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Vasculitis in Juvenile-Onset Systemic Lupus Erythematosus

Author

Smith EMD1,2, Lythgoe H3, Hedrich CM1,2. Front Pediatr. 2019 May 9;7:149. doi: 10.3389/fped.2019.00149. eCollection 2019.

Author Information

1 Department of Women and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, United Kingdom.

2 Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, United Kingdom.

3 St Helen's and Knowsley Teaching Hospital NHS Trust, St Helens, United Kingdom.

Abstract

Juvenile-onset systemic lupus erythematosus (JSLE) is a rare, heterogeneous multisystem autoimmune disease that can affect any organ, and present with diverse clinical and serological manifestations. Vasculitis can be a feature of JSLE. It more commonly presents as cutaneous vasculitis than visceral vasculitis, which can affect the central nervous system, peripheral nervous system, lungs, gut, kidneys, heart, and large vessels. The incidence and prevalence of vasculitis in JSLE has not been well described to date. Symptoms of vasculitis can be non-specific and overlap with other features of JSLE, requiring careful consideration for the diagnosis to be achieved and promptly treated. Biopsies are often required to make a definitive diagnosis and differentiate JSLE related vasculitis from other manifestations of JSLE, vasculopathies, and JSLE related antiphospholipid syndrome. Visceral vasculitis can be life threatening, and its presence at the time of JSLE diagnosis is associated with permanent organ damage, which further highlights the importance of prompt recognition and treatment. This review will focus on the presentation, diagnosis, management and outcomes of vasculitis in JSLE, highlighting gaps in the current evidence base.