Systemic Lupus Erythematosus in Children and Young People

AbstractPurpose of ReviewJuvenile-onset systemic lupus erythematosus ((j)SLE) is an autoimmune/inflammatory disease that results in significant damage and disability. When compared to patients with disease onset in adulthood, jSLE patients exhibit increased disease activity, damage and require more aggressive treatments. This manuscript summarises age-specific pathogenic mechanisms and underscores the need for age group –specific research, classification and treatment.Recent FindingsGenetic factors play a significant role in the pathophysiology of jSLE, as>  7% of patients develop disease as a result of single gene mutations. Remaining patients carry genetic variants that are necessary for disease development, but require additional factors. Increased ‘genetic impact’ likely contributes to earlier disease onset and more severe phenotypes. Epigene tic events have only recently started to be addressed in jSLE, and add to the list of pathogenic mechanisms that may serve as biomarkers and/or treatment targets. To allow meaningful and patient-oriented paediatric research, age-specific classification criteria and treatment targets require to be de fined as currently available tools established for adult-onset SLE have limitations in the paediatric cohort.SummarySignificant progress has been made in understanding the pathophysiology of jSLE. Meaningful laboratory and clinical research can only be performed using age group –specific tools, classification criteria and tre...

Source: Current Rheumatology Reports - February 10, 2021 Category: Rheumatology Source Type: research