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Vasculitis in Juvenile-Onset Systemic Lupus Erythematosus
Eve M D Smith1,2, Hanna Lythgoe3, Christian M Hedrich1,2
1Department of Women and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, United Kingdom.
Frontiers in Pediatrics
|May 31, 2019
Summary
Juvenile-onset systemic lupus erythematosus (JSLE) can involve vasculitis, affecting various organs. Early diagnosis and treatment are crucial to prevent severe organ damage in children with JSLE.
Area of Science:
- Pediatric Rheumatology
- Autoimmune Diseases
- Vasculitis
Background:
- Juvenile-onset systemic lupus erythematosus (JSLE) is a rare, multisystem autoimmune disease with diverse clinical presentations.
- Vasculitis, inflammation of blood vessels, is a potential manifestation of JSLE, occurring more frequently in the skin than internally.
- The incidence, prevalence, and diagnostic challenges of vasculitis in JSLE remain incompletely understood.
Purpose of the Study:
- To review the presentation, diagnosis, management, and outcomes of vasculitis in JSLE.
- To highlight the critical need for prompt recognition and treatment of vasculitis in JSLE.
- To identify gaps in the current evidence base regarding vasculitis in JSLE.
Main Methods:
- Literature review focusing on vasculitis in JSLE.
- Analysis of clinical and serological manifestations.
- Discussion of diagnostic strategies, including biopsy.
- Examination of management approaches and patient outcomes.
Main Results:
- Vasculitis in JSLE can affect multiple organs, including the central nervous system, lungs, kidneys, and heart.
- Symptoms of vasculitis may be non-specific and overlap with other JSLE features.
- Visceral vasculitis is associated with life-threatening complications and permanent organ damage if not promptly treated.
Conclusions:
- Vasculitis is a significant concern in JSLE, requiring careful diagnostic consideration.
- Timely diagnosis and intervention are essential to mitigate severe outcomes and organ damage in pediatric patients.
- Further research is needed to address current evidence gaps in the understanding and management of vasculitis in JSLE.
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