What is the appropriate management plan for a pediatric patient with Systemic Lupus Erythematosus (SLE) and nephritis?

Management of Pediatric Systemic Lupus Erythematosus with Nephritis

The appropriate management for a pediatric patient with SLE and nephritis includes hydroxychloroquine as cornerstone therapy, high-dose glucocorticoids (initially IV methylprednisolone followed by oral prednisone), and cyclophosphamide for induction therapy, followed by maintenance immunosuppression. 1, 2

Initial Assessment and Diagnosis

• Pediatric SLE typically presents with more severe disease compared to adult-onset SLE, with higher risk for renal involvement (OR 1.5-2.4) 3, 1
• Renal biopsy is essential for any child with SLE showing signs of renal involvement (proteinuria, hematuria, abnormal renal function) to guide treatment decisions 2
• Regular monitoring is crucial as kidney involvement can remain asymptomatic for significant periods 3
• Higher index of suspicion should be maintained for patients of Asian, African/Caribbean, and Hispanic descent 3

Induction Therapy for Lupus Nephritis

• Initial treatment should include three consecutive pulses of intravenous methylprednisolone, followed by oral prednisone, with goal of reducing to ≤10 mg/day by 4-6 months 2
• Cyclophosphamide is recommended for induction therapy in proliferative lupus nephritis (Class III-IV), administered monthly for 6 months 2, 4
• Mycophenolate mofetil/mycophenolic acid is an alternative to cyclophosphamide for induction therapy, particularly in certain populations with better response rates 2
• Hydroxychloroquine should be prescribed for all pediatric SLE patients regardless of disease severity at a dose of 6.5 mg/kg/day or 400 mg/day, whichever is lower 1, 2

Maintenance Therapy

• After successful induction therapy, maintenance therapy should include either mycophenolate mofetil/mycophenolic acid or azathioprine 2
• Maintenance therapy should be continued for at least 3 years to prevent flares 2
• Hydroxychloroquine should be continued indefinitely as it reduces flare rates and improves long-term outcomes 1, 2

Supportive Care

• Blood pressure control is essential, with medications such as enalapril and nifedipine commonly used 3
• Low-salt diet is recommended to help manage hypertension and edema 3
• Calcium and vitamin D supplementation should be provided to prevent glucocorticoid-induced osteoporosis 2

Monitoring

• Regular monitoring should include body weight, blood pressure, serum creatinine, estimated GFR, serum albumin, proteinuria, urinary sediment, serum C3/C4, and anti-dsDNA antibody levels 2
• Visits should be scheduled every 2-4 weeks for the first 2-4 months after diagnosis or flare, and then according to response to treatment 3
• Complete renal response should be achieved preferably by 6 months but no later than 12 months after starting treatment 2
• Repeat CBC, urinalysis, and creatinine are recommended 2 weeks after first dose of cyclophosphamide to monitor for toxicity 5

Multidisciplinary Care

• Pediatric SLE patients should be co-managed by pediatric nephrologists and rheumatologists with expertise in lupus 1, 2
• Additional support from clinical psychologists, psychiatrists, or social workers is often beneficial for addressing psychosocial aspects of chronic disease 1

Prognosis and Long-term Considerations

• Long-term data shows survival rates without advanced CKD, kidney failure, or death of 94.2%, 92.7%, and 83.2% at 5,10, and 20 years, respectively 1
• Despite improvements in treatment, 10-30% of patients with lupus nephritis will progress to end-stage renal disease within 15 years of diagnosis 3
• Children with SLE tend to receive more intensive immunosuppressive treatment and accrue more damage, often related to glucocorticoid toxicity, compared to adults 3

Special Considerations

• Non-live vaccines should be administered according to standard schedules for all pediatric SLE patients 2
• For adolescent patients approaching reproductive age, medication adjustments may be needed without reducing treatment intensity 2
• Growth concerns should guide glucocorticoid dosing strategies to minimize exposure 2

By following this comprehensive management approach, outcomes for pediatric patients with SLE and nephritis can be optimized, reducing morbidity and mortality while preserving kidney function.