- First UK-based guidelines for the diagnosis and management of systemic lupus erythematosus (SLE).
Why this matters
- SLE affects nearly 1 in 1000 people in the United Kingdom.
- Antiphospholipid antibodies (aPLs) should be tested in all patients at baseline.
- Confirmatory tests for antiphospholipid syndrome (APS) are positive lupus anticoagulants, anti-cardiolipin antibody and/or anti-beta-2 glycoprotein-1 on 2 occasions ≥12 wk apart.
- Patients with active disease should be reviewed at least every 1-3 mo.
- Patients with low disease activity or in remission can be reviewed every 6-12 mo.
- Presence of aPLs in previously negative patients warrants re-evaluation prior to pregnancy or surgery, or in the presence of new severe manifestation or vascular event.
- Check anti-Ro and anti-La antibodies prior to pregnancy.
- Treatment of mild disease includes hydroxychloroquine (Plaquenil, Quinoric) and methotrexate (Ebetrex, Maxtrex, Metoject), and short courses of NSAIDs for symptom control.
- Prednisolone ≤7.5 mg/d may be required for maintenance.
- Prednisolone ≤0.5 mg/kg/d or intramuscular/intravenous methylprednisolone for moderate disease.
- Consider methotrexate, azathioprine (Imuran), mycophenolate mofetil (Cellcept), ciclosporin (Neoral, Sandimmun Deximune), and other calcineurin inhibitors for arthritis, cutaneous disease, serositis, vasculitis or cytopenias if hydroxychloroquine is insufficient.
- For refractory cases, belimumab (Benlysta) or rituximab (Truxima, MabThera) may be considered.
- Patients with severe SLE should receive immunosuppression and/or anticoagulation.
- Mycophenolate mofetil or ciclosporin for lupus nephritis and refractory, severe non-renal disease.
- Consider belimumab where immunosuppressives fail or are poorly tolerated.
- Consider intravenous immunoglobulin and plasmapheresis for refractory cytopenias, thrombotic thrombocytopenic purpura, rapidly deteriorating acute confusional state and catastrophic APS.