Childhood-onset Takayasu arteritis -- experience from a tertiary care center in South India
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Published on: September 20, 2018
Ruchika Goel1, T Sathish Kumar1, Debashish Danda2
1From the Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India.R. Goel, MD, Postdoctoral Fellow, Clinical Immunology and Rheumatology; T.S. Kumar, MD, Pediatrics, Professor, Department of Child Health; D. Danda, MD, DM, FRCP, Clinical Immunology Professor, and Head, Department of Clinical Immunology and Rheumatology; G. Joseph, MD, DM, Cardiology, Professor, Department of Cardiology; V. Jeyaseelan, PhD, Lecturer, Department of Biostatistics; A.K. Surin, MD, Postdoctoral Fellow, and Assistant Professor, Clinical Immunology and Rheumatology, Christian Medical College; P. Bacon, MB, BChir, MRCP, FRCP, Emeritus Professor, Department of Rheumatology, University of Birmingham, Birmingham, UK.
Insights
Childhood-onset Takayasu arteritis (c-TA) in Indian children often presents with hypertension and fever. Despite aggressive treatment, one-third of patients experience disease progression, highlighting the need for ongoing monitoring.
Area of Science:
- Pediatric Rheumatology
- Vascular Inflammation
- Autoimmune Diseases
Background:
- Childhood-onset Takayasu arteritis (c-TA) is a rare, chronic inflammatory condition affecting large arteries.
- Understanding the clinical characteristics and outcomes in Asian Indian children is crucial for effective management.
Purpose of the Study:
- To investigate the clinical profile and treatment outcomes of Asian Indian children diagnosed with c-TA.
- To identify factors associated with disease progression and remission.
Main Methods:
- Retrospective analysis of 40 children with c-TA diagnosed before age 16.
- Assessment of disease activity and damage using DEI.TAK, ITAS 2010, and TADS.
- Kaplan-Meier analysis for cumulative incidence of sustained remission.
Main Results:
- Common symptoms included hypertension, headache, malaise, and fever; 62.5% had pulseless disease.
- High baseline DEI.TAK and TADS scores were observed, with active inflammation in most.
- While 30/34 patients achieved remission, only 29% had sustained remission at 5 years; 38% developed new vessel involvement.
Conclusions:
- One-third of c-TA patients experienced disease progression despite aggressive immunosuppression and interventions.
- Persistent inflammation correlated with increased Takayasu Arteritis Damage Score (TADS).
- Continuous surveillance using clinical assessments and imaging is essential for managing c-TA.
Objective:
To study the clinical profile and outcome of Asian Indian children with childhood-onset Takayasu arteritis (c-TA).
Methods:
Records were studied of patients with c-TA onset prior to age 16. Disease Extent Index-Takayasu (DEI.TAK), Indian Takayasu Arteritis Score 2010, and Takayasu Arteritis Damage Score (TADS) were calculated retrospectively from electronic records. Cumulative incidence of sustained remission was estimated using the Kaplan-Meier curve.
Results:
There were 40 patients with c-TA, with median age of onset of 12.5 years (range 1-16) and median diagnostic delay of 11.3 months (range 1-60). The most common presenting features were hypertension, headache, malaise, and fever. Pulseless disease was observed in 25 cases (62.5%). The majority (n = 28) had active disease with raised inflammatory markers, high baseline median DEI.TAK score of 10 (range 3-24), and high median TADS of 7 (range 1-14). Of the 34 patients followed for 21.5 months (range 3-192), remission was attained in 30. However, cumulative sustained remission was achieved in only 29% of them at 5 years. Median period of sustained remission was 22.5 months (95% CI 17.1-26.8). New areas of vessel involvement were observed in 13 patients (38%). Disease progression was arrested in the majority (n = 22, 66%) through aggressive medical management and endovascular intervention. All 11 patients with an increment in TADS of ≥ 4 during followup had persistently active or relapsing disease. There was a single fatality.
Conclusion:
Despite aggressive immunosuppression, damage progressed in one-third of patients with c-TA in association with persistent inflammation, warranting surveillance with clinical instruments and followup imaging.
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