Systemic Lupus Erythematosus in Children and Young People

Abstract

Purpose of Review Juvenile-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 Findings Genetic 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. Epigenetic 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 defined as currently available tools established for adult-onset SLE have limitations in the paediatric cohort. Summary Significant 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 treatment targets.

Çocuklarda sistemik lupus eritematozusun klinik özellikleri ve uzun dönem sonuçları

“…The current study showed that most cases of cSLE appeared after the pubertal period (>12 years of age). It might be explained by the hormonal effects on SLE patients after puberty has been also observed in most studies (5).…”

Comparison of the sensitivity of the EULAR / ACR 2019 and SLICC 2012 classification criteria in a Colombian population with systemic lupus erythematosus

“…Childhood-onset lupus, unlike adult-onset, is more aggressive with high disease activity, greater use of immunosuppressants, and accumulated damage that causes greater morbidity and mortality [ 27 ]. Despite these important differences, there are no specific classification criteria for this population group, which is why, over time, the same criteria have been used as in adults.…”

Drug delivery systems as immunomodulators for therapy of infectious disease: Relevance to COVID-19

“…Furthermore, later in disease, as a result of tissue damage and cell death, type 1 interferon expression may “derail” and perpetuate inflammation and damage [9] . This is likely of significant relevance, as pathological spontaneous and/or increased activation of the type 1 interferon pathway is associated with autoimmune/inflammatory conditions, such as SLE, that share some clinical aspects of COVID-19, including cytokine storm, tissue damage, thromboembolic events, end others [9] , [43] . Below, we discuss the involvement of particular immune mechanisms, in COVID-19, and how drug delivery systems, such as nanoformulation, may improve therapeutic options.…”