Systemic lupus erythematosus

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.

References

1.EULAR Recommendations for the Management of Systemic Lupus Erythematosus (2023 update); KDIGO 2024 Lupus Nephritis Guideline (2023)