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Paediatric-onset systemic lupus erythematosus

Clara Malattia1, Alberto Martini

  • 1Department of Pediatrics, University of Genoa and Pediatria e Reumatologia, G. Gaslini Institute, Genoa, Italy.

Best Practice & Research. Clinical Rheumatology
|November 19, 2013
PubMed

Insights

Pediatric-onset systemic lupus erythematosus (SLE) is more severe in children, often affecting the kidneys and central nervous system. Early-onset SLE may indicate rare genetic or congenital conditions.

Area of Science:

  • Pediatric Rheumatology
  • Immunology
  • Genetics

Background:

  • Pediatric-onset systemic lupus erythematosus (SLE) presents with greater severity compared to adult-onset SLE.
  • Renal and central nervous system involvement are more frequent in pediatric SLE.
  • Early-onset SLE, particularly before age five, is uncommon.

Purpose of the Study:

  • To highlight the distinct characteristics and severity of pediatric SLE.
  • To emphasize the importance of considering monogenic or congenital diseases in early-onset SLE cases.
  • To inform clinical suspicion for rare underlying conditions in young children with SLE symptoms.

Main Methods:

  • Review of clinical presentations and disease characteristics in pediatric SLE patients.
  • Analysis of disease activity and damage assessment measures.
  • Correlation of early-onset SLE with genetic and congenital disease associations.

Main Results:

  • Pediatric SLE demonstrates a more aggressive disease course than adult SLE.
  • Higher rates of kidney and central nervous system complications are observed in children.
  • SLE onset before five years of age warrants investigation for rare genetic or congenital disorders.

Conclusions:

  • Early diagnosis and management are crucial for severe pediatric SLE.
  • Suspicion of underlying monogenic or congenital diseases is vital for SLE presenting in early childhood.
  • Further research into genetic and congenital factors influencing pediatric SLE is needed.

Keywords:
Juvenile SLEMonogenic SLEPaediatric-onset SLESLE-associated genetic diseases

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