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2019 update of the EULAR recommendations for the management of SLE

EULAR has published an update to a set of recommendations for the management of systemic lupus erythematosus (SLE). 
Treatment in SLE aims at remission or low disease activity and prevention of flares. The updated recommendations provide physicians and patients with updated consensus guidance on the management of SLE.
A systematic literature review was performed, followed by the modified Delphi method, which was used to form questions, elicit expert opinions and reach consensus. The methods / methodological approach included pharmacologic treatment of SLE; management of specific manifestations; monitoring SLE and treatment targets; comorbidities and adjunct therapy.
Based on the evidence and expert opinion from an international task force, overarching principles and recommendations were formulated. Four overarching principles and 13 recommendations were agreed upon. The four overarching principles are:
  • SLE is a multisystem disease – occasionally limited to one or few organs – diagnosed on clinical grounds in the presence of characteristic serologic abnormalities.
  • SLE care is multidisciplinary, based on a shared patient-physician decision, and should consider individual, medical and societal costs.
  • Treatment of organ-/life-threatening SLE includes an initial period of high-intensity immunosuppressive therapy to control disease activity, followed by a longer period of less intensive therapy to consolidate response and prevent relapses.
  • Treatment goals include long-term patient survival, prevention of organ damage and optimisation of health-related quality of life.
The recommendations are categorised into four parts:
  1. Goals of treatment
  2. Treatment of SLE (general)
  3. Specific manifestations
  4. Comorbidities.
The summary of recommendations in lay format are:
  1. The goal of treatment in lupus is to control activity of the disease and prevent flares
  2. All patients with SLE should receive hydroxychloroquine, with regular monitoring for eye toxicity
  3. Glucocorticoids (GC, cortisone) can help to control symptoms when the disease is active. In the long-term its dose must not exceed 7.5 mg/day of prednisone
  4. Immunosuppressive drugs such as methotrexate, azathioprine and mycophenolate can be used to better control the disease and allow using less glucocorticoids
  5. When lupus cannot be controlled with the conventional drugs, biologic drugs like belimumab or rituximab can be used
  6. Skin disease in lupus is initially treated with creams/ointments or hydroxychloroquine, with or without oral glucocorticoids. When these do not control the disease, immunosuppressives or biologics can be used
  7. Symptoms involving the brain and nervous system are not always due to lupus. When caused by lupus, immunosuppressive drugs or aspirin/anticoagulants are used
  8. Severe drops in platelets or low blood counts from SLE are treated with GC and immunosuppressive drugs; in case of relapses, rituximab should be considered
  9. Biospy of the kidney is essential to diagnose kidney involvement in lupus.Immunosuppressive drugs of first-choice are mycophenolate mofetil and cyclophosphamide
  10. A second kidney biopsy may be considered in cases of incomplete response after one year of treatment
  11. Patients with lupus should be tested for antiphospholipid antibodies, because the latter are associated with blood clots (thrombosis), pregnancy loses s and other complications such as strokes.
  12. Patients with SLE have an increased risk for infections and should be vaccinated against influenza and pneumococcus, as well as human papilloma virus (adolescents)
  13. Patients with SLE may suffer more from heart attacks and strokes. To decrease the risk for these complications, quitting smoking, and control of high blood pressure, dyslipidaemia, diabetes are essential.
The level of evidence, grade of recommendation and level of agreement were allocated to each statement.