Red Flags
- Severe SLE flare with major organ involvement (lupus nephritis, neuropsychiatric SLE, pulmonary haemorrhage, severe haematological) — admit; high-dose immunosuppression; specialist co-management[1]
- Pregnancy with active SLE or anti-Ro/anti-La positive — joint rheumatology and obstetric clinic; aspirin + hydroxychloroquine; fetal echocardiography 16–28 weeks[1]
- Antiphospholipid antibody syndrome with prior thrombosis or recurrent miscarriage — anticoagulation; pre-pregnancy planning[1]
- Severe drug reaction or infection on immunosuppression — pause and investigate; opportunistic infection (PJP, CMV, TB) cover where indicated[1]
First-line treatment
Interventions
- Hydroxychloroquine for all unless contraindicated[1]Backbone of all SLE care — reduces flares, organ damage, mortality, and thrombotic events; safe in pregnancy and breastfeeding; baseline retinal exam and annual screening from year 5
- Treat-to-target with rapid glucocorticoid taper[1]Aim remission or low disease activity (SLEDAI ≤4); minimise prednisolone to ≤5 mg/day long-term; use immunosuppressant or biologic to spare steroid
- Lupus nephritis class III/IV induction and maintenance[1]Induction: methylprednisolone IV pulse 250–500 mg × 3 days, prednisolone 0.5–1 mg/kg/day with rapid taper, plus mycophenolate mofetil 2–3 g/day or low-dose IV cyclophosphamide (Euro-Lupus). Add belimumab or voclosporin as triple therapy. Maintenance: MMF or azathioprine + hydroxychloroquine
- Cardiovascular and bone health[1]Manage hypertension, lipids, smoking; statin per CV risk; calcium, vitamin D, bisphosphonate per fracture risk on glucocorticoid; vaccination updated
First-line drug therapy
| Drug | Class | Adult | Paediatric | Notes |
|---|---|---|---|---|
| Hydroxychloroquine[1] | Antimalarial / immunomodulator | 200–400 mg PO daily (max 5 mg/kg actual body weight) | — | First-line for all SLE; baseline retinal exam and annual screening from year 5; safe in pregnancy and breastfeeding |
| Prednisolone[1] | Systemic corticosteroid | Severe organ disease 0.5–1 mg/kg/day with rapid taper to ≤5 mg/day; preceded by methylprednisolone IV pulse 250–500 mg × 3 days for severe nephritis or NPSLE | — | Bridge to immunosuppressant; minimise duration and dose; PJP prophylaxis if prolonged; bone protection |
| Mycophenolate mofetil[1] | Inosine monophosphate dehydrogenase inhibitor | Induction lupus nephritis 2–3 g/day PO in divided doses × 6 months; maintenance 1–2 g/day | 600 mg/m² BD (max 2 g/day) | First-line induction and maintenance for lupus nephritis class III/IV; teratogenic — strict contraception and switch ≥6 weeks pre-pregnancy |
| Cyclophosphamide[1] | Alkylating agent | Euro-Lupus 500 mg IV every 2 weeks × 6 doses; or NIH high-dose 0.5–1 g/m² IV monthly × 6 months | Per local paediatric rheumatology protocol | Severe lupus nephritis or neuropsychiatric SLE; counsel about gonadal toxicity, bladder cancer; mesna; PJP prophylaxis; preserve fertility (ovarian protection, sperm banking) |
| Belimumab[1] | Anti-BAFF monoclonal antibody | 10 mg/kg IV at 0, 2, 4 weeks then monthly; or 200 mg SC weekly | — | Add-on for moderate-severe extra-renal SLE and lupus nephritis (per BLISS-LN); reduces flares; live-vaccine hold |
| Anifrolumab[1] | Type I interferon receptor antibody | 300 mg IV every 4 weeks | — | Moderate-severe extra-renal SLE refractory to standard therapy; herpes zoster reactivation (vaccinate first); upper respiratory infections |
