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

An 11-year-old boy presented with erythematous plaques over the bilateral mandibular and mental regions for 2 years, accompanied by cough and dyspnea for more than 2 months. Chest computed tomography angiography revealed marked stenosis of the right pulmonary artery, irregular aortic caliber, and aortic wall thickening. Histopathological examination of the skin lesion, including immunohistochemistry and special stains, confirmed a chronic suppurative inflammation. Whole-exome sequencing was negative. A final diagnosis of granuloma faciale and Takayasu arteritis was established. Combination therapy with systemic tocilizumab, prednisone, and methotrexate, along with topical 0.1% tacrolimus ointment, resulted in a favorable clinical response. This report summarizes the clinical features of a pediatric case of granuloma faciale and Takayasu arteritis and reviews the etiology, diagnostic approach, and current treatment strategies for the disorders, aiming to enhance clinicians' understanding of these conditions.
Humans ; Male ; Child ; Takayasu Arteritis/diagnosis* ; Facial Dermatoses/diagnosis*

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: 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

Takayasu arteritis (TAK) is a chronic inflammatory disease characterized by granulomatous vasculitis of the aorta and its major branches. The rarity of the disease along with its heterogeneous clinical presentation typically lead to late diagnosis and delayed treatment. Furthermore, clinical and serological indices for monitoring disease activity are suboptimal, with no definitive evidence supporting therapeutic approaches in TAK. Nevertheless, there have been recent advances in disease assessment with new scoring systems (Indian Takayasu Arteritis Score), biomarkers including pentraxin 3 and soluble human leukocyte antigen-E, and imaging modalities such as 18F-fluorodeoxyglucose-positron emission tomography. Most of the new information for management of TAK has come from increasing experience with biological agents, such as tumor necrosis factor inhibitors and tocilizumab, used in the treatment of resistant TAK. A number of potential new therapeutic targets that may be useful for the treatment of TAK have been reported, and randomized controlled trials are needed to establish optimal therapeutic approaches
Aorta ; Biological Factors ; Biomarkers ; Delayed Diagnosis ; Diagnosis ; Humans ; Leukocytes ; Takayasu Arteritis* ; Tumor Necrosis Factor-alpha ; Vasculitis

PURPOSE: To report a rare case of traction retinal detachment and retinal ischemia in inactive Takayasu's arteritis at ophthalmologic clinic. CASE SUMMARY: A 23-year-old woman presented with a floater, photophobia, and visual loss in her right eye one week prior to visit. She had no other systemic disease, such as diabetes mellitus or hypertension, or previous ophthalmic abnormalities except for a tumor in the adrenal gland. We found bilateral retinal ischemia and traction retinal detachment in the right eye on fundus examination without iris neovascularization. Pars plana vitrectomy, traction removal, endolaser treatment, and intravitreal bevacizumab injection were performed. Steroid eye drops and steroid systemic administration relieved the inflammation. On carotid doppler sonography, we found severe stenosis and thickness of the inner layer in both carotid arteries. We diagnosed the patient with an inactive phase of Takayasu's arteritis, which was conclusively correlated with the clinical features. Vascular anastomosis surgery along with follow-up was proposed by both the cardiology and vascular surgery departments. CONCLUSIONS: When a young patient presents with traction retinal detachment and retinal ischemia, Takayasu's arteritis should be considered for differential diagnosis and a systemic work-up should be performed as soon as possible.
Adrenal Glands ; Bevacizumab ; Cardiology ; Carotid Arteries ; Constriction, Pathologic ; Diabetes Mellitus ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Hypertension ; Inflammation ; Iris ; Ischemia ; Ophthalmic Solutions ; Photophobia ; Retinal Detachment* ; Retinaldehyde* ; Takayasu Arteritis* ; Traction* ; Vitrectomy ; Young Adult

