Takayasu arteritis in childhood: retrospective experience from a tertiary referral centre in the United Kingdom

Abstract

IntroductionTakayasu arteritis (TA) is an idiopathic large-vessel vasculitis affecting the aorta and its major branches. Although the disease rarely affects children, it does occur, even in infants. The objective of this study was to evaluate the clinical features, disease activity, treatment and outcome of childhood TA in a tertiary UK centre.MethodsWe analysed a retrospective case series of children fulfilling the TA classification criteria of the European League against Rheumatism, the Paediatric Rheumatology European Society and the Paediatric Rheumatology International Trials Organisation. Data regarding demographics, clinical features, treatments and outcomes were recorded. Descriptive statistics are expressed as median and range. Fisher’s exact test was used for group comparisons. The Paediatric Vasculitis Activity Score (PVAS), Paediatric Vasculitis Damage Index (PVDI), Disease Extent Index-Takayasu (DEI.Tak) and Indian Takayasu Arteritis Activity Score (ITAS2010) were calculated retrospectively.ResultsA total of 11 children (64% female) with age at diagnosis of 11.8 (1.3 to 17) years were identified over a 23-year period. The median time to diagnosis was 17 (0 to 132) months. The most common clinical features at presentation were arterial hypertension (72.7%), systemic features (36%) and cardiovascular (45%), neurological (36%), pulmonary (27%), skin (9%), renal (9%) and gastrointestinal (9%) involvement. At presentation, PVAS was 5/63 (1 to 13); DEI.Tak was 7/81 (2 to 12) and ITAS2010 was 9/57 (6 to 20). Treatment included corticosteroids (81.8%), combined with methotrexate in most cases (72.7%). Cyclophosphamide (36.4%) and biologic agents (45.5%) were reserved for severe and/or refractory cases. PVDI at latest follow-up was 5.5/72 (3 to 15). Mortality was 27%. Young age at disease onset (<5 years old) and permanent PVDI scores ≥3 were significantly associated with mortality risk (P = 0.024).ConclusionTA is a rare and potentially life-threatening large-vessel vasculitis. Improved awareness of TA is essential to secure a timely diagnosis. Although the evidence base for the treatment of TA in children is weak, we found that it is essential to treat it aggressively because our data emphasise that the mortality and morbidity in the paediatric population remains high.Electronic supplementary materialThe online version of this article (doi:10.1186/s13075-015-0545-1) contains supplementary material, which is available to authorized users.

Clinical study of children with Takayasu arteritis: a retrospective study from a single center in China

“…Gender differences may be related to the role of estrogen or genetics [12]. The average time to diagnosis in our cohort was shorter than that reported previously [3, 9, 10, 13, 14], which may be due to pediatricians’ increased awareness of childhood TA in recent years, as well as regional differences. Tuberculosis infection is thought to be one factor underlying TA [15] and many researchers have reported the co-existence tuberculosis infection in those with TA [3, 14, 15].…”

Portal hypertension: an uncommon clinical manifestation of Takayasu arteritis in a 9-year-old child

“…Eleftheriou et al2 found permanent damage items on PVDI scoring in the follow-up (score ≥3) were associated with mortality ( P =0.024). Other factors such as the time to diagnosis >12 months, cardiac involvement, or need for biological therapy were not significantly associated 2…”

“…. Treatment was based on high-dose corticosteroids and immunosuppressive drugs (eg, methotrexate, azathioprine, and cyclophosphamide) 2,5,17…”

“…A large series of cases with a long-term follow-up from the Mayo Clinic in the US reported better survival rates than the past (97% at 10 years and 86% at 15 years), and Serra et al5 showed a decrease in the mortality rate by 2.8% during the 2000–2010 follow-up period. The use of disease-modifying antirheumatic drugs and biological agents seems to be associated with a decreased rate of mortality 2. However, due to the absence of randomized trials comparing different immunosuppressive agents, no immunosuppressive agent can be considered more effective than others 5.…”

Diagnostic approach and current treatment options in childhood vasculitis

“…Stenosis is irreversible and may result in severe complications including renovascular hypertension, severe claudication (limping), myocardial infarction and stroke. Early angioplasty is needed in patients with stenosis in renal arteries, aortic valve failure and stenosis in more than three sites (6)(7)(8)(9). Tosilizumab which was shown to be efficient in adult studies has been reported to be also efficient in pediatric TA cases in case reports (15,16).…”