Based on emerging new evidence and expert consensus, a task force put together by the European League Against Rheumatism (EULAR) released updated recommendations for the management of systemic lupus erythematosus (SLE). This report was published in Annals of the Rheumatic Diseases.

The investigators performed a systematic review of studies focused on treatment strategies for the management of SLE published between January 2007 and December 2017. Recommendations for 4 broad categories were developed following the modified Delphi method, in which the task force devised related questions, elicited expert opinions, and reached consensus.

Recommendations for Goals of Treatment

Goals for the treatment of SLE should aim to target remission of disease symptoms, prevention of damage from disease activity, minimization of drug side-effects, and improvement of quality of life measures. Close monitoring of disease activity and patient adherence to treatment are recommended to help reduce the risk for flare.

Recommendations for Treatment of Systemic Lupus Erythematosus

Hydroxychloroquine

With multiple beneficial effects, hydroxychloroquine is recommended for all patients with SLE. However, retinal toxicity is a risk in long-term use of hydroxychloroquine, especially for patients with increased dosage, chronic kidney disease, or preexisting retinal or macular disease. For this reason, EULAR recommends a dose not exceeding 5 mg/kg real body weight.

Glucocorticoids

During chronic maintenance, glucocorticoids can provide rapid symptom relief, but long-term glucocorticoid use may be damaging to organs. In the medium term, EULAR recommends minimizing dose to less than 7.5 mg/d (prednisone equivalent) and, when possible, should be discontinued.

Immunosuppressive Drugs

Appropriate initiation of immunosuppressive drugs is recommended to facilitate rapid glucocorticoid tapering and possibly prevent disease flares. Patient age, childbearing potential, prevailing disease manifestations, safety concerns, and cost are factors that should inform the choice of immunosuppressive agent (including methotrexate, azathioprine, and mycophenolate mofetil). Methotrexate and azathioprine should be considered in patients with poor symptom control after receiving glucocorticoids and hydroxychloroquine or when hydroxychloroquine alone is unlikely to be sufficient.

Biologic Agents

Belimumab should be considered as an add-on therapy for patients with extrarenal disease who experience persistent disease activity or frequent flares. Currently, rituximab is only recommended in patients with severe refractory disease and it should only be considered in patients when more than 1 immunosuppressant agent has failed.

Recommendations for Specific Manifestations

Skin Disease

First-line treatments of skin disease recommended for patients with SLE include topical agents (glucocorticoids, calcineurin inhibitors), antimalarials (hydroxychloroquine, quinacrine), and systemic glucocorticoids. In patients who do not respond to first-line treatments or who require high-dose glucocorticoids, methotrexate, retinoids, dapsone, or mycophenolate may be considered as add-on therapy.

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Neuropsychiatric Disease

SLE-related neuropsychiatric manifestations, as opposed to non-SLE-related neuropsychiatric disorders, should be evaluated using neuroimaging techniques, testing cerebrospinal fluid, and assessing risk factors (onset disease manifestations, patient age, non-neurological SLE activity, presence of antiphospholipid antibodies) to exclude confounding. Recommended treatments include glucocorticoids and immunosuppressive agents if the manifestations reflect an inflammatory process or anticoagulants for antiphospholipid-related manifestations.

Hematological Disease

Treatment of thrombocytopenia in SLE requires acute management of disease activity with high-dose glucocorticoid therapy (including intravenous methylprednisolone) or intravenous immunoglobulin G treatments. Beyond the acute stage, immunosuppressive-sparing or glucocorticoid-sparing agents (such as mycophenolate, azathioprine, and cyclosporine) may be used for maintenance therapy. Rituximab or cyclophosphamide are only recommended in severe refractory cases.

Renal Disease

Early recognition and diagnosis of renal disease in SLE is essential for managing outcomes. Clinicians should perform a biopsy if renal involvement is suspected. Recommended induction treatments for renal disease include mycophenolate or low-dose intravenous cyclophosphamide due to their optimal efficacy/toxicity ratio. Higher doses may be considered for patients at high risk for renal failure. Mycophenolate and azathioprine should be used in ongoing maintenance therapy.

Recommendations for Comorbidities

Antiphospholipid Antibodies and Antiphospholipid Syndrome

EULAR recommends that all patients with SLE be screened at diagnosis for the presence of antiphospholipid antibodies. Patients with SLE and high-risk antiphospholipid profiles (including presence of atherosclerotic and/or thrombophilic factors) may be treated with primary prophylaxis with antiplatelet agents. Secondary prevention should apply the same therapeutic approach for primary antiphospholipid syndrome.

Infections

Patients with SLE should be assessed for risk factors related to infection, including advanced age, diabetes mellitus, renal involvement, use of immunosuppressive agents or biologics, or glucocorticoid use. General preventative measures are recommended, such as immunizations, as is the early recognition and treatment of infection.

Cardiovascular Disease

Clinicians recommend that patients with SLE undergo regular assessment for risk factors related to cardiovascular disease, including ongoing disease activity, increased disease duration, medium-to-high levels of antiphospholipid antibodies, renal involvement, and chronic glucocorticoid use. Depending on individual cardiovascular risk profiles, these patients may benefit from general preventative strategies, such as low-dose aspirin or lipid-lowering medications.

Multiple authors declare affiliations with the pharmaceutical industry. Please refer to reference for a complete list of authors’ disclosures.

Reference

Fanouriakis A, Kostopoulou M, Alunno A, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus [published online March 29, 2019]. Ann Rheum Dis. doi:10.1136/annrhumdis-2019-215089