Systemic
lupus erythematosusDiagnosis
Four of:
- Malar disease
- Photosensitivity
- Renal disorder
- Haematological disorder
- ANA positive
- Discoid rash
- Oral ulcers
- Serositis
- Neurological disorder
- Immunological disorder
eg: arthralgia, mucocutaneous manifestations, fatigue, photosensitive malar rash, rapid hair loss, less synovitis than RA.
Investigations
- ESR
- ANA
- dsDNA (develops later: often initially negative)
- ENA screen
- C3 and C4
- Anticardiolipin ab
- Clotting screen
- Leucopoenia, lymphopoenia common
- Creatinine clearance
- CXR
Monitoring
- FBC WBC/differential
- BP
- dsDNA (quantitative)
- Urine protein
- Creatinine
Management
- Avoid sunlight: sunscreen
- Low dose COC or POP (avoid IUCD because of infection risk)
- NSAID (sulindac, nabumetone)
- Hydroxychloroquine for rash/arthritis/malaise
- Steroids for severe flare (60mg+)
- Azathioprine (steroid sparing: up to 2.5mg/kg), methotrexate, cyclophosphamide + steroids (severe flare)
- Aspirin or anticoagulants if clotting problem
Lupus-like syndromes
- Antiphospholipid syndrome
Secondary to SLE or primary. Thrombosis, livedo reticularis, thrombocytopoenia. Lupus anticoagulant and antiphospholipid antibodies. - Subacute cutaneous LE
Photosensitivity. Ro/La antibodies. - Discoid LE
Discoid lesions, few systemic effects, low antibody titres. - Drug induced lupus
Hydralazine, phenytoin, chlorpromazine, penicillamine, methyl dopa. - Overlap syndromes
Scleroderma-like, rheumatoid-like, myositis-like. - Raynauds + sausage digits.