2. Secondary nephrotic syndrome refers to a condition
where nephrotic syndrome occurs secondary to an
underlying disease process.one notable cause is
systemic lupus erythematosus (SLE), an autoimmune
disease that can affect various organs, including the
kidneys.
INTRODUCTION
3. Lupus Erythematosus
Lupus erythematosus (LE) is a chronic autoimmune disease
characterized by the presence of autoantibodies and immune complex
deposition, leading to inflammation and tissue damage.- It can affect
multiple organs, including the skin, joints, kidneys, and central nervous
system.- In pediatric cases, lupus nephritis (LN) is a significant
manifestation, leading to secondary nephrotic syndrome. LE
encompasses various subtypes, including systemic lupus erythematosus
(SLE), which is the most common and severe form. SLE can present with
diverse clinical manifestations, including mucocutaneous,
musculoskeletal, renal, hematological, and neuropsychiatric
involvement.- Renal involvement, including lupus nephritis, is a common
and potentially serious complication of pediatric SLE, contributing to
secondary nephrotic syndrome.
4. Pathogenesis of Lupus Erythematosus
The pathogenesis of LE involves immune
dysregulation, genetic predisposition,
environmental triggers, and hormonal
influences.- Autoantibodies targeting nuclear
antigens, such as anti-double-stranded DNA
(anti-dsDNA) antibodies, and immune
complex formation contribute to tissue
damage and inflammation in various organs,
including the kidneys.
5. Renal Manifestations
Renal involvement in pediatric SLE can manifest as
lupus nephritis, which is characterized by immune
complex-mediated glomerulonephritis.- Clinical
features of lupus nephritis include proteinuria,
hematuria, hypertension, and impaired renal function.-
Secondary nephrotic syndrome may develop in
some patients with lupus nephritis, presenting with
significant proteinuria, hypoalbuminemia, edema,
and hyperlipidemia.
6. Diagnostic Evaluation
-Diagnosis of lupus erythematosus in pediatric
patients involves a combination of clinical,
laboratory, and serological findings.- Laboratory
tests include assessment of autoantibodies (e.g.,
anti-dsDNA, anti-Smith antibodies), complement
levels, and inflammatory markers.- Renal
involvement is confirmed through urinalysis, renal
function tests, and kidney imaging studies.
7. Treatment
• Treatment aims to achieve disease remission, alleviate
symptoms, prevent disease flares, and minimize long-
term complications.
• Immunosuppressive therapy, including
corticosteroids, disease-modifying antirheumatic
drugs (DMARDs), and biologic agents, is utilized to
suppress autoimmunity and inflammation.
8. Corticosteroid Therapy
Corticosteroids, such as prednisone, are commonly
used as first-line therapy for inducing remission and
controlling disease activity in pediatric SLE.
- Initial doses may range from 0.5 to 2 mg/kg/day, with
adjustments made based on disease activity and
response.- Once disease activity is controlled, the
dosage is typically tapered gradually to the lowest
effective maintenance dose to minimize long-term side
effects.
9. Disease-Modifying Antirheumatic Drugs
(DMARDs)
- Hydroxychloroquine: Often used as a first-line
DMARD in SLE. - The typical dosage is based on
weight, with a maximum daily dose not exceeding 6.5
6.5 mg/kg/day.
- Methotrexate: Another DMARD option for pediatric
SLE, especially for patients with more severe disease.
- Dosage varies based on weight and disease severity,
typically starting at 10 to 15 mg/m^2 once weekly,
with gradual dose escalation as tolerated.
10. Immunosuppressive Agents
- Mycophenolate mofetil (MMF): Commonly used in
pediatric lupus nephritis and refractory SLE. - The
dosage is weight-based, with typical starting doses of
600 to 1,000 mg/m^2 twice daily, adjusted based on
response and tolerability.
- Cyclophosphamide: Reserved for severe lupus
nephritis or refractory disease. - Dosage and
administration vary based on the protocol used, which
11. Conclusion
- Lupus erythematosus is a complex autoimmune disease
that can affect multiple organ systems, including the
kidneys.
- Renal involvement, including lupus nephritis, contributes to
secondary nephrotic syndrome and represents a significant
cause of morbidity and mortality in lupus erythematosus.
- Early recognition, prompt diagnosis, and aggressive
treatment are essential for achieving disease remission,
preserving organ function, and optimizing long-term
outcomes in children with lupus erythematosus.
