Abstract
PURPOSE OF REVIEW: Provide an update of studies published in last 2 years on the outcomes and therapies in childhood-onset systemic lupus erythematous (cSLE). RECENT FINDINGS: Additional evidence has been provided about the benefits of universal hydroxychloroquine in SLE patients, although antimalarial maculopathy may be more prevalent than previously thought. Recent studies support lower glucocorticoid doses than used in the past may provide comparable therapeutic benefits, and cSLE patients can mount adequate immunogenic response and sustain long-term seroprotective titers when vaccinated. Long-term studies of adults with cSLE confirmed that damage accrual increases with disease duration. Cardiovascular disease, renal transplants, replacement arthroplasties, and myocardial infarctions occur between 20 and 40 years of age. Higher prednisone doses predicted higher damage trajectory and antimalarial exposure was protective. There were no prospective clinical trials published in pediatric patients with cSLE, but positive results from phase II trials with bariticinib and ustekinumab in adult SLE may raise the expectation that these drugs could be beneficial when used in cSLE. SUMMARY: The dire need for more clinical trials and licensed medications for cSLE persist as well as decreasing damage accrual.