Background Outcomes of patients with cSLE into adulthood are poorly understood. There is no information about the longitudinal trajectory of damage in cSLE patients. We undertook this study to: 1) Determine the longitudinal damage trajectory– as measured by the ACR/SLICC SLE damage index (SDI)– in patients with cSLE. 2) Identify both baseline and disease course (time-varying) predictors of damage trajectory.
Methods Single centre, retrospective, inception cohort. We included 473 patients who were diagnosed and followed, from 1st January 1985 to 30th September 2011. Patients had to be <18 years at diagnosis, have satisfied the ACR classification criteria for SLE, were treated for <3 months with steroids or an immunosuppressant for any other disease, and have had at least 3 visits. Longitudinal childhood data was obtained from our database while adulthood data was obtained from either a research database or patients’ charts. Clinical information at every visit was collected: for SLE disease activity index 2000 (SLEDAI2K), the SDI, laboratory results, and medications. Predictors were identified using a weighted generalised estimating equation (WGEE). Time-varying predictors: disease activity, individual items of SLEDAI2K, corticosteroid, immunosuppressant and antimalarial exposures, were lagged by 6, 12, 18 and 24 months prior to each visit.
Results 67/473 (14%) patients were lost to follow-up. There were 14097 visits, 3290 patient-years. The median follow-up duration was 5.5 years, median age at diagnosis was 14.1 years and median age at last visit was19.5 years (range 6.0–41.9 years). 67% of patients were >18 years old at last follow-up. The predicted average population damage was 0.7 at 5 years, 1.3 at 10 years, 1.9 at 15 years, 2.3 at 20 years and 2.7 at 25 years. Cataract (14%), avascular necrosis (10%) and osteoporosis (5%) were the commonest damage items. Only 2 had myocardial infarctions. Life-threatening major organ manifestations predicted higher initial damage but the accrual slowed down over time. Higher prednisone dose (12, 24 months before) and the use of cyclophosphamide (6, 12,18, 24 months before) predicted an increased damage trajectory at current visit. Antimalarial exposure (6 months before), mucosal ulcers (6, 12, 18, 24 months before) and pericarditis (6 months before) were protective against an increase in damage trajectory.
Conclusion Patients with cSLE accrue damage steadily throughout their disease course into adulthood. Baseline factors that predicted higher initial damage and influenced damage trajectory. SLE clinical features and therapeutic exposures during the course of disease, predicted a change in damage trajectory.
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.