To update the EULAR recommendations for the management of systemic lupus erythematosus
(SLE) based on emerging new evidence.An international Task Force formed the questions
for the systematic literature reviews (January 2018-December 2022), followed by formulation
and finalisation of the statements after a series of meetings. A predefined voting
process was applied to each overarching principle and recommendation. Levels of evidence
and strengths of recommendation were assigned, and participants finally provided their
level of agreement with each item.The Task Force agreed on 5 overarching principles
and 13 recommendations, concerning the use of hydroxychloroquine (HCQ), glucocorticoids
(GC), immunosuppressive drugs (ISDs) (including methotrexate, mycophenolate, azathioprine,
cyclophosphamide (CYC)), calcineurin inhibitors (CNIs, cyclosporine, tacrolimus, voclosporin)
and biologics (belimumab, anifrolumab, rituximab). Advice is also provided on treatment
strategies and targets of therapy, assessment of response, combination and sequential
therapies, and tapering of therapy. HCQ is recommended for all patients with lupus
at a target dose 5 mg/kg real body weight/day, considering the individual's risk for
flares and retinal toxicity. GC are used as 'bridging therapy' during periods of disease
activity; for maintenance treatment, they should be minimised to equal or less than
5 mg/day (prednisone equivalent) and, when possible, withdrawn. Prompt initiation
of ISDs (methotrexate, azathioprine, mycophenolate) and/or biological agents (anifrolumab,
belimumab) should be considered to control the disease and facilitate GC tapering/discontinuation.
CYC and rituximab should be considered in organ-threatening and refractory disease,
respectively. For active lupus nephritis, GC, mycophenolate or low-dose intravenous
CYC are recommended as anchor drugs, and add-on therapy with belimumab or CNIs (voclosporin
or tacrolimus) should be considered. Updated specific recommendations are also provided
for cutaneous, neuropsychiatric and haematological disease, SLE-associated antiphospholipid
syndrome, kidney protection, as well as preventative measures for infections, osteoporosis,
cardiovascular disease.The updated recommendations provide consensus guidance on the
management of SLE, combining evidence and expert opinion.
