Treatment of Pediatric Systemic Lupus Erythematosus

Introduction to Treatment

The American Academy of Pediatrics recommends that all pediatric patients with systemic lupus erythematosus (SLE) receive hydroxychloroquine (≤5 mg/kg of actual body weight) as fundamental therapy, unless contraindicated, as it reduces disease activity, prevents flare-ups, and improves survival 1, 2, 3

The European League Against Rheumatism recommends hydroxychloroquine for almost all patients with pediatric SLE and should be continued long-term 1, 2, 3
Ophthalmologic screening is necessary at baseline, after 5 years, and then annually to monitor retinal toxicity 2
Photoprotection with sunscreens is recommended to prevent cutaneous flare-ups 2
Calcium and vitamin D supplementation is recommended for all patients on long-term corticosteroid therapy 2

The American College of Rheumatology recommends that glucocorticoids be used with the primary goal of minimizing chronic exposure to <7.5 mg/day of prednisone equivalent, using immunosuppressive agents to facilitate reduction 1, 2

Methylprednisolone intravenous bolus (250-1000 mg/day for 1-3 days) for severe or life-threatening manifestations 2, 4
Followed by moderate to high-dose oral prednisone (0.5-1 mg/kg/day) 4
Early initiation of immunomodulatory agents accelerates glucocorticoid reduction/discontinuation 2

Limiting glucocorticoid exposure is particularly important in children to prevent growth delay 1
Growth concerns may favor steroid-sparing strategies 1

Methotrexate is recommended as the first choice for cutaneous and articular manifestations 2
Topical corticosteroids and topical calcineurin inhibitors for cutaneous manifestations 2

Mycophenolate mofetil (MMF) or low-dose intravenous cyclophosphamide are the preferred immunosuppressive choices 5, 6, 1, 2
Recent data show that 36% of pediatric patients receive MMF and 34% receive cyclophosphamide as induction therapy 1
Low-dose cyclophosphamide is preferred over high-dose due to comparable efficacy and lower gonadotoxicity 2
Fertility concerns, especially as patients approach adolescence, may favor limiting cyclophosphamide exposure 1

Mycophenolate mofetil is the most commonly used maintenance drug (55% of pediatric patients) 1
Azathioprine is an alternative, particularly suitable for girls contemplating pregnancy 2
Maintenance therapy should be continued for at least 12 months after achieving clinical remission 7

Belimumab (anti-BAFF antibody) is approved by the FDA for the treatment of pediatric SLE in children >5 years and can be used as add-on therapy for active extrarenal SLE and lupus nephritis 2

Angiotensin-converting enzyme inhibitors (ACE inhibitors) or angiotensin receptor blockers (ARBs) are recommended as preferred treatment in all non-pregnant patients with significant proteinuria or hypertension 6
Strict control of proteinuria and blood pressure is required in pediatric SLE 6
Target blood pressure <130/80 mmHg 6
Low-dose aspirin may be considered for patients with antiphospholipid antibodies, those receiving corticosteroids, or those with cardiovascular risk factors 2

Due to the high risk of pneumococcal infection, immunization is recommended for pediatric SLE patients 3
Ideally, vaccinations should be administered before starting immunosuppressive therapy 3
Only use non-live vaccines 6

Pediatric SLE patients should be co-managed by pediatric nephrologists and rheumatologists with experience in lupus 1, 3
Additional support from clinical psychologists, psychiatrists, or social workers is often beneficial to address psychosocial issues related to school and peer socialization 1, 3

Regular monitoring of disease activity using validated indices is crucial 2
Monitor anti-dsDNA, C3, C4, complete blood count, creatinine, proteinuria, and urine sediment 6, 2
Long-term data show survival rates without advanced chronic kidney disease, renal failure, or death of 94.2%, 92.7%, and 83.2% at 5, 10, and 20 years, respectively 1, 3