Direkrit Chiewchengchol1, Ruth Murphy1, Thomas Morgan1, Steven W Edwards1, Valentina Leone1, Mark Friswell1, Clarissa Pilkington1, Kjell Tullus1, Satyapal Rangaraj1, Janet E McDonagh1, Janet Gardner-Medwin1, Nick Wilkinson1, Phil Riley1, Jane Tizard1, Kate Armon1, Manish D Sinha1, Yiannis Ioannou1, Rebecca Mann1, Kathryn Bailey2, Joyce Davidson1, Eileen M Baildam1, Clare E Pain1, Gavin Cleary1, Liza J McCann1, Michael W Beresford3. 1. Institute of Translational Medicine, Alder Hey Children's NHS Foundation Trust Hospital and Institute of Integrative Biology, University of Liverpool, Liverpool, Department of Dermatology, Queens Medical Centre, Nottingham University Teaching Hospitals, Nottingham, Institute of Integrative Biology, University of Liverpool, Liverpool, Leeds General Infirmary, Leeds Teaching Hospital Trust, Leeds, Great North Children's Hospital Foundation Trust, Newcastle-upon-Tyne, Great Ormond Street Hospital for Children NHS Trust, London, Nottingham Children's Hospital and Nottingham University Hospital NHS Trust, Nottingham, Birmingham Children's Hospital NHS Foundation Trust, Birmingham, School of Medicine, Glasgow University, Glasgow, Children's Hospital, Oxford Radcliffe Hospital NHS Trust, Oxford, Royal Manchester Children's NHS Trust Hospital, Manchester, Bristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust, Bristol, Jenny Lind Children's Hospital, Norfolk and Norwich University Hospital NHS Foundation Trust, Norfolk, Evelina Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, University College London Hospitals NHS Foundation Trust and Arthritis Research UK Centre for Adolescent Rheumatology, University College London, London, Musgrove Park Hospital, Taunton and Somerset NHS Foundation Trust, Taunton, University Hospital Coventry and Warwickshire NHS Trust, Coventry, George Eliot Hospital NHS Trust, Nuneaton, Royal Hospital for Sick Children NHS Lothian University Trust, Edinburgh and Royal Hospital for Sick Children NHS Greater Glasgow and Clyde, Glasgow and Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK. 2. Institute of Translational Medicine, Alder Hey Children's NHS Foundation Trust Hospital and Institute of Integrative Biology, University of Liverpool, Liverpool, Department of Dermatology, Queens Medical Centre, Nottingham University Teaching Hospitals, Nottingham, Institute of Integrative Biology, University of Liverpool, Liverpool, Leeds General Infirmary, Leeds Teaching Hospital Trust, Leeds, Great North Children's Hospital Foundation Trust, Newcastle-upon-Tyne, Great Ormond Street Hospital for Children NHS Trust, London, Nottingham Children's Hospital and Nottingham University Hospital NHS Trust, Nottingham, Birmingham Children's Hospital NHS Foundation Trust, Birmingham, School of Medicine, Glasgow University, Glasgow, Children's Hospital, Oxford Radcliffe Hospital NHS Trust, Oxford, Royal Manchester Children's NHS Trust Hospital, Manchester, Bristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust, Bristol, Jenny Lind Children's Hospital, Norfolk and Norwich University Hospital NHS Foundation Trust, Norfolk, Evelina Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, University College London Hospitals NHS Foundation Trust and Arthritis Research UK Centre for Adolescent Rheumatology, University College London, London, Musgrove Park Hospital, Taunton and Somerset NHS Foundation Trust, Taunton, University Hospital Coventry and Warwickshire NHS Trust, Coventry, George Eliot Hospital NHS Trust, Nuneaton, Royal Hospital for Sick Children NHS Lothian University Trust, Edinburgh and Royal Hospital for Sick Children NHS Greater Glasgow and Clyde, Glasgow and Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK. Institute of Translational Medicine, Alder Hey Children's NHS Foundation Trust Hospital and Institute of Integrative Biology, University of Liverpool, Liverpool, Department of Dermatology, Queens Medical Centre, Nottingham University Teaching Hospitals, Nottingham, Institute of Integrative Biology, Universi 3. Institute of Translational Medicine, Alder Hey Children's NHS Foundation Trust Hospital and Institute of Integrative Biology, University of Liverpool, Liverpool, Department of Dermatology, Queens Medical Centre, Nottingham University Teaching Hospitals, Nottingham, Institute of Integrative Biology, University of Liverpool, Liverpool, Leeds General Infirmary, Leeds Teaching Hospital Trust, Leeds, Great North Children's Hospital Foundation Trust, Newcastle-upon-Tyne, Great Ormond Street Hospital for Children NHS Trust, London, Nottingham Children's Hospital and Nottingham University Hospital NHS Trust, Nottingham, Birmingham Children's Hospital NHS Foundation Trust, Birmingham, School of Medicine, Glasgow University, Glasgow, Children's Hospital, Oxford Radcliffe Hospital NHS Trust, Oxford, Royal Manchester Children's NHS Trust Hospital, Manchester, Bristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust, Bristol, Jenny Lind Children's Hospital, Norfolk and Norwich University Hospital NHS Foundation Trust, Norfolk, Evelina Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, University College London Hospitals NHS Foundation Trust and Arthritis Research UK Centre for Adolescent Rheumatology, University College London, London, Musgrove Park Hospital, Taunton and Somerset NHS Foundation Trust, Taunton, University Hospital Coventry and Warwickshire NHS Trust, Coventry, George Eliot Hospital NHS Trust, Nuneaton, Royal Hospital for Sick Children NHS Lothian University Trust, Edinburgh and Royal Hospital for Sick Children NHS Greater Glasgow and Clyde, Glasgow and Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK. m.w.beresford@liverpool.ac.uk.
