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Systemic Lupus Erythematosus (SLE)

The British Society for Rheumatology guideline for the management of systemic lupus erythematosus in adults. Published: Oct 2017.

Guidelines

Test Interpretation Notes
Antinuclear antibody (ANA) Screening test for lupus

Highly sensitive (~95%) but not specific		 -ve ANA makes SLE unlikely, +ve in ~5% healthy adults

Poor diagnostic value alone
Anti-dsDNA antibodies Specific marker for SLE (but less sensitive)

Correlate with disease activity, especially lupus nephritis		 +ve anti-dsDNA makes SLE very likely
Anti-Smith (Sm) antibodies Specific marker for SLE (but less sensitive) +ve anti-dsDNA makes SLE very likely
Complement levels (C3, C4) Decreased levels in active disease High negative predictive value for excluding active disease
ESR Elevated in active disease but non-specific  
CRP Usually normal in active disease (or raised modest) Elevated CRP suggests infection or inflammation unrelated to lupus

Note that anti-Ro (SSA), anti-La (SSB), and anti-RNP antibodies can also be +ve in SLE but is less specific as they are also seen in other rheumatological conditions.

 

The combination of +ve ANA (screening test) and +ve anti-dsDNA or anti-Sm antibodies (confirmatory test) makes SLE very likely.

  • All SLE patients should be tested for antiphospholipid syndrome
  • Screen for renal involvement by urinalysis, renal function +/- spot urine albumin:creatinine ratio

The following 2 types of lupus has qutie distinctly different serology from idiopathic SLE (above):
  • Drug-induced lupus
    • Typical serology is +ve ANA and +ve anti-histone antibodies
    • Anti-dsDNA and anti-Sm antibodies are usually -ve
 
  • Cutaneous lupus
    • Typical exam serology: seronegative 
    • ANA is typically -ve or more variable pattern
    • Anti-dsDNA and anti-Sm antibodies are usually -ve
    • Anti-Ro (SSA) and anti-La (SSB) is most likely +ve antibodies
 
​​​​​​​ 

Anti-dsDNA and anti-Sm antibodies, highly specific for idiopathic systemic lupus erythematosus (SLE), are typically absent or low in drug-induced lupus and cutaneous lupus

Patients with active SLE should be reviewed at least every 1-3 months, with the following assessment:
  • Blood pressure
  • Urinalysis and renal function
 
  • Blood tests
    • Anti-dsDNA antibodies
    • Complement levels
    • CRP
    • FBC and LFT

  • Sun protection (advise on sunavoidance and use of high-sun protection factor sunscreen)
  • Smoking cessation
  • Healthy diet
  • Regular exercise

Also important to optimise and treat comorbidity, esp. cardiovascular risk management (SLE increases CVD risk)

  • Hydroxychloroquine - corestone drug recommended for ALL patients with SLE
  • NSAIDs - to be used for symptom control of mild MSK pain

  • Steroids - aim for short courses only with lowest effective dose
 
  • Steroid-sparing immunosuppressive agents
    • Methotrexate
    • Azathioprine
    • Mycophenolate mofetil
    • Calcineurin inhibitors (e.g. ciclosporin, tacrolimus)

  • High-dose steroids (IV pulses)
 
  • Biologics
    • Belimumab
    • Rituximab
 
  • IV immunoglobulin and plasmapheresis 

All SLE patients with suspected renal involvement requires renal biopsy to confirm and classify lupus nephritis.

For class III/IV lupus nephritis:
  • High-dose steroid + mycophenolate / cyclophosphamide

Otherwise: steroids +/- azathioprine should be sufficient

Consider retixuimab / belimumab in refractory or relapsing cases.

References

Author: Adams Lau
Reviewer:
Last edited: 22/07/25