Childhood Takayasu arteritis: disease course and response to therapy

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dc.contributor.authorAeschlimann, Florence A
dc.contributor.authorEng, Simon W M
dc.contributor.authorSheikh, Shehla
dc.contributor.authorLaxer, Ronald M
dc.contributor.authorHebert, Diane
dc.contributor.authorNoone, Damien
dc.contributor.authorTwilt, Marinka
dc.contributor.authorPagnoux, Christian
dc.contributor.authorBenseler, Susanne M
dc.contributor.authorYeung, Rae S M
dc.date.accessioned2018-11-07T17:04:38Z
dc.date.available2018-11-07T17:04:38Z
dc.date.issued2017-11-22
dc.date.updated2018-11-07T17:04:38Z
dc.description.abstractAbstract Background Takayasu arteritis (TAK) is a large vessel vasculitis that rarely affects children. Data on childhood TAK are scarce. The aim of this study was to analyze the presenting features, course and outcome of children with TAK, compare efficacy of treatment regimens and identify high-risk factors for adverse outcome. Methods A single-center cohort study of consecutive children fulfilling the EULAR/PRINTO/PReS criteria for childhood TAK between 1986 and 2015 was performed. Clinical phenotypes, laboratory markers, imaging features, disease course and treatment were documented. Disease activity was assessed using the Pediatric Vasculitis Disease Activity Score at each visit. Outcome: disease flare defined as new symptoms and/or increased inflammatory markers necessitating therapy escalation and/or new angiographic lesions, or death. Analysis: logistic regression tested relevant variables for flare. Kaplan-Meier analyses compared treatment regimens. Results Twenty-seven children were included; 74% were female, median age at diagnosis was 12.4 years. Twenty-two (81%) children presented with active disease at diagnosis. Treatment regimens included corticosteroids alone (15%), corticosteroids plus methotrexate (37%), cyclophosphamide (19%), or a biologic agent (11%). Adverse outcomes were documented in 14/27 (52%) children: two (7%) died within 6 months of diagnosis, and 13 (48%) experienced disease flares. The 2-year flare-free survival was 80% with biologic treatments compared to 43% in non-biologic therapies (pā€‰=ā€‰0.03); at last follow-up, biologic therapies resulted in significantly higher rates of inactive disease (pā€‰=ā€‰0.02). No additional outcome predictor was identified. Conclusions Childhood TAK carries a high disease burden; half of the children experienced flares and 7% died. Biologic therapies were associated with better control of disease activity.
dc.identifier.citationArthritis Research & Therapy. 2017 Nov 22;19(1):255
dc.identifier.doihttps://doi.org/10.1186/s13075-017-1452-4
dc.identifier.urihttp://hdl.handle.net/1880/109032
dc.identifier.urihttps://doi.org/10.11575/PRISM/45475
dc.language.rfc3066en
dc.rights.holderThe Author(s).
dc.titleChildhood Takayasu arteritis: disease course and response to therapy
dc.typeJournal Article
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