TY - JOUR
T1 - Disease activity, severity, and damage in the UK Juvenile-Onset Systemic Lupus Erythematosus Cohort
AU - Watson, Louise
AU - Leone, Valentina
AU - Pilkington, Clarissa
AU - Tullus, Kjell
AU - Rangaraj, Satyapal
AU - McDonagh, Janet E.
AU - Gardner-Medwin, Janet
AU - Wilkinson, Nick
AU - Riley, Phil
AU - Tizard, Jane
AU - Armon, Kate
AU - Sinha, Manish D.
AU - Ioannou, Yiannis
AU - Archer, Neil
AU - Bailey, Kathryn
AU - Davidson, Joyce
AU - Baildam, Eileen M.
AU - Cleary, Gavin
AU - McCann, Liza J.
AU - Beresford, Michael W.
PY - 2012/7
Y1 - 2012/7
N2 - 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 aSystemic 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.
AB - 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 aSystemic 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.
UR - http://www.scopus.com/inward/record.url?scp=84863194095&partnerID=8YFLogxK
U2 - 10.1002/art.34410
DO - 10.1002/art.34410
M3 - Article
C2 - 22294381
AN - SCOPUS:84863194095
SN - 0004-3591
VL - 64
SP - 2356
EP - 2365
JO - Arthritis and Rheumatism
JF - Arthritis and Rheumatism
IS - 7
ER -