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Table 1 Treatment recommendation for juvenile SLE

From: CAR-T cell therapy for juvenile-onset autoimmune diseases: a promising future?

Treat-to-target recommendations for juvenile SLE (SHARE/PreS/EULAR)

1. Hydroxychloroquine

◦ 5 mg/kg/day

2. Glucocorticoid

• at the lowest dosage

◦ initially 1–2 mg/kg/day or in case of severe disease: intravenous pulse therapy 30 mg/kg/dose three consecutive days

◦ 10%–20% at 1-week or 2-week interval based on clinical improvement

3. Methotrexate

• skin and joint involvement

4. Azathioprine/mycophenolate mofetil

• Class II, III, IV, V lupus nephritis

5. Tacrolimus, ciclosporine

• Pure class V lupus nephritis in selected case (nephrotoxicity)

Then, in addition of DMARDs

6. Belimumab

• Class III, IV lupus nephritis

• skin and joint involvement

â—¦ 10 mg/kg/month

7. Rituximab

• Refractory lupus nephritis

◦ 4 × 375 mg/m2/week

◦ 2 × 750 mg/m2/14 days

8. Cyclophosphamide

• class III/IV or refractory lupus nephritis

• Severe neurolupus

◦ 6 × 500 mg or 500 mg/1,73 m2 every 2 weeks for three months EURO-LUPUS

◦ 0,75–1 g/m2 monthly for 6–9 months NIH lupus regimen

  1. JAK inhibitors could be a valuable option in monogenic lupus with specific mutations
  2. SHARE Single Hub and Access point for pediatric Rheumatology in Europe, PreS Paediatric Rheumatology European Society, EULAR European Alliance of Associations for Rheumatology, NIH: National Institutes of Health