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Most adults with childhood-onset SLE incur significant early damage
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Most adults with childhood-onset systemic lupus erythematosus develop significant damage at a young age and do not achieve drug-free remission, negatively impacting their health-related quality of life, according to findings published in Arthritis & Rheumatology.
“In children with [childhood-onset] SLE, health-related quality of life (HRQoL) has been shown to be impaired compared to healthy peers, which was at least partially attributed to disease activity and damage,” Sylvia Kamphuis, MD, PhD, of Sophia Children’s Hospital-Erasmus University Medical Center, the Netherlands, and colleagues wrote. “There are no data available regarding HRQoL in patients with [childhood-onset] SLE after reaching adulthood, and which factors could influence HRQoL in these patients.”
To determine the long-term clinical characteristics and quality of life among adult patients with childhood-onset SLE, the researchers contacted rheumatologists and other specialists at 88 Dutch hospitals and asked them to identify adult patients in their care who were diagnosed with lupus prior to their 18th birthday. Patients who agreed to participate met with researchers for a single 1.5-hour visit, where they completed data collection forms and questionnaires. In all, 111 patients participated in the study.
Information collected during the study visit included demographics, current health, disease activity, damage, disease onset and progression over time, and current and previous medication use. All information was verified using medical records. Participants also answered questions regarding effects of medication use on physical appearance, physical health and mental health, as well as education and employment, fertility and family planning, fatigue, depression, coping and resilience. Kamphuis and colleagues focused their analysis on disease activity, medication use, disease manifestations over time, damage and quality of life.
According to the researchers, the 111 participants had a median disease duration of 20 years, and disease activity was generally low, with a median SLEDAI score of 4. Among the participants, the majority of new lupus-related manifestations developed within 2 years of the initial diagnosis, with damage, such as myocardial infarctions, starting after year 5.
In addition, 62% of the patients experienced damage predominantly in the musculoskeletal, neuropsychiatric and renal systems. The researchers also noted that cerebrovascular accidents, renal transplants, replacement arthroplasties and myocardial infarctions appeared at young age, developing at a median age of 20, 24, 34 and 39, respectively.
Comparing the participants to the general Dutch population, damage occurrence reduced health-related quality of life in one domain. In addition, high disease activity and alterations in physical appearance strongly impaired quality of life.
“This is the first study to give insight into the development of disease manifestations and damage over time in adults with [childhood-onset] SLE and their HRQoL,” Kamphuis and colleagues wrote. “These results urge for optimal control of disease activity and (cardiovascular) preventative screening measures, starting before the age of 30, to facilitate a better disease prognosis. HRQoL of adults with [childhood-onset] SLE is impaired and affected by other factors than disease activity or damage alone. By identifying and addressing these factors, like physical appearance and potentially coping styles, HRQoL may be improved.” – by Jason Laday
Disclosure: The researchers report financial support from the Dutch Arthritis Foundation and the National Association for Lupus, APS, Scleroderma and MCTD.
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