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J Rheumatol. 2014 Jun;41(6):1183-9. doi: 10.3899/jrheum.131117. Epub 2014 May 1.

Childhood-onset Takayasu arteritis -- experience from a tertiary care center in South India.

Author information

1
From 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.
2
From 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. debashisdandacmc@hotmail.com.

Abstract

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.

KEYWORDS:

INDIA; ITAS 2010; PEDIATRIC; TAKAYASU ARTERITIS

PMID:
24786922
DOI:
10.3899/jrheum.131117
[Indexed for MEDLINE]

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