Childhood-Onset Systemic Lupus Erythematosus Negatively Affects Quality of Life in Adulthood

young girl holding her knee
young girl holding her knee
Childhood-onset SLE has major effects on quality of life in adulthood.

Adults with childhood-onset systemic lupus erythematosus (cSLE) have evidence of significant damage at a young age and have an impaired health-related quality of life (HRQoL), according to the results of a study published in Arthritis & Rheumatology. The study also found that the HRQoL of adults with cSLE was affected by factors other than disease activity or damage alone, including disease effects on physical appearance.

Childhood-onset SLE, defined as onset before 18 years of age, is a rare disease, with an incidence of 0.3 to 0.9 per 100,000 person-years and a prevalence of 1.89 to 25.7 per 100,000 children worldwide. To date, long-term studies of cSLE are limited and no data are available regarding HRQoL in patients with cSLE after they reach adulthood.

Researchers analyzed data from the Childhood-onset SLE in the Netherlands (CHILL-NL) study to determine the effect of disease course and damage accrual over time on HRQoL. The CHILL-NL study included all patients with cSLE who were older than 18 years, treated in any Dutch hospital, and who met the American College of Rheumatology criteria for SLE. Patients underwent a single study visit that included a structured history and physical examination. The investigators assessed disease activity using the SLE Disease Activity Index (SLEDAI-2K) and measured damage using the SLE International Collaborating Clinics Damage Index. They also evaluated HRQoL and retrieved medical records.

The investigators included 111 patients with cSLE with a median disease duration of 20 years, 91% of whom were women and 72% of whom were white. The median SLEDAI was 4, and 71% of patients used prednisone, hydroxychloroquine, or disease-modifying antirheumatic drugs. Most disease manifestations occurred within 2 years of diagnosis, and damage such as myocardial infarctions began to occur 5 years after diagnosis. Sixty-two percent of patients had damage, mostly in the musculoskeletal, neuropsychiatric, and renal systems. Damage accrual was associated with disease duration (odds ratio [OR], 1.15; P <.001) antiphospholipid-antibody positivity (OR, 3.56; P =.026), and hypertension (OR, 3.21; P =.043). The median age for cerebrovascular accidents, renal transplants, replacement arthroplasties, and myocardial infarctions was 20, 24, 34, and 39 years, respectively. HRQoL was impaired in patients with cSLE compared with the general Dutch population. High disease activity (SLEDAI ≥8) and alterations in physical appearance had significant negative effects on HRQoL.

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The authors note that the study is limited by the patient population, which may have excluded patients with more severe disease. They suggest this could explain the relatively low renal involvement in the CHILL-NL cohort compared with other cSLE studies. The authors chose to report only disease characteristics that could be verified with medical records, and no data were available from deceased patients.

The authors suggest that by identifying and addressing factors such as physical appearance in patients with cSLE, HRQoL may be improved.

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Groot N, Shaikhani D, Teng YKO, et al. Long-term clinical outcomes in a cohort of adults with childhood-onset systemic lupus erythematosus [published online August 27, 2018]. Arthritis Rheumatol. doi: 10.1002/art.40697