ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2664

Long-Term Clinical Outcomes in a Cohort of Adults with Childhood-Onset Systemic Lupus Erythematosus

Noortje Groot1, Y.K. Onno Teng2, Karina de Leeuw3, Marc Bijl4, Radboud J. E. M. Dolhain5, Els J. Zirkzee6, Ruth D.E. Fritsch-Stork7, Irene E.M. Bultink8 and Sylvia S.M. Kamphuis9, 1Pediatric Rheumatology, Sophia Children's Hospital – Erasmus University Medical Centre, Rotterdam, Netherlands, 2Department of Nephrology, Leiden University Medical Center, Leiden, the Netherlands, Leiden, Netherlands, 3Rheumatology and Clinical Immunology, University Medical Center Groningen, Groningen, Netherlands, 4Internal Medicine and Rheumatology, Martini Hospital, Groningen, Netherlands, 5Rheumatology, Erasmus University Medical Centre, Rotterdam, Netherlands, 6Department of Rheumatology, Maasstad Hospital, Rotterdam, Netherlands, 7Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, Netherlands, 8Rheumatology, Amsterdam Rheumatology and immunology Center | VU University Medical Center, Amsterdam, Netherlands, Amsterdam, Netherlands, 9Pediatric Rheumatology, Sophia Children's Hospital – Erasmus University Medical Center, Rotterdam, Netherlands

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: ,

Session Information

Date: Tuesday, October 23, 2018

Session Title: Systemic Lupus Erythematosus – Clinical Poster III: Treatment

Session Type: ACR Poster Session C

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

Background/Purpose: Childhood-onset SLE (cSLE) is a severe lifelong multisystem autoimmune disease. Long-term outcome data are limited. Here, we report for the first time on the development of disease manifestations and damage over time in adults with cSLE and their health-related quality of life (HRQOL).

Methods: All patients underwent a single study visit comprising a structured history and physical examination. Disease activity (SLEDAI-2K), damage (SLICC-Damage Index (SDI)) and HRQOL (SF-36) were determined. Medical records were retrieved.

Results: In total, 111 cSLE patients were included, median disease duration 20 years, 91% female and 72% white. Disease activity was low (median SLEDAI 4), with 68% of patients using prednisone and/or disease-modifying anti-rheumatic drugs. The vast majority of new cSLE-related manifestations developed within 2 years of diagnosis. Damage like myocardial infarctions started occurring after 5 years. Most patients (62%) had damage, predominantly in the musculoskeletal, neuropsychiatric and renal systems. Cerebrovascular accidents, renal transplants, replacement arthroplasties and myocardial infarctions, developed at young age (median age 20, 24, 34 and 39 years respectively). Multivariate logistic regression showed that damage accrual was associated with disease duration (OR=1.15;p<0.001), antiphospholipid-antibody positivity (OR=03.56;p=0.026), and hypertension (OR=3.21;p=0.043). Current HCQ-monotherapy was associated with an SDI-score of 0 (OR=0.16;p=0.009). HRQOL was impaired compared to the Dutch population. Presence of damage reduced HRQOL in only one domain. High disease activity (SLEDAI≥8) and changes in physical appearance strongly reduced HRQOL (4/8 and 7/8 domains).

Conclusion: The majority of adults with cSLE in this large cohort developed significant damage at young age and have impaired HRQOL without achieving drug free remission, illustrating the great impact of cSLE on future life.

Disclosure: N. Groot, None; Y. K. O. Teng, None; K. de Leeuw, None; M. Bijl, None; R. J. E. M. Dolhain, None; E. J. Zirkzee, None; R. D. E. Fritsch-Stork, None; I. E. M. Bultink, None; S. S. M. Kamphuis, None.

To cite this abstract in AMA style:

Groot N, Teng YKO, de Leeuw K, Bijl M, Dolhain RJEM, Zirkzee EJ, Fritsch-Stork RDE, Bultink IEM, Kamphuis SSM. Long-Term Clinical Outcomes in a Cohort of Adults with Childhood-Onset Systemic Lupus Erythematosus [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/long-term-clinical-outcomes-in-a-cohort-of-adults-with-childhood-onset-systemic-lupus-erythematosus/. Accessed February 3, 2023.

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