Overarching Principles

Summary of Recommendations

Document Overview

Design and created by Guideline Central in participation with the European League Against Rheumatism (EULAR).

Management of Systemic Lupus Erythematosus

European League Against Rheumatism (EULAR)

Publication Date: Jul 22, 2024

Page Last Updated: May 6, 2026

Summary of Recommendations

1. Hydroxychloroquine is recommended for all patients (1b/A), unless contraindicated, at a target dose of 5 mg/kg real body weight/day (2b/B) but individualised based on risk for flare (2b/B) and retinal toxicity.
2. Glucocorticoids, if needed, are dosed based on the type and severity of organ involvement (2b/C), and should be reduced to maintenance dose of ≤5 mg/day (prednisone equivalent) (2a/B) and, when possible, withdrawn; in patients with moderate-to-severe disease, pulses of intravenous methylprednisolone (125–1000 mg/day, for 1–3 days) (3b/C) can be considered.
3. In patients not responding to hydroxychloroquine (alone or in combination with glucocorticoids) or patients unable to reduce glucocorticoids below doses acceptable for chronic use, addition of immunomodulating/immunosuppressive agents (eg, methotrexate (1b/B), azathioprine (2b/C) or mycophenolate (2a/B)) and/or biological agents (eg, belimumab (1a/A) or anifrolumab (1a/A)) should be considered.
4. In patients with organ-threatening or life-threatening disease, intravenous cyclophosphamide (2b/C) should be considered; in refractory cases, rituximab (2b/C) may be considered.
5. Treatment of active skin disease should include topical agents (glucocorticoids, calcineurin inhibitors) (2b/B), antimalarials (hydroxychloroquine, chloroquine) (1a/A), and/or systemic glucocorticoids (4/C) as needed, with methotrexate (1b/B), mycophenolate (4/C), anifrolumab (1a/A), or belimumab (1a/B) considered as second-line therapy.
6. In active neuropsychiatric disease attributed to SLE, glucocorticoids and immunosuppressive agents for inflammatory manifestations (1b/A) and antiplatelet agents/anticoagulants for atherothrombotic/aPL-related manifestations (2b/C) should be considered.
7. For acute treatment of severe autoimmune thrombocytopenia, high-dose glucocorticoids (including pulses of intravenous methylprednisolone) (4/C), with or without intravenous immunoglobulin G (4/C), and/or rituximab (2b/B), and/or high-dose intravenous cyclophosphamide (4/C), followed by maintenance therapy with rituximab (2b/B), azathioprine (2b/C), mycophenolate (2b/C), or cyclosporine (4/C) should be considered.
8. Patients with active proliferative lupus nephritis should receive low-dose (EuroLupus) intravenous cyclophosphamide (1a/A) or mycophenolate (1a/A) and glucocorticoids (pulses of intravenous methylprednisolone followed by lower oral doses); combination therapy with belimumab (either with cyclophosphamide or mycophenolate (1b/A)) or calcineurin inhibitors (especially voclosporin or tacrolimus, combined with mycophenolate, 1b/A) should be considered.
9. Following renal response, treatment of lupus nephritis should continue for at least 3 years (2b/B); patients initially treated with mycophenolate alone or in combination with belimumab or a calcineurin inhibitor should remain on these drugs (1a/A), whereas azathioprine or mycophenolate should replace cyclophosphamide for those initially treated with cyclophosphamide alone (1a/A) or in combination with belimumab (1a/A).
10. In patients at high-risk for renal failure (defined as reduced GFR, histological presence of cellular crescents or fibrinoid necrosis, or severe interstitial inflammation), high-dose (NIH regimen) intravenous cyclophosphamide (1a/A) in combination with pulse intravenous methylprednisolone, can be considered.
11. In patients with SLE achieving sustained remission, gradual tapering of treatment should be considered, with withdrawal of glucocorticoids first (2a/B).
12. SLE associated with thrombotic antiphospholipid syndrome (APS) should be managed with long-term vitamin K antagonists after the first arterial or unprovoked venous thrombotic event (1b/B); low dose aspirin (75–100 mg/day) should be considered in patients with SLE without APS but with high-risk aPL profile (2a/B).
13. Immunisations for the prevention of infections (herpes zoster virus, human papillomavirus, influenza, COVID-19 and pneumococcus), management of bone health, nephroprotection and cardiovascular risk, and screening for malignancies, should be performed (5/D).

Recommendation Grading

Document Overview

Document Title: Management of Systemic Lupus Erythematosus

Authoring Society: European League Against Rheumatism (EULAR)

Document Publication Date: Jul 22, 2024

Page Last Reviewed/Updated: May 6, 2026

Document Type: Guideline

Country of Publication: European

Full Text Freely Available: Yes

Full Text Guideline: ard.bmj.com/content/83/1/15

Source Citation: Mucke J, Aringer M. Die 2023 EULAR-Empfehlungen zur Behandlung des systemischen Lupus erythematodes – Implikationen für die Versorgung in Deutschland [EULAR recommendations 2023 on the treatment of systemic lupus erythematosus -Implications for treatment in Germany]. Z Rheumatol. 2024 Aug;83(6):431-438. German. doi: 10.1007/s00393-024-01544-5. Epub 2024 Jul 22. PMID: 39037547.

Document Scope, Criteria, and Use Cases

Document Objectives: Provide updated recommendations for the management of systemic lupus erythematosus (SLE) based on emerging new evidence.

Scope: Assessment and Screening, Management

Diseases/Conditions (MeSH)

D008180 - Lupus Erythematosus, Systemic

Keywords: Systemic Lupus Erythematosus, lupus nephritis

Target Patient Population: Patients with systemic lupus erythematosus

Target Provider Population: Rheumatologists, immunologists, nephrologists, dermatologists, neurologists, cardiologists, hematologists, primary care physicians

Inclusion Criteria: Male, Female, Adult, Older Adult

Health Care Settings: Ambulatory

Intended Users: Nurse, Nurse Practitioner, Physician, Physician Assistant

LOE	Meaning
1a	systematic review of RCTs
1b	individual RCT
2a	systematic review of cohort studies
2b	individual cohort study (and low-quality RCT)
3a	systematic review of case–control studies
3b	individual case–control study
4	case series and poor-quality cohort and case–control studies
5	expert opinion without explicit critical appraisal, or based on physiology, bench research or ‘first principles’

SOR	Meaning
A	consistent level 1 studies
B	consistent level 2 or 3 studies, or extrapolations from level 1 studies
C	level 4 studies or extrapolations from level 2 or 3 studies
D	level 5 evidence or troublingly inconsistent or inconclusive studies of any level