A 31-year-old woman with a 15-year history of Takayasu's arteritis (TA) and a 13-year history of Hashimoto's thyroiditis presented with hematochezia. She received a diagnosis of Sjögren's syndrome at 1 month before her visit to Kyungpook National University Medical Center. Her colonoscopic findings were compatible with a diagnosis of ulcerative colitis (UC). She was treated with oral mesalazine, and her hematochezia symptoms subsequently disappeared. The coexistence of UC and TA has been reported; however, reports on the coexistence of UC and Sjögren's syndrome, or of UC and Hashimoto's thyroiditis are rare. Although the precise etiologies of these diseases are unknown, their presence together suggests that they may have a common pathophysiologic background. Furthermore, in patients with autoimmune or vascular diseases, including TA, systemic manifestations should be assessed with consideration of inflammatory bowel diseases including UC in the presence of gastrointestinal symptoms such as diarrhea and hematochezia.
Academic Medical Centers ; Adult ; Colitis, Ulcerative* ; Diagnosis ; Diarrhea ; Female ; Gastrointestinal Hemorrhage ; Gyeongsangbuk-do ; Hashimoto Disease ; Humans ; Inflammatory Bowel Diseases ; Mesalamine ; Sjogren's Syndrome ; Takayasu Arteritis* ; Thyroid Gland* ; Thyroiditis* ; Ulcer* ; Vascular Diseases

Takayasu arteritis (TA) and ulcerative colitis (UC), both immune-mediated inflammatory diseases, rarely occur together. This report describes TA in a 29-year old female patient who was being treated for UC for three years. As she had left-side neck pain and headache, she was diagnosed with TA and her response to tumor necrosis factor (TNF) inhibitor was assessed by fluorine-18-fluorodeoxyglucose (¹⁸F-FDG) positron emission tomography (PET)/computed tomography (CT). Positive responses to the TNF inhibitor were seen by PET/CT for the TA and by endoscopy for the UC. We conclude that TNF inhibitors are effective treatments for both TA and UC. We found that PET/CT is a useful for diagnosing and assessing TA.
Colitis, Ulcerative* ; Diagnosis* ; Electrons* ; Endoscopy ; Female ; Fluorodeoxyglucose F18* ; Headache ; Humans ; Neck Pain ; Positron-Emission Tomography ; Positron-Emission Tomography and Computed Tomography ; Takayasu Arteritis* ; Tumor Necrosis Factor-alpha ; Ulcer*

Most common peripheral neuropathy around foot and ankle is diabetic neuropathy, but there are another cause of peripheral neuropathy, such as rheumatoid arthritis, metabolic disease, genetic disease, toxic material, and so on. The main symptom of peripheral neuropathy is pain. The disturbance of sensory and balancing, weakness of muscle, deformity of foot and neuropathic arthropathy are also the symptoms of the peripheral neuropathy. History taking is most important to identify the cause of peripheral neuropathy. Neurological exam have to include the pin prick test, vibration test, 10 g-monofilamant test and ankle reflex test. Simple radiography is essential to observe the deformities or neuropathic arthropathy at foot and ankle. The presence of peripheral neuropathy, involvement and severity can be identified from nerve conduction study. The study of occlusive arteritis is essential for diabetic neuropathy. The medical treatment of associated disease is important but the pain of peripheral neuropathy should be controlled simultaneously. Medicine include the antidepressants, anticonvulsants, opioids and topical agents. The surgical treatment of peripheral neuropathy include lengthening of Achilles tendon, correction of deformity, the total contact cast and arthrodesis. Surgical decompression of specific nerve might helpful in pain control of peripheral neuropathy.
Achilles Tendon ; Analgesics, Opioid ; Ankle* ; Anticonvulsants ; Antidepressive Agents ; Arteritis ; Arthritis, Rheumatoid ; Arthrodesis ; Congenital Abnormalities ; Decompression, Surgical ; Diabetic Neuropathies ; Diagnosis ; Foot* ; Metabolic Diseases ; Neural Conduction ; Peripheral Nervous System Diseases* ; Radiography ; Reflex ; Somatoform Disorders* ; Vibration