Juvenile-onset systemic lupus erythematosus: Update on clinical presentation, pathophysiology and treatment options

Smith, Eve Mary Dorothy, Lythgoe, Hanna, Midgley, Angela, Beresford, Michael William ORCID: 0000-0002-5400-9911 and Hedrich, Christian Michael ORCID: 0000-0002-1295-6179 (2019) Juvenile-onset systemic lupus erythematosus: Update on clinical presentation, pathophysiology and treatment options. CLINICAL IMMUNOLOGY, 209. 108274-.

Text
Update on Lupus in children revised unmarked 11.10.19.docx - Author Accepted Manuscript
Download (589kB)

Official URL: http://dx.doi.org/10.1016/j.clim.2019.108274

Abstract

Juvenile-onset systemic lupus erythematosus (jSLE) accounts for up to 20% of all SLE patients. Key differences between juvenile- and adult-onset (aSLE) disease include higher disease activity, earlier development of damage, and increased use of immunosuppressive treatment in jSLE suggesting (at least partial) infectivity secondary to variable pathomechanisms. While the exact pathophysiology of jSLE remains unclear, genetic factors, immune complex deposition, complement activation, hormonal factors and immune cell dysregulation are involved to variable extents, promising future patient stratification based on immune phenotypes. Though less effective and potentially toxic, jSLE patients are treated based upon evidence from studies in aSLE cohorts. Here, age-specific clinical features of jSLE, underlying pathomechanisms, treatment options and disease outcomes will be addressed. Future directions to improve the care of jSLE patients, including implementation of the Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) recommendations, biomarkers, treat to target and personalized medicine approaches are discussed.

Item Type:	Article
Uncontrolled Keywords:	Childhood, Systemic lupus erythematosus, Juvenile, JSLE, Management, Pathogenesis
Depositing User:	Symplectic Admin
Date Deposited:	10 Jan 2020 11:17
Last Modified:	19 Jan 2023 00:11
DOI:	10.1016/j.clim.2019.108274
Related URLs:	Author Publisher
URI:	https://livrepository.liverpool.ac.uk/id/eprint/3069381