| Voclosporin (lupus nephritis)[1] | Calcineurin inhibitor | 23.7 mg PO BD × 12 months | — | Add to MMF + steroid in lupus nephritis class III–V; rapid proteinuria reduction; eGFR <45 contraindicated; BP and electrolyte monitoring |
Hydroxychloroquine[1]
Antimalarial / immunomodulator
- Adult
- 200–400 mg PO daily (max 5 mg/kg actual body weight)
- Paediatric
- —
First-line for all SLE; baseline retinal exam and annual screening from year 5; safe in pregnancy and breastfeeding
Prednisolone[1]
Systemic corticosteroid
- Adult
- Severe organ disease 0.5–1 mg/kg/day with rapid taper to ≤5 mg/day; preceded by methylprednisolone IV pulse 250–500 mg × 3 days for severe nephritis or NPSLE
- Paediatric
- —
Bridge to immunosuppressant; minimise duration and dose; PJP prophylaxis if prolonged; bone protection
Mycophenolate mofetil[1]
Inosine monophosphate dehydrogenase inhibitor
- Adult
- Induction lupus nephritis 2–3 g/day PO in divided doses × 6 months; maintenance 1–2 g/day
- Paediatric
- 600 mg/m² BD (max 2 g/day)
First-line induction and maintenance for lupus nephritis class III/IV; teratogenic — strict contraception and switch ≥6 weeks pre-pregnancy
Cyclophosphamide[1]
Alkylating agent
- Adult
- Euro-Lupus 500 mg IV every 2 weeks × 6 doses; or NIH high-dose 0.5–1 g/m² IV monthly × 6 months
- Paediatric
- Per local paediatric rheumatology protocol
Severe lupus nephritis or neuropsychiatric SLE; counsel about gonadal toxicity, bladder cancer; mesna; PJP prophylaxis; preserve fertility (ovarian protection, sperm banking)
Belimumab[1]
Anti-BAFF monoclonal antibody
- Adult
- 10 mg/kg IV at 0, 2, 4 weeks then monthly; or 200 mg SC weekly
- Paediatric
- —
Add-on for moderate-severe extra-renal SLE and lupus nephritis (per BLISS-LN); reduces flares; live-vaccine hold
Anifrolumab[1]
Type I interferon receptor antibody
- Adult
- 300 mg IV every 4 weeks
- Paediatric
- —
Moderate-severe extra-renal SLE refractory to standard therapy; herpes zoster reactivation (vaccinate first); upper respiratory infections
Voclosporin (lupus nephritis)[1]
Calcineurin inhibitor
- Adult
- 23.7 mg PO BD × 12 months
- Paediatric
- —
Add to MMF + steroid in lupus nephritis class III–V; rapid proteinuria reduction; eGFR <45 contraindicated; BP and electrolyte monitoring
Safety-net
- Hydroxychloroquine is the most important medication in SLE — take it daily without missing; the protective effect on flares, kidneys, and survival depends on continuity[1]
- Tell every clinician (dentist, surgeon, A&E) about your SLE diagnosis and immunosuppression — affects infection management, surgery planning, and analgesia[1]
- Plan pregnancy with your rheumatologist — disease must be quiescent ≥6 months and medications switched to pregnancy-safe agents[1]
Referral criteria
- All suspected new SLERheumatology[1]
- New or worsening proteinuria, sediment, or rising creatinine — suspected lupus nephritisNephrology and rheumatology with kidney biopsy[1]
- Neuropsychiatric SLE, pulmonary haemorrhage, severe haematological, or treatment-resistant diseaseTertiary lupus centre[1]
- Pregnancy planning or pregnancy with SLEJoint rheumatology and obstetric clinic[1]
Clinical summary
Diagnosis and treat-to-target management of SLE in adults including hydroxychloroquine backbone, lupus nephritis, and severe-organ-disease pathways.