The European Alliance of Associations for Rheumatology (EULAR) have updated their guidelines on how doctors should manage lupus.

 

Who are EULAR?

“EULAR” stands for “The European Alliance of Associations for Rheumatology”. They represent patients with rheumatic diseases, health professionals, and scientific societies about rheumatology across Europe.

You can find out more about EULAR and their work here: www.eular.org/whoweare

What are the EULAR recommendations on lupus for?

EULAR have recommendations about how to treat lupus, which are developed by experts in lupus who look at the latest research and treatment available. They are used to help guide doctors in Europe about how to treat lupus.

Do doctors in the UK follow the EULAR guidelines?

In the UK, we have the British Society for Rheumatology (BSR). They also publish their own guidelines, which will be updated next year (2024). The BSR guidelines are the ones that doctors in the UK usually follow to help them treat lupus patients effectively. However, doctors in the UK will also look at the EULAR guidelines to keep up-to-date on best practice, especially before the BSR guidelines are also updated.

The EULAR guidelines cover multiple countries, so some of the recