Disease activity, severity, and damage in the UK juvenile‐onset systemic lupus erythematosus cohort
Identifieur interne : 001A82 ( Istex/Curation ); précédent : 001A81; suivant : 001A83Disease activity, severity, and damage in the UK juvenile‐onset systemic lupus erythematosus cohort
Auteurs : Louise Watson [Royaume-Uni] ; Valentina Leone [Royaume-Uni] ; Clarissa Pilkington [Royaume-Uni] ; Kjell Tullus [Royaume-Uni] ; Satyapal Rangaraj [Royaume-Uni] ; Janet E. Mcdonagh [Royaume-Uni] ; Janet Gardner-Medwin [Royaume-Uni] ; Nick Wilkinson [Royaume-Uni] ; Phil Riley [Royaume-Uni] ; Jane Tizard [Royaume-Uni] ; Kate Armon [Royaume-Uni] ; Manish D. Sinha [Royaume-Uni] ; Yiannis Ioannou [Royaume-Uni] ; Neil Archer [Royaume-Uni] ; Kathryn Bailey [Royaume-Uni] ; Joyce Davidson [Royaume-Uni] ; Eileen M. Baildam [Royaume-Uni] ; Gavin Cleary [Royaume-Uni] ; Liza J. Mccann [Royaume-Uni] ; Michael W. Beresford [Royaume-Uni]Source :
- Arthritis & Rheumatism [ 0004-3591 ] ; 2012-07.
Abstract
Objective: The UK Juvenile‐Onset Systemic Lupus Erythematosus (JSLE) Cohort Study is a multicenter collaborative network established with the aim of improving the understanding of juvenile SLE. The present study was undertaken to describe the clinical manifestations and disease course in patients with juvenile SLE from this large, national inception cohort. Methods: Detailed data on clinical phenotype were collected at baseline and at regular clinic reviews and annual followup assessments in 232 patients from 14 centers across the UK over 4.5 years. Patients with SLE were identified according to the American College of Rheumatology (ACR) SLE classification criteria. The present cohort comprised children with juvenile SLE (n = 198) whose diagnosis fulfilled ≥4 of the ACR criteria for SLE. Results: Among patients with juvenile SLE, the female:male sex distribution was 5.6:1 and the median age at diagnosis was 12.6 years (interquartile range 10.4–14.5 years). Male patients were younger than female patients (P < 0.01). Standardized ethnicity data demonstrated a greater risk of juvenile SLE in non‐Caucasian UK patients (P < 0.05). Scores on the pediatric adaptation of the 2004 British Isles Lupus Assessment Group disease activity index demonstrated significantly increased frequencies of musculoskeletal (82%), renal (80%), hematologic (91%), immunologic (54%), and neurologic (26%) involvement among the patients over time. A large proportion of the patients (93%) were taking steroids and 24% of the patients required treatment with cyclophosphamide. Disease damage was common, with 28% of the patients having a Systemic Lupus International Collaborating Clinics/ACR damage score of ≥1. Conclusion: The data on these patients from the UK JSLE Cohort Study, comprising one of the largest national inception cohorts of patients with juvenile SLE to date, indicate that severe organ involvement and significant disease activity are primary characteristics in children with juvenile SLE. In addition, accumulation of disease‐associated damage could be seen.
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DOI: 10.1002/art.34410
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<record><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">Disease activity, severity, and damage in the UK juvenile‐onset systemic lupus erythematosus cohort</title><author><name sortKey="Watson, Louise" sort="Watson, Louise" uniqKey="Watson L" first="Louise" last="Watson">Louise Watson</name><affiliation wicri:level="1"><mods:affiliation>University of Liverpool and Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>University of Liverpool and Alder Hey Children's NHS Foundation Trust Hospital, Liverpool</wicri:regionArea></affiliation></author><author><name sortKey="Leone, Valentina" sort="Leone, Valentina" uniqKey="Leone V" first="Valentina" last="Leone">Valentina Leone</name><affiliation wicri:level="1"><mods:affiliation>Great North Children's Hospital Foundation Trust, Newcastle‐upon‐Tyne, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Great North Children's Hospital Foundation Trust, Newcastle‐upon‐Tyne</wicri:regionArea></affiliation></author><author><name sortKey="Pilkington, Clarissa" sort="Pilkington, Clarissa" uniqKey="Pilkington C" first="Clarissa" last="Pilkington">Clarissa Pilkington</name><affiliation wicri:level="1"><mods:affiliation>Great Ormond Street Hospital for Children NHS Trust, London, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Great Ormond Street Hospital for Children NHS Trust, London</wicri:regionArea></affiliation></author><author><name sortKey="Tullus, Kjell" sort="Tullus, Kjell" uniqKey="Tullus K" first="Kjell" last="Tullus">Kjell Tullus</name><affiliation wicri:level="1"><mods:affiliation>Great Ormond Street Hospital for Children NHS Trust, London, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Great Ormond Street Hospital for Children NHS Trust, London</wicri:regionArea></affiliation></author><author><name sortKey="Rangaraj, Satyapal" sort="Rangaraj, Satyapal" uniqKey="Rangaraj S" first="Satyapal" last="Rangaraj">Satyapal Rangaraj</name><affiliation wicri:level="1"><mods:affiliation>Nottingham Children's Hospital and Nottingham University Hospital NHS Trust, Nottingham, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Nottingham Children's Hospital and Nottingham University Hospital NHS Trust, Nottingham</wicri:regionArea></affiliation></author><author><name sortKey="Mcdonagh, Janet E" sort="Mcdonagh, Janet E" uniqKey="Mcdonagh J" first="Janet E." last="Mcdonagh">Janet E. Mcdonagh</name><affiliation wicri:level="1"><mods:affiliation>Birmingham Children's Hospital NHS Foundation Trust, Birmingham, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Birmingham Children's Hospital NHS Foundation Trust, Birmingham</wicri:regionArea></affiliation></author><author><name sortKey="Gardner Edwin, Janet" sort="Gardner Edwin, Janet" uniqKey="Gardner Edwin J" first="Janet" last="Gardner-Medwin">Janet Gardner-Medwin</name><affiliation wicri:level="1"><mods:affiliation>Royal Hospital for Sick Children NHS Lothian University Trust, Edinburgh, UK, and Royal Hospital for Sick Children NHS Greater Glasgow and Clyde, Glasgow, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Royal Hospital for Sick Children NHS Lothian University Trust, Edinburgh, UK, and Royal Hospital for Sick Children NHS Greater Glasgow and Clyde, Glasgow</wicri:regionArea></affiliation></author><author><name sortKey="Wilkinson, Nick" sort="Wilkinson, Nick" uniqKey="Wilkinson N" first="Nick" last="Wilkinson">Nick Wilkinson</name><affiliation wicri:level="1"><mods:affiliation>The Children's Hospital, Oxford Radcliffe Hospital NHS Trust, Oxford, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>The Children's Hospital, Oxford Radcliffe Hospital NHS Trust, Oxford</wicri:regionArea></affiliation></author><author><name sortKey="Riley, Phil" sort="Riley, Phil" uniqKey="Riley P" first="Phil" last="Riley">Phil Riley</name><affiliation wicri:level="1"><mods:affiliation>Royal Manchester Children's NHS Trust Hospital, Manchester, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Royal Manchester Children's NHS Trust Hospital, Manchester</wicri:regionArea></affiliation></author><author><name sortKey="Tizard, Jane" sort="Tizard, Jane" uniqKey="Tizard J" first="Jane" last="Tizard">Jane Tizard</name><affiliation wicri:level="1"><mods:affiliation>Bristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust, Bristol, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Bristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust, Bristol</wicri:regionArea></affiliation></author><author><name sortKey="Armon, Kate" sort="Armon, Kate" uniqKey="Armon K" first="Kate" last="Armon">Kate Armon</name><affiliation wicri:level="1"><mods:affiliation>Jenny Lind Children's Hospital, Norfolk and Norwich University Hospital NHS Foundation Trust, Norfolk, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Jenny Lind Children's Hospital, Norfolk and Norwich University Hospital NHS Foundation Trust, Norfolk</wicri:regionArea></affiliation></author><author><name sortKey="Sinha, Manish D" sort="Sinha, Manish D" uniqKey="Sinha M" first="Manish D." last="Sinha">Manish D. Sinha</name><affiliation wicri:level="1"><mods:affiliation>Evelina Children's Hospital, Guy's and St. Thomas' NHS Foundation Trust, London, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Evelina Children's Hospital, Guy's and St. Thomas' NHS Foundation Trust, London</wicri:regionArea></affiliation></author><author><name sortKey="Ioannou, Yiannis" sort="Ioannou, Yiannis" uniqKey="Ioannou Y" first="Yiannis" last="Ioannou">Yiannis Ioannou</name><affiliation wicri:level="1"><mods:affiliation>University College London Hospitals NHS Foundation Trust, London, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>University College London Hospitals NHS Foundation Trust, London</wicri:regionArea></affiliation></author><author><name sortKey="Archer, Neil" sort="Archer, Neil" uniqKey="Archer N" first="Neil" last="Archer">Neil Archer</name><affiliation wicri:level="1"><mods:affiliation>Musgrove Park Hospital, Taunton and Somerset NHS Foundation Trust, Taunton, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Musgrove Park Hospital, Taunton and Somerset NHS Foundation Trust, Taunton</wicri:regionArea></affiliation></author><author><name sortKey="Bailey, Kathryn" sort="Bailey, Kathryn" uniqKey="Bailey K" first="Kathryn" last="Bailey">Kathryn Bailey</name><affiliation wicri:level="1"><mods:affiliation>University Hospital Coventry and Warwickshire NHS Trust, Coventry, UK, and George Eliot Hospital NHS Trust, Nuneaton, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>University Hospital Coventry and Warwickshire NHS Trust, Coventry, UK, and George Eliot Hospital NHS Trust, Nuneaton</wicri:regionArea></affiliation></author><author><name sortKey="Davidson, Joyce" sort="Davidson, Joyce" uniqKey="Davidson J" first="Joyce" last="Davidson">Joyce Davidson</name><affiliation wicri:level="1"><mods:affiliation>Royal Hospital for Sick Children NHS Lothian University Trust, Edinburgh, UK, and Royal Hospital for Sick Children NHS Greater Glasgow and Clyde, Glasgow, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Royal Hospital for Sick Children NHS Lothian University Trust, Edinburgh, UK, and Royal Hospital for Sick Children NHS Greater Glasgow and Clyde, Glasgow</wicri:regionArea></affiliation></author><author><name sortKey="Baildam, Eileen M" sort="Baildam, Eileen M" uniqKey="Baildam E" first="Eileen M." last="Baildam">Eileen M. Baildam</name><affiliation wicri:level="1"><mods:affiliation>Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Alder Hey Children's NHS Foundation Trust Hospital, Liverpool</wicri:regionArea></affiliation></author><author><name sortKey="Cleary, Gavin" sort="Cleary, Gavin" uniqKey="Cleary G" first="Gavin" last="Cleary">Gavin Cleary</name><affiliation wicri:level="1"><mods:affiliation>Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Alder Hey Children's NHS Foundation Trust Hospital, Liverpool</wicri:regionArea></affiliation></author><author><name sortKey="Mccann, Liza J" sort="Mccann, Liza J" uniqKey="Mccann L" first="Liza J." last="Mccann">Liza J. Mccann</name><affiliation wicri:level="1"><mods:affiliation>Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Alder Hey Children's NHS Foundation Trust Hospital, Liverpool</wicri:regionArea></affiliation></author><author><name sortKey="Beresford, Michael W" sort="Beresford, Michael W" uniqKey="Beresford M" first="Michael W." last="Beresford">Michael W. Beresford</name><affiliation wicri:level="1"><mods:affiliation>University of Liverpool and Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>University of Liverpool and Alder Hey Children's NHS Foundation Trust Hospital, Liverpool</wicri:regionArea></affiliation><affiliation wicri:level="1"><mods:affiliation>E-mail: m.w.beresford@liverpool.ac.uk</mods:affiliation><country wicri:rule="url">Royaume-Uni</country></affiliation><affiliation wicri:level="1"><mods:affiliation>Correspondence address: Department of Women's and Children's Health, Institute of Translational Medicine, Alder Hey Children's NHS Foundation Trust Hospital, Eaton Road, Liverpool L12 2AP, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Correspondence address: Department of Women's and Children's Health, Institute of Translational Medicine, Alder Hey Children's NHS Foundation Trust Hospital, Eaton Road, Liverpool L12 2AP</wicri:regionArea></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:1B4F5A3B9D3D2B27CB230AF14296F24B56D29898</idno><date when="2012" year="2012">2012</date><idno type="doi">10.1002/art.34410</idno><idno type="url">https://api.istex.fr/ark:/67375/WNG-1N5K3F88-R/fulltext.pdf</idno><idno type="wicri:Area/Istex/Corpus">001A82</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Corpus" wicri:corpus="ISTEX">001A82</idno><idno type="wicri:Area/Istex/Curation">001A82</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main">Disease activity, severity, and damage in the UK juvenile‐onset systemic lupus erythematosus cohort</title><author><name sortKey="Watson, Louise" sort="Watson, Louise" uniqKey="Watson L" first="Louise" last="Watson">Louise Watson</name><affiliation wicri:level="1"><mods:affiliation>University of Liverpool and Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>University of Liverpool and Alder Hey Children's NHS Foundation Trust Hospital, Liverpool</wicri:regionArea></affiliation></author><author><name sortKey="Leone, Valentina" sort="Leone, Valentina" uniqKey="Leone V" first="Valentina" last="Leone">Valentina Leone</name><affiliation wicri:level="1"><mods:affiliation>Great North Children's Hospital Foundation Trust, Newcastle‐upon‐Tyne, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Great North Children's Hospital Foundation Trust, Newcastle‐upon‐Tyne</wicri:regionArea></affiliation></author><author><name sortKey="Pilkington, Clarissa" sort="Pilkington, Clarissa" uniqKey="Pilkington C" first="Clarissa" last="Pilkington">Clarissa Pilkington</name><affiliation wicri:level="1"><mods:affiliation>Great Ormond Street Hospital for Children NHS Trust, London, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Great Ormond Street Hospital for Children NHS Trust, London</wicri:regionArea></affiliation></author><author><name sortKey="Tullus, Kjell" sort="Tullus, Kjell" uniqKey="Tullus K" first="Kjell" last="Tullus">Kjell Tullus</name><affiliation wicri:level="1"><mods:affiliation>Great Ormond Street Hospital for Children NHS Trust, London, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Great Ormond Street Hospital for Children NHS Trust, London</wicri:regionArea></affiliation></author><author><name sortKey="Rangaraj, Satyapal" sort="Rangaraj, Satyapal" uniqKey="Rangaraj S" first="Satyapal" last="Rangaraj">Satyapal Rangaraj</name><affiliation wicri:level="1"><mods:affiliation>Nottingham Children's Hospital and Nottingham University Hospital NHS Trust, Nottingham, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Nottingham Children's Hospital and Nottingham University Hospital NHS Trust, Nottingham</wicri:regionArea></affiliation></author><author><name sortKey="Mcdonagh, Janet E" sort="Mcdonagh, Janet E" uniqKey="Mcdonagh J" first="Janet E." last="Mcdonagh">Janet E. Mcdonagh</name><affiliation wicri:level="1"><mods:affiliation>Birmingham Children's Hospital NHS Foundation Trust, Birmingham, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Birmingham Children's Hospital NHS Foundation Trust, Birmingham</wicri:regionArea></affiliation></author><author><name sortKey="Gardner Edwin, Janet" sort="Gardner Edwin, Janet" uniqKey="Gardner Edwin J" first="Janet" last="Gardner-Medwin">Janet Gardner-Medwin</name><affiliation wicri:level="1"><mods:affiliation>Royal Hospital for Sick Children NHS Lothian University Trust, Edinburgh, UK, and Royal Hospital for Sick Children NHS Greater Glasgow and Clyde, Glasgow, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Royal Hospital for Sick Children NHS Lothian University Trust, Edinburgh, UK, and Royal Hospital for Sick Children NHS Greater Glasgow and Clyde, Glasgow</wicri:regionArea></affiliation></author><author><name sortKey="Wilkinson, Nick" sort="Wilkinson, Nick" uniqKey="Wilkinson N" first="Nick" last="Wilkinson">Nick Wilkinson</name><affiliation wicri:level="1"><mods:affiliation>The Children's Hospital, Oxford Radcliffe Hospital NHS Trust, Oxford, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>The Children's Hospital, Oxford Radcliffe Hospital NHS Trust, Oxford</wicri:regionArea></affiliation></author><author><name sortKey="Riley, Phil" sort="Riley, Phil" uniqKey="Riley P" first="Phil" last="Riley">Phil Riley</name><affiliation wicri:level="1"><mods:affiliation>Royal Manchester Children's NHS Trust Hospital, Manchester, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Royal Manchester Children's NHS Trust Hospital, Manchester</wicri:regionArea></affiliation></author><author><name sortKey="Tizard, Jane" sort="Tizard, Jane" uniqKey="Tizard J" first="Jane" last="Tizard">Jane Tizard</name><affiliation wicri:level="1"><mods:affiliation>Bristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust, Bristol, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Bristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust, Bristol</wicri:regionArea></affiliation></author><author><name sortKey="Armon, Kate" sort="Armon, Kate" uniqKey="Armon K" first="Kate" last="Armon">Kate Armon</name><affiliation wicri:level="1"><mods:affiliation>Jenny Lind Children's Hospital, Norfolk and Norwich University Hospital NHS Foundation Trust, Norfolk, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Jenny Lind Children's Hospital, Norfolk and Norwich University Hospital NHS Foundation Trust, Norfolk</wicri:regionArea></affiliation></author><author><name sortKey="Sinha, Manish D" sort="Sinha, Manish D" uniqKey="Sinha M" first="Manish D." last="Sinha">Manish D. Sinha</name><affiliation wicri:level="1"><mods:affiliation>Evelina Children's Hospital, Guy's and St. Thomas' NHS Foundation Trust, London, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Evelina Children's Hospital, Guy's and St. Thomas' NHS Foundation Trust, London</wicri:regionArea></affiliation></author><author><name sortKey="Ioannou, Yiannis" sort="Ioannou, Yiannis" uniqKey="Ioannou Y" first="Yiannis" last="Ioannou">Yiannis Ioannou</name><affiliation wicri:level="1"><mods:affiliation>University College London Hospitals NHS Foundation Trust, London, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>University College London Hospitals NHS Foundation Trust, London</wicri:regionArea></affiliation></author><author><name sortKey="Archer, Neil" sort="Archer, Neil" uniqKey="Archer N" first="Neil" last="Archer">Neil Archer</name><affiliation wicri:level="1"><mods:affiliation>Musgrove Park Hospital, Taunton and Somerset NHS Foundation Trust, Taunton, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Musgrove Park Hospital, Taunton and Somerset NHS Foundation Trust, Taunton</wicri:regionArea></affiliation></author><author><name sortKey="Bailey, Kathryn" sort="Bailey, Kathryn" uniqKey="Bailey K" first="Kathryn" last="Bailey">Kathryn Bailey</name><affiliation wicri:level="1"><mods:affiliation>University Hospital Coventry and Warwickshire NHS Trust, Coventry, UK, and George Eliot Hospital NHS Trust, Nuneaton, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>University Hospital Coventry and Warwickshire NHS Trust, Coventry, UK, and George Eliot Hospital NHS Trust, Nuneaton</wicri:regionArea></affiliation></author><author><name sortKey="Davidson, Joyce" sort="Davidson, Joyce" uniqKey="Davidson J" first="Joyce" last="Davidson">Joyce Davidson</name><affiliation wicri:level="1"><mods:affiliation>Royal Hospital for Sick Children NHS Lothian University Trust, Edinburgh, UK, and Royal Hospital for Sick Children NHS Greater Glasgow and Clyde, Glasgow, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Royal Hospital for Sick Children NHS Lothian University Trust, Edinburgh, UK, and Royal Hospital for Sick Children NHS Greater Glasgow and Clyde, Glasgow</wicri:regionArea></affiliation></author><author><name sortKey="Baildam, Eileen M" sort="Baildam, Eileen M" uniqKey="Baildam E" first="Eileen M." last="Baildam">Eileen M. Baildam</name><affiliation wicri:level="1"><mods:affiliation>Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Alder Hey Children's NHS Foundation Trust Hospital, Liverpool</wicri:regionArea></affiliation></author><author><name sortKey="Cleary, Gavin" sort="Cleary, Gavin" uniqKey="Cleary G" first="Gavin" last="Cleary">Gavin Cleary</name><affiliation wicri:level="1"><mods:affiliation>Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Alder Hey Children's NHS Foundation Trust Hospital, Liverpool</wicri:regionArea></affiliation></author><author><name sortKey="Mccann, Liza J" sort="Mccann, Liza J" uniqKey="Mccann L" first="Liza J." last="Mccann">Liza J. Mccann</name><affiliation wicri:level="1"><mods:affiliation>Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Alder Hey Children's NHS Foundation Trust Hospital, Liverpool</wicri:regionArea></affiliation></author><author><name sortKey="Beresford, Michael W" sort="Beresford, Michael W" uniqKey="Beresford M" first="Michael W." last="Beresford">Michael W. Beresford</name><affiliation wicri:level="1"><mods:affiliation>University of Liverpool and Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>University of Liverpool and Alder Hey Children's NHS Foundation Trust Hospital, Liverpool</wicri:regionArea></affiliation><affiliation wicri:level="1"><mods:affiliation>E-mail: m.w.beresford@liverpool.ac.uk</mods:affiliation><country wicri:rule="url">Royaume-Uni</country></affiliation><affiliation wicri:level="1"><mods:affiliation>Correspondence address: Department of Women's and Children's Health, Institute of Translational Medicine, Alder Hey Children's NHS Foundation Trust Hospital, Eaton Road, Liverpool L12 2AP, UK</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Correspondence address: Department of Women's and Children's Health, Institute of Translational Medicine, Alder Hey Children's NHS Foundation Trust Hospital, Eaton Road, Liverpool L12 2AP</wicri:regionArea></affiliation></author></analytic><monogr></monogr><series><title level="j" type="main">Arthritis & Rheumatism</title><title level="j" type="alt">ARTHRITIS AND RHEUMATISM</title><idno type="ISSN">0004-3591</idno><idno type="eISSN">1529-0131</idno><imprint><biblScope unit="vol">64</biblScope><biblScope unit="issue">7</biblScope><biblScope unit="page" from="2356">2356</biblScope><biblScope unit="page" to="2365">2365</biblScope><biblScope unit="page-count">10</biblScope><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="2012-07">2012-07</date></imprint><idno type="ISSN">0004-3591</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0004-3591</idno></seriesStmt></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Objective: The UK Juvenile‐Onset Systemic Lupus Erythematosus (JSLE) Cohort Study is a multicenter collaborative network established with the aim of improving the understanding of juvenile SLE. The present study was undertaken to describe the clinical manifestations and disease course in patients with juvenile SLE from this large, national inception cohort. Methods: Detailed data on clinical phenotype were collected at baseline and at regular clinic reviews and annual followup assessments in 232 patients from 14 centers across the UK over 4.5 years. Patients with SLE were identified according to the American College of Rheumatology (ACR) SLE classification criteria. The present cohort comprised children with juvenile SLE (n = 198) whose diagnosis fulfilled ≥4 of the ACR criteria for SLE. Results: Among patients with juvenile SLE, the female:male sex distribution was 5.6:1 and the median age at diagnosis was 12.6 years (interquartile range 10.4–14.5 years). Male patients were younger than female patients (P < 0.01). Standardized ethnicity data demonstrated a greater risk of juvenile SLE in non‐Caucasian UK patients (P < 0.05). Scores on the pediatric adaptation of the 2004 British Isles Lupus Assessment Group disease activity index demonstrated significantly increased frequencies of musculoskeletal (82%), renal (80%), hematologic (91%), immunologic (54%), and neurologic (26%) involvement among the patients over time. A large proportion of the patients (93%) were taking steroids and 24% of the patients required treatment with cyclophosphamide. Disease damage was common, with 28% of the patients having a Systemic Lupus International Collaborating Clinics/ACR damage score of ≥1. Conclusion: The data on these patients from the UK JSLE Cohort Study, comprising one of the largest national inception cohorts of patients with juvenile SLE to date, indicate that severe organ involvement and significant disease activity are primary characteristics in children with juvenile SLE. In addition, accumulation of disease‐associated damage could be seen.</div></front></TEI></record>Pour manipuler ce document sous Unix (Dilib)
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|étape= Curation
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|clé= ISTEX:1B4F5A3B9D3D2B27CB230AF14296F24B56D29898
|texte= Disease activity, severity, and damage in the UK juvenile‐onset systemic lupus erythematosus cohort
}}
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