SOAP Note: Management of Pediatric Systemic Lupus Erythematosus with Nephritis

Subjective

Patient presents with Systemic Lupus Erythematosus (SLE) and nephritis.

Pediatric SLE with lupus nephritis requiring comprehensive immunosuppressive therapy based on nephritis classification.

Renal biopsy is essential to guide treatment decisions for any pediatric patient with SLE showing signs of renal involvement (proteinuria ≥0.5 g/24h, glomerular hematuria, cellular casts) 1, 2
Complete ISN/RPS 2003 classification with assessment of active and chronic glomerular and tubulointerstitial changes 1
Baseline laboratory evaluation: serum creatinine, eGFR, serum albumin, proteinuria, urinary sediment, serum C3/C4, anti-dsDNA antibody levels, complete blood count 1
Antiphospholipid antibodies and lipid profile should be measured at baseline 1

For Class III-IV lupus nephritis: Initiate high-dose glucocorticoids plus either mycophenolate mofetil (MMF) or cyclophosphamide (CYC) 2
- Begin with three consecutive pulses of IV methylprednisolone followed by oral prednisone 2
- Target oral prednisone reduction to ≤10 mg/day by 4-6 months 2
- MMF may be preferred over CYC in certain populations due to better response rates 2
For Class V lupus nephritis with nephrotic-range proteinuria:
- Mycophenolic acid (MPA) or MMF (target dose 2-3 g/day) in combination with glucocorticoids 3
For Class I-II lupus nephritis:
- Class I: Treatment guided by extrarenal manifestations 3
- Class II with proteinuria <1 g/day: No specific immunosuppressive therapy needed beyond treatment for extrarenal manifestations 3

Hydroxychloroquine (6.5 mg/kg/day or 400 mg/day, whichever is lower) for ALL pediatric SLE patients regardless of disease severity 2, 4
ACE inhibitors or angiotensin receptor blockers for patients with proteinuria or hypertension 1
Statins for persistent dyslipidemia (target LDL-cholesterol 2.58 mmol/L) 1
Consider anticoagulant treatment in nephrotic syndrome with serum albumin <20 g/L, especially if persistent or with antiphospholipid antibodies 1

After achieving response, continue maintenance immunosuppression with MMF or azathioprine for at least 3 years 1, 2
If initial treatment was MMF/MPA, continue with MMF/MPA for maintenance 3
Hydroxychloroquine should be continued indefinitely 3, 4

Schedule visits every 2-4 weeks for first 2-4 months after diagnosis or flare, then adjust according to response 1
Regular monitoring at each visit: body weight, blood pressure, serum creatinine, eGFR, serum albumin, proteinuria, urinary sediment, serum C3/C4, anti-dsDNA antibody levels, CBC 1
Treatment targets:
- Reduction in proteinuria of at least 25% by 3 months 3
- At least 50% reduction by 6 months 3
- Target UPCR <500-700 mg/g by 12 months 3
Lifelong monitoring for renal and extrarenal disease activity at least every 3-6 months 1
Consider repeat renal biopsy for worsening or refractory disease, at relapse, or to guide immunosuppression withdrawal 1, 5

For patients who fail treatment with MMF or CYC:
- Switch from MMF to CYC, or CYC to MMF 1
- Consider rituximab for refractory cases 1, 6
- Belimumab is FDA-approved for children >5 years with SLE 7, 6

Growth concerns should guide glucocorticoid dosing strategies to minimize exposure 2
For adolescent females of reproductive potential, counsel on effective contraception during treatment 7
For pregnancy planning: medications should be adjusted without reducing treatment intensity 2
- Acceptable medications during pregnancy: hydroxychloroquine, low-dose prednisone, azathioprine 1
- MPA/MMF or CYC should not be used in pregnancy 1
- Acetylsalicylic acid recommended to reduce pre-eclampsia risk 1

Counsel on importance of medication adherence, especially hydroxychloroquine 4
Discuss need for regular ophthalmological examinations while on hydroxychloroquine 4
Non-live vaccines should be administered according to standard schedules 2
Regular follow-up is essential for early detection and management of disease flares 1