Disease activity, severity, and damage in the UK juvenile‐onset systemic lupus erythematosus cohort

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 nonCaucasian 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

Transition of Care and Health‐Related Outcomes in Pediatric‐Onset Systemic Lupus Erythematosus

“…Maximizing accessibility to health care may diminish the financial difficulties surrounding transition and impact medical outcomes. However, outcome data from countries with a differently structured health care system, such as the UK and France, report comparable findings with regard to morbidity and mortality among young adults (45,46).…”

Європейські Доказові Рекомендації По Діагностиці Та Лікуванню Люпус-Нефриту У Дітей: Ініціатива SHARE

“…SLE in children is rare, with a prevalence ranging from 1.89 to 25.7 per 100,000 children and an incidence of 0.3 to 0.9 per 100,000 childrenyears worldwide (reviewed in (2)(3)(4)). cSLE and SLE share the same pathogenesis, but in general, cSLE appears to have a more severe phenotype (5)(6)(7)(8). 50%-60% of patients with cSLE will develop lupus nephritis (LN) (5)(6)(7)(8).…”

“…cSLE and SLE share the same pathogenesis, but in general, cSLE appears to have a more severe phenotype (5)(6)(7)(8). 50%-60% of patients with cSLE will develop lupus nephritis (LN) (5)(6)(7)(8). Timely and accurate recognition of renal involvement combined with appropriate treatment choices will optimise clinical outcome and decrease renal-associated morbidity and mortality (1).…”

Appearance concern and depression in adolescent girls with systemic lupus erythematous

“…The onset of SLE occurs in childhood or adolescence and accounts for around 15 % of affected individuals [1]. Young persons with SLE generally have severe disease at onset, requiring high-dose corticosteroid and immunosuppressive drugs for disease control [2].…”