Related Experiment Video
Updated: Aug 18, 2025
A Metadata Extraction Approach for Clinical Case Reports to Enable Advanced Understanding of Biomedical Concepts
Published on: September 20, 2018
Infantile Takayasu: clinical features and long-term outcome
Adi Miller-Barmak1,2, Flavio Sztajnbok3, Zeynep Balik4
1Department of Pediatrics B, Ruth Rappaport Children's Hospital, Rambam Health Care Campus, Haifa, Israel.
Insights
Infantile Takayasu arteritis (TAK) is severe, often requiring biologic agents and invasive procedures. This largest case series highlights unique infant characteristics and complications compared to older children with this rare large-vessel vasculitis.
Area of Science:
- Pediatric Rheumatology
- Vasculitis Research
- Immunology
Background:
- Takayasu arteritis (TAK) is a rare large-vessel vasculitis predominantly affecting children and young adults.
- Infantile cases of TAK are exceptionally rare, with limited data on their specific clinical and demographic profiles.
- Existing literature on pediatric TAK often excludes or underrepresents infants, necessitating focused research on this age group.
Purpose of the Study:
- To present the largest case series of infantile Takayasu arteritis (TAK).
- To identify the demographic and clinical characteristics of infants diagnosed with TAK.
- To compare the presentation and management of infantile TAK with that of older children.
Main Methods:
- An international multicenter retrospective cohort study was conducted.
- Data were collected from patient charts of children diagnosed with TAK before the age of 5 years.
- Inclusion required meeting both EULAR/PReS 2008 and 1990 ACR/EULAR criteria for Takayasu arteritis.
Main Results:
- Twelve infants (50% female) with a median onset age of 11 months were included, with a 4-month diagnostic delay.
- Hypertension, blood pressure differences, and fever were the most common presenting symptoms.
- The abdominal aorta and renal arteries were most frequently involved; 50% received biologics, with infliximab showing a 40% remission rate.
Conclusions:
- This study represents the largest cohort of infantile Takayasu arteritis.
- Infants with TAK exhibit more severe disease compared to older children.
- Infantile TAK is associated with a higher likelihood of biologic agent use, complications, and need for invasive interventions.
Objectives:
Takayasu arteritis (TAK) is a large-vessel vasculitis rarely reported in children and infants. Most articles on paediatric TAK have not focused on infants. We present the largest case series of infantile TAK, aiming to identify its demographic and clinical characteristics and compare them with existing data on older children.
Methods:
We conducted an international multicentre retrospective cohort study. Epidemiological and clinical data were collected from patients' charts from six rheumatology centres. All patients met both the EULAR/PReS 2008 criteria and the 1990 ACR/EULAR criteria and were diagnosed with TAK at age <5 years.
Results:
Twelve patients were included (50% female). Median age of symptom onset was 11 months, with a diagnostic delay of 4 months. The most common symptoms at presentation were hypertension, blood pressure differences between limbs, and fever. The most commonly involved arteries were the abdominal aorta and renal artery. Medications included steroids, conventional and biologic DMARDs, and other immunosuppressive therapies. Half of the patients received biologic agents, of which infliximab had the highest complete remission rate (40%). Other medications resulting in complete remission were CYC (40%) and MTX (38%). Invasive procedures were required for 58% of patients. The most common complications were cardiac (50%), stroke (42%), and serious infections (33%). No patients died.
Conclusion:
This study presents the largest series of infantile TAK. Compared with other reported series on older children, infants with TAK have more severe disease and were more likely to receive biologic agents, develop complications, and require invasive interventions.
More Related Videos
07:36Modeling Encephalopathy of Prematurity Using Prenatal Hypoxia-ischemia with Intra-amniotic Lipopolysaccharide in Rats
Published on: November 20, 2015
06:35An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
Published on: February 8, 2019
Related Concept Videos
01:22Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies
01:29Acute Kidney Injury III: Clinical Manifestations
01:27Myocarditis II: Clinical Features and Diagnostic Tests
01:24Chronic Kidney Disease II: Clinical Manifestations
01:23Rheumatic Heart Disease I: Introduction
01:27Atherosclerosis II: Clinical Manifestations and Diagnostic Tests