Background/Purpose: Systemic lupus erythematosus (SLE) is a prototypic autoimmune disease that is characterized by the presence of antinuclear autoantibodies, complement activation and the formation and deposition of immune complexes resulting in multisystem organ damage. Trends in SLE vary by age, with pediatric SLE (pedSLE) patients presenting with more active, severe and rapidly progressive disease than adult SLE patients. However, the basis for this variation is unknown. In this investigation, we sought to characterize the clinical phenotype associated with the age of diagnosis including pedSLE, adult SLE and late onset SLE in a large multiethnic cohort. 

Methods: We evaluated clinical manifestations, disease course and severity (per the Systemic Lupus International Collaborating Clinics/ACR Damage Index) and autoantibody profiles using a total of 2,564 SLE patients with a cumulative presence of at least 4 of 11 revised and updated ACR classification criteria for SLE, self-defined race/ethnicity (African American, European American, Hispanic), disease duration £10 years at enrollment and with at least 2 years follow up included in the PROFILE longitudinal cohort. Patients were stratified by age of onset including pedSLE (≤16 years), adult SLE (17-49 years) and late onset SLE (≥ 50 years). Risk estimates were calculated using multivariable logistic regression, although frequencies are provided herein.

Results: A total of 2,564 SLE patients were evaluated. The mean (±standard deviation, SD) age at diagnosis in each of three age strata was 14±3 years, 32±9 years and 58±7 years for pedSLE, adult SLE and late onset SLE, respectively and the mean (±SD) disease duration was 10.9±8.7 years, 5.7±5.8 years and 3.8±3.4 years for each of the respective groups. Across each age of onset group, the majority were female (≥86%) and slightly more pedSLE patients were of African American (38%) or Hispanic (13%) ancestry. We confirm that compared to SLE patients with adult onset, pedSLE patients are more likely to present with acute and severe disease features including discoid rash (27% vs. 18%), serositis (48% vs. 44%), renal involvement (62% vs. 41%), neurologic involvement (18% vs. 10%), hematologic involvement (73% vs. 68%) and immunological involvement (89% vs. 79%). In contrast, SLE patients with late onset were significantly less likely to present with serositis (34%), renal involvement (20%), neurological involvement (8%) and immunological involvement (67%) compared to each of the pedSLE and adult onset SLE groups. Consistent with increasing age, the late onset SLE group demonstrated more damage associated with ocular, neuropsychiatric, pulmonary, cardiovascular, musculoskeletal, diabetes and malignancy domains than did the pedSLE or adult SLE groups, whereas the pedSLE group had significantly more renal damage.

Conclusion: These findings confirm more aggressive disease, particularly renal involvement, in SLE patients presenting in childhood. They further suggest SLE disease manifestations decrease with age of onset throughout adulthood. These results will inform future studies aimed at delineating the etiology and natural history of the more aggressive clinical phenotype observed in children.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/differences-in-the-sle-clinical-phenotype-by-age-of-diagnosis/