Systemic Lupus Erythematosus (SLE)

A chronic autoimmune disease that can affect almost any organ, causing generalised and organ-specific symptoms. Female >> male; peak incidence during childbearing years (15–45). Clinical course is one of remission and relapse. Younger age of onset and certain ethnic groups (e.g. Asian, Indigenous Australian) carry an increased risk of more severe disease. Atherosclerotic cardiovascular disease is the leading cause of mortality.

Diagnosis

Classification requires 4 of the 11 ACR criteria below — a useful mnemonic is SOAP BRAIN MD (Serositis, Oral ulcers, Arthralgia/arthritis, Photosensitivity, Blood disorders, Renal, ANA, Immunological, Neuropsychiatric, Malar rash, Discoid rash):

Criterion	Definition
Malar rash	Flat or raised malar erythema
Discoid rash	Erythematous raised patches, keratotic scaling, follicular plugging
Photosensitivity	Unusual skin reaction to sunlight
Oral ulcers	Oral/nasopharyngeal ulcers, usually painless
Arthritis	Non-erosive arthritis, ≥2 peripheral joints, tenderness/swelling/effusion
Serositis	Pleurisy or pericarditis
Renal	Proteinuria or cellular casts
Neuropsychiatric	Seizures or psychosis
Haematological	Haemolytic anaemia, leucopenia, lymphopenia or thrombocytopenia
Immunological	Anti-dsDNA, anti-Smith, or antiphospholipid antibodies
ANA positive	In the absence of drugs known to cause drug-induced lupus

Management

Overall care model (BSR Lupus guideline, 2026)

• Suspected SLE in primary care warrants an ANA test, with a 3-week referral wait if clinical suspicion is strong.
• Diagnosis is confirmed in secondary/tertiary care (dsDNA, ENAs, antiphospholipid antibodies, lupus anticoagulant, C3/C4) by an experienced clinician, ideally in a cohorted lupus clinic with specialist nurse, pharmacist and allied-health input.
• Treat-to-target: aim for (cumulative) low lupus disease activity state (LLDAS) or remission. Active disease is reassessed 1–3-monthly; inactive disease up to 6-monthly (children) or 12-monthly (adults).
• Annual review covers damage accrual, bone health, cardiovascular risk, vaccinations and hydroxychloroquine monitoring.
• Cutaneous disease: non-facial topical glucocorticoid/calcineurin inhibitor, systemic immunosuppression, with anifrolumab now the preferred first-line biologic for skin disease not controlled by these measures.
• Escalation by severity (BILAG/SLEDAI-graded): short-term glucocorticoids + hydroxychloroquine as bridging/first therapy for flares → methotrexate/mycophenolate/azathioprine/ciclosporin/tacrolimus for non-response or steroid-sparing → biologic therapy (belimumab, rituximab, anifrolumab) or clinical trial for resistant/severe disease.
• Life- or organ-threatening disease (including neuropsychiatric lupus, severe autoimmune thrombocytopenia): high-dose glucocorticoids + cyclophosphamide and/or rituximab, ± IVIg/plasma exchange.
• Lupus nephritis is managed per its own dedicated guideline pathway for induction and maintenance (registry: bsr-lupus-2026; verified 2026-07-23).

Organ-directed symptomatic management

• Arthralgia/myalgia/arthritis: analgesia; NSAIDs/hydroxychloroquine for persistent symptoms.
• Raynaud/digital ischaemia: amlodipine, nifedipine MR or felodipine MR; specialist referral if severe.
• Mucosal/cutaneous: aphthous ulcers — topical hydrocortisone 1%, benzydamine 1%, or lidocaine 2% solution. Discoid lesions — sun protection, topical steroid (betamethasone dipropionate 0.05%, mometasone furoate 0.1%), oral steroid if severe.
• Serositis: NSAIDs for mild disease; IV methylprednisolone for acute severe disease; prednisolone + a DMARD (hydroxychloroquine, methotrexate, azathioprine) for persistent/recurrent disease.
• Haematological (immune cytopenia): no treatment for minor cytopenia; immunomodulatory therapy (prednisolone, azathioprine, cyclophosphamide, mycophenolate) for moderate disease; high-dose steroid ± IVIg/rituximab/ plasma exchange for severe disease.
• Vasculitis: specialist-managed — prednisolone, azathioprine, other immunomodulators.
• Antiphospholipid syndrome: suspect in unprovoked venous thrombosis, stroke <50y, or recurrent first-trimester miscarriage/unexplained 2nd–3rd trimester loss. Specialist referral; low-dose aspirin, heparin, warfarin.
• Renal: mild isolated haematuria usually needs no treatment; heavier haematuria/proteinuria needs specialist referral, renal biopsy, and high-dose prednisolone + mycophenolate/cyclophosphamide/other DMARD. Aggressive BP and lipid control improves renal outcomes.
• Hepatic: refer for biopsy if autoimmune liver disease or PBC suspected.
• Neurological: headache/migraine most common; small-vessel cerebral vasculopathy can cause cognitive decline, depression, thought disorder; seizures; cerebral vasculitis and transverse myelitis are rare.
• Reproductive health: COCP can trigger a flare — monitor ESR/CRP/dsDNA at 3 months or consider an alternative. Pregnancy can trigger a flare — check disease-activity markers in the 6 months preceding conception; continue hydroxychloroquine through pregnancy under specialist supervision. Postmenopausal HRT is not usually associated with a significant increase in flares.
• Children: managed similarly to adults, via paediatric rheumatology.