ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 392

From Childhood to Adulthood: Longitudinal Trajectory of Damage in Patients with Childhood-Onset Systemic Lupus Erythematosus (cSLE)

Lily Siok Hoon Lim1,2,3,4, Eleanor Pullenayegum5, Lillian Lim6, Dafna Gladman7, Brian Feldman8 and Earl Silverman9, 1Institute of Health Policy Management and Evaluation, University of Toronto, Toronto, ON, Canada, 2Rheumatology, SickKids, Toronto, ON, Canada, 3Rheumatology, Hospital for Sick Children, Toronto, ON, Canada, 4Children's Hospital Research Institute of Manitoba, University of Manitoba, Winnipeg, MB, Canada, 5Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, ON, Canada, 6Pediatrics, The Hospital for Sick Children, Toronto, ON, Canada, 7Rheumatology, University of Toronto, Toronto Western Hospital, Toronto, ON, Canada, 8Division of Rheumatology, The Hospital for Sick Children and University of Toronto, Toronto, ON, Canada, 9Division of Rheumatology, Hospital for Sick Children and University of Toronto, Toronto, ON, Canada

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: , , ,

Session Information

Date: Sunday, November 8, 2015

Title: Pediatric Rheumatology – Clinical and Therapeutic Aspects Poster I: Lupus, Scleroderma, JDMS

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose: Outcomes of patients with cSLE over time and into adulthood are poorly understood. There has been no information about the longitudinal trajectory of organ damage in cSLE patients. We undertook this study to: 1) determine the longitudinal damage trajectory– as measured by the American College of Rheumatology (ACR)/ SLE International Collaborating Clinics (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. To be included, 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 pediatric-age data was obtained from our research database while adult-age 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.  Longitudinal trajectory of damage fit best with a 2ndorder fractional polynomial (0.5,1). Predictors were identified using a weighted generalized 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 in different models. 

Results: 67/473  (14%) patients were lost to follow-up. There were 14097 visits, totaling 3290 patient-years.  The median follow-up duration was 5.5 years. The median age at diagnosis was 14.1 years and median age at last visit was19.5 years (range 6.0–41.9 years). 65% 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%) was the most common item of damage, followed by avascular necrosis (10%) and osteoporosis (5%). Only 2 patients had myocardial infarctions. Life-threatening major organ manifestations predicted higher initial damage but the accrual of damage 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. We identified the baseline factors that predict higher initial damage and influence damage trajectory. Over time, SLE clinical features and therapeutic exposures during the course of disease, predicted a change in damage trajectory.

Disclosure: L. S. H. Lim, None; E. Pullenayegum, None; L. Lim, None; D. Gladman, None; B. Feldman, None; E. Silverman, None.

To cite this abstract in AMA style:

Lim LSH, Pullenayegum E, Lim L, Gladman D, Feldman B, Silverman E. From Childhood to Adulthood: Longitudinal Trajectory of Damage in Patients with Childhood-Onset Systemic Lupus Erythematosus (cSLE) [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/from-childhood-to-adulthood-longitudinal-trajectory-of-damage-in-patients-with-childhood-onset-systemic-lupus-erythematosus-csle/. Accessed .

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