrical blood pressure. Erythrocyte sedimentation rate (ESR) was elevated in both patients. SAH was confirmed by brain computerized tomography (CT) or lumbar puncture. Occlusive cerebrovascular disease was diagnosed by brain magnetic resonance imaging (MRI), brain magnetic resonance angiography (MRA), and cerebral angiography. The findings of aortography and cerebral angiography were compatible with Takayasu's arteritis, but intracranial aneurysm was not found in either patient.
Adult ; Cerebral Angiography ; Cerebrovascular Disorders/complications/diagnosis ; Female ; Humans ; Subarachnoid Hemorrhage/*complications/diagnosis ; Takayasu Arteritis/*complications/diagnosis ; Tomography, X-Ray Computed