Abstract
OBJECTIVE: To determine whether mucocutaneous manifestations are associated with major organ involvement in a UK national cohort of juvenile-onset SLE (JSLE) patients. METHODS: JSLE patients (n = 241) from 15 different centres whose diagnosis fulfilled four or more of the ACR criteria were divided into two groups: those with at least one ACR mucocutaneous criterion (ACR skin feature positive) and those without (ACR skin feature negative) at diagnosis. The relative frequency of skin involvement was described by the paediatric adaptation of the 2004 British Isles Lupus Assessment Group (pBILAG-2004) index. RESULTS: One hundred and seventy-nine patients (74%) had ACR-defined skin involvement with no significant demographic differences compared with those without. ACR skin feature negative patients showed greater haematological (84% vs 67%), renal (43% vs 26%) (P < 0.05) and neurological (16% vs 4%) involvement (P = 0.001). Forty-two per cent of ACR skin feature negative patients had skin involvement using pBILAG-2004, which included maculopapular rash (17%), non-scaring alopecia (15%), cutaneous vasculitis (12%) and RP (12%). ACR skin feature negative patients with moderate to severe skin involvement by pBILAG-2004 showed greater renal and haematological involvement at diagnosis and over the follow-up period (P < 0.05). Higher immunosuppressive drug use in the skin feature negative group was demonstrated. CONCLUSION: Patients who fulfil the ACR criteria but without any of the mucocutaneous criteria at diagnosis have an increased risk of major organ involvement. The pBILAG-2004 index has shown that other skin lesions may go undetected using the ACR criteria alone, and these lesions show a strong correlation with disease severity and major organ involvement.
OBJECTIVE: To determine whether mucocutaneous manifestations are associated with major organ involvement in a UK national cohort of juvenile-onset SLE (JSLE) patients. METHODS: JSLE patients (n = 241) from 15 different centres whose diagnosis fulfilled four or more of the ACR criteria were divided into two groups: those with at least one ACR mucocutaneous criterion (ACR skin feature positive) and those without (ACR skin feature negative) at diagnosis. The relative frequency of skin involvement was described by the paediatric adaptation of the 2004 British Isles Lupus Assessment Group (pBILAG-2004) index. RESULTS: One hundred and seventy-nine patients (74%) had ACR-defined skin involvement with no significant demographic differences compared with those without. ACR skin feature negative patients showed greater haematological (84% vs 67%), renal (43% vs 26%) (P < 0.05) and neurological (16% vs 4%) involvement (P = 0.001). Forty-two per cent of ACR skin feature negative patients had skin involvement using pBILAG-2004, which included maculopapular rash (17%), non-scaring alopecia (15%), cutaneous vasculitis (12%) and RP (12%). ACR skin feature negative patients with moderate to severe skin involvement by pBILAG-2004 showed greater renal and haematological involvement at diagnosis and over the follow-up period (P < 0.05). Higher immunosuppressive drug use in the skin feature negative group was demonstrated. CONCLUSION:Patients who fulfil the ACR criteria but without any of the mucocutaneous criteria at diagnosis have an increased risk of major organ involvement. The pBILAG-2004 index has shown that other skin lesions may go undetected using the ACR criteria alone, and these lesions show a strong correlation with disease severity and major organ involvement.
Authors: Mario Diplomatico; Pierluigi Marzuillo; Angela La Manna; Andrea Apicella; Stefano Guarino; Vincenzo Piccolo Journal: Dermatol Pract Concept Date: 2020-10-26
Authors: Pongsawat Rodsaward; Titipong Prueksrisakul; Tawatchai Deekajorndech; Steven W Edwards; Michael W Beresford; Direkrit Chiewchengchol Journal: Am J Clin Dermatol Date: 2017-12 Impact factor: 7.403
Authors: Tom Dudding; Simon Haworth; Penelope A Lind; J Fah Sathirapongsasuti; Joyce Y Tung; Ruth Mitchell; Lucía Colodro-Conde; Sarah E Medland; Scott Gordon; Benjamin Elsworth; Lavinia Paternoster; Paul W Franks; Steven J Thomas; Nicholas G Martin; Nicholas J Timpson Journal: Nat Commun Date: 2019-03-05 Impact factor: 14.919