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The bm12 Inducible Model of Systemic Lupus Erythematosus SLE in C57BL/6 Mice
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Systemic Lupus Erythematosus in Children.

Kunal Chandwar1, Amita Aggarwal2

  • 1Department of Clinical Immunology & Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226014, Uttar Pradesh, India.

Indian Journal of Pediatrics
|September 15, 2023
PubMed
Summary

Systemic lupus erythematosus (SLE) is an autoimmune disease affecting multiple organs. Early diagnosis and improved treatments have increased the 10-year survival rate to nearly 90%.

Keywords:
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Area of Science:

  • Immunology
  • Rheumatology
  • Pediatrics

Background:

  • Systemic lupus erythematosus (SLE) is a complex autoimmune disorder affecting various organs, including skin, joints, and kidneys.
  • Characterized by autoantibodies (ANA, anti-dsDNA, anti-RNA-associated proteins), SLE pathogenesis involves immune complex deposition, complement activation, and cellular dysfunction.
  • Pediatric SLE presents with diverse symptoms like fever, cytopenias, rash, and organ-specific manifestations, necessitating prompt evaluation, especially for renal involvement.

Purpose of the Study:

  • To provide a comprehensive overview of Systemic Lupus Erythematosus (SLE) in children.
  • To highlight the diagnostic criteria, pathogenic mechanisms, and clinical manifestations of pediatric SLE.
  • To discuss current treatment strategies and the evolving prognosis of SLE.

Main Methods:

  • Review of existing literature on SLE pathogenesis, clinical presentation, and management in pediatric populations.
  • Analysis of diagnostic markers, including autoantibodies and organ-specific assessments.
  • Examination of therapeutic interventions ranging from corticosteroids to immunosuppressants and emerging targeted therapies.

Main Results:

  • Pediatric SLE diagnosis relies on a constellation of clinical and serological findings, with renal and hematologic involvement being common.
  • Pathological mechanisms include immune complex-mediated damage, inflammatory cytokine release (e.g., interferon-alpha), and neutrophil extracellular trap (NET) formation.
  • Treatment strategies are tailored to disease severity, utilizing agents like hydroxychloroquine, mycophenolate mofetil, azathioprine, cyclophosphamide, and pulse methylprednisolone for severe cases.

Conclusions:

  • The prognosis for pediatric SLE has significantly improved, with 10-year survival rates approaching 90% due to advancements in early diagnosis and treatment.
  • Integrated management, including regular follow-up and comorbidity treatment, is crucial for minimizing long-term organ damage and improving patient outcomes.
  • Ongoing research into targeted therapies holds promise for further reducing morbidity and enhancing the quality of life for children with SLE.