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

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          Abstract

          Takayasu 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.

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          EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria.

          S. Ozen, A. Pistorio, S. M. Iusan … (2010)
          To validate the previously proposed classification criteria for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA). Step 1: retrospective/prospective web-data collection for children with HSP, c-PAN, c-WG and c-TA with age at diagnosis
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            The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis.

            W P Arend, B Michel, D Bloch … (1990)
            Criteria for the classification of Takayasu arteritis were developed by comparing 63 patients who had this disease with 744 control patients with other forms of vasculitis. Six criteria were selected for the traditional format classification: onset at age less than or equal to 40 years, claudication of an extremity, decreased brachial artery pulse, greater than 10 mm Hg difference in systolic blood pressure between arms, a bruit over the subclavian arteries or the aorta, and arteriographic evidence of narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities. The presence of 3 or more of these 6 criteria demonstrated a sensitivity of 90.5% and a specificity of 97.8%. A classification tree also was constructed with 5 of these 6 criteria, omitting claudication of an extremity. The classification tree demonstrated a sensitivity of 92.1% and a specificity of 97.0%.
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              Development and initial validation of the Indian Takayasu Clinical Activity Score (ITAS2010).

              Rajiva Gupta, Ramnath Misra, Lakshmanan Jeyaseelan … (2013)
              There are no valid instruments to measure disease activity in Takayasu arteritis (TA). We aim to provide a valid measure to assess clinical disease activity with or without incorporating acute phase reactants. The Indian Takayasu Clinical Activity Score (ITAS) was initially derived from disease manifestations scored in the Disease Extent Index (DEI.Tak). The ITAS was validated by a group of physicians scoring both live and paper cases for inter-rater reliability (IRR), convergence with BVAS, correlation with the Physician's Global Assessment (PGA) and ESR/CRP. It was further validated at a single centre in 177 patients for its ability to discriminate between active and inactive disease state at first visit and sensitivity to change in 132 active patients measured serially at two follow-up visits. ITAS-A also included graded scores for ESR/CRP. The final ITAS2010 contains 44 items with 33 features arising from the cardiovascular system. Seven key items are weighted to score 2 and all others score 1 only. Inter-observer variability was highly satisfactory (IRR 0.97). The ITAS showed superior inter-rater agreement compared with the BVAS (IRR 0.9) and PGA (IRR 0.82). In the single-centre study, median ITAS scores at first visit were significantly higher in active disease (5.62 ± 3.14) compared with grumbling (3.36 ± 1.96) and inactive disease (1.27 ± 1.26, P < 0.0001). The therapy induced a significant decrease in the ITAS2010 but the higher ITAS-A scores remained elevated. The ITAS2010, validated in over 300 TA patients and sensitive to change, is a useful measure of clinical disease activity for patient monitoring. Higher ITAS-A scores suggest poor control of active disease by current therapy.
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                Author and article information

                Journal
                Journal ID (iso-abbrev): Arthritis Res. Ther.
                Title: Arthritis research & therapy
                Publisher: Springer Science and Business Media LLC
                ISSN (Electronic): 1478-6362
                ISSN (Print): 1478-6354
                Publication date (Electronic): Feb 25 2015
                Volume: 17
                Affiliations
                [1 ] UCL Institute of Child Health, 30 Guilford Street and Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N1EH, UK. d.eleftheriou@ucl.ac.uk.
                [2 ] UCL Institute of Child Health, 30 Guilford Street and Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N1EH, UK. giulia.varnier@gosh.nhs.uk.
                [3 ] Department of Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK. giulia.varnier@gosh.nhs.uk.
                [4 ] Department of Paediatrics and Adolescent Medicine, Paediatric Rheumatology Unit, Charles University in Prague and General University Hospital in Prague, Prague, Czech Republic. dolezalova.pavla@vfn.cz.
                [5 ] Department of Paediatric Rheumatology, Sheffield Children's Hospital, Sheffield, South Yorkshire S10 2TH, UK. ammcmahon72@googlemail.com.
                [6 ] Department of Paediatric Rheumatology, Sheffield Children's Hospital, Sheffield, South Yorkshire S10 2TH, UK. Muthana.Al-Obaidi@sch.nhs.uk.
                [7 ] UCL Institute of Child Health, 30 Guilford Street and Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N1EH, UK. p.brogan@ucl.ac.uk.
                Article
                Publisher Item ID: 10.1186/s13075-015-0545-1
                DOI: 10.1186/s13075-015-0545-1
                PMC ID: 4392477
                PubMed ID: 25879697
                SO-VID: 4e5dea40-c9cf-425a-88e0-4c43d164945c
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