Childhood-Onset SLE Often Results in Damage to Several Organ Systems

doctor examining young girl
doctor examining young girl
Disease damage in patients with childhood-onset SLE is common, even with relatively short disease durations.

The Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) indicates that disease damage in the cutaneous, musculoskeletal, and neuropsychiatric organs is common in pediatric patients with childhood-onset systemic lupus erythematosus (SLE), according to study findings published in Arthritis Care & Research.

Investigators prospectively collected SDI information from patients with childhood-onset SLE (n=1048). Researchers also included physician-rated disease-related damage severity as rated by the visual analog scale (MD-VASdamage) in a subset of 559 patients. Patient data were collected from the United Kingdom Juvenile-onset SLE Cohort Study (n=350), the childhood-onset SLE cohort at Cincinnati Children’s Hospital Medical Center (n=139), and an international cohort (n=559) consisting of patients from Latin America, Australia, Asia, and Europe from the Pediatric Rheumatology International Trials Organization.

Results showed that 44.2% (n=463) of patients had an SDI summary-score >0 (maximum: 14) after an average disease duration of 3.8 years, indicating that almost half of patients with childhood-onset SLE experience disease damage. Cognitive impairment, proteinuria, and scarring alopecia were the most frequently scored SDI items in this patient population. A significant association between SDI summary-scores and MD-VASdamage (P <.0001) existed in patients with disease damage. The kidney, skin, musculoskeletal, and neuropsychiatric organ systems were the most frequently damaged systems.

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Although this study relied on physician ratings for assessing damage severity, only data from select patients were available for assessment. In addition, there may have been inter-rater and intra-rater variations in physician global assessments, which may have limited the findings.

Despite the usefulness of the SDI in this patient population, the investigators agreed that the current form of this index “is inadequate for estimating the severity of [childhood-onset] SLE-associated damage.”

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Holland MJ, Beresford MW, Feldman BM, et al. Measuring disease damage and its severity in childhood-onset systemic lupus erythematosus [published online February 6, 2018]. Arthritis Care Res (Hoboken). doi:10.1002/acr.23531