Risk Factors Determined for Pediatric-Onset Discoid and Systemic Lupus Erythematosus

Baseline risk factors for pediatric patients with discoid lupus erythematosus (DLE) and systemic lupus erythematosus (SLE) included being adolescents, positive serologies, and the presence of end-organ damage, according to study results in the Journal of the American Academy of Dermatology.

Though rare in children, DLE causes scarring on the face at a developmentally-sensitive time. Children with DLE are also at risk for SLE, which is more aggressive when diagnosed in childhood than during adulthood.

In a multicenter retrospective observational study, researchers assessed baseline risk factors for DLE and SLE in a pediatric population.

Researchers identified data from patients who were aged 18 years and younger with DLE and reviewed their medical records manually to validate the diagnosis. Clinical and laboratory variables were collected to determine if patients also had SLE at their baseline visit, based on the 1997 American College of Rheumatology (ACR) classification criteria (ACR classification criteria, ³4).

A total of 438 patients were included in the study. Patients with DLE and SLE were more likely to be of the female sex, Black or Asian, and an adolescent (aged between 10 and 19 years). Overall, the female to male ratio was 2.6:1, and 4.1:1 in patients with DLE plus SLE, and 2.1:1 in patients with DLE only. Among 151 patients who were Black and 40 who were Asian, 42% and 58% were diagnosed with SLE at the baseline visit, respectively, compared with 23% and 27% of patients who were White and Hispanic, respectively. The median age at DLE rash-onset was 8.9 years for patients with DLE only compared with 12.9 years for patients with DLE plus SLE (P <.001).

The ACR clinical characteristics were more prevalent in patients with DLE plus SLE vs those with DLE only, including lesions both above and below the neck (39% vs 17%; P <.001), malar rash (45% vs 4%; P <.001), photosensitivity (38% vs 11%; P <.001), and mucosal ulcers (39% vs 4%; P <.001). Among patients with DLE plus SLE, 41% had single-organ involvement (1 of arthritis, renal disease, seizures psychosis, pleuritis, or pericarditis) and 23% had involvement of 2 or more organ systems.

Laboratory data showed immunologic evidence of SLE in patients with DLE plus SLE, with nearly all immunologic laboratory measures present in more than 25% of patients (P <.001).

Limitations of the study included the retrospective design, which did not allow the researchers to measure disease activity or compare the onset of DLE and SLE. There were also concerns about the quality and completeness of the data including potentially inconsistent antinuclear antibody (ANA) assay sensitivity and lack of follow-up data for patients with DLE plus SLE. Finally, the researchers noted the possibility of referral bias as all sites were tertiary centers.

The researchers concluded, “In adolescence, diagnosis of DLE should prompt thorough screening for SLE including referral to rheumatology and consideration of [SLE screening labs]…” They added, “Continued collaboration between dermatologists and rheumatologists will facilitate better understanding of this disease in children.”

Disclosure: Some study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors’ disclosures.

Reference

Ezeh, N, Ardalan K, Buhr K, et al. Cross-sectional characteristics of pediatric-onset discoid lupus erythematosus: results of a multicenter, retrospective cohort study. J Am Acad Dermatol. Published online April 25, 2022. doi:10.1016/j.jaad.2022.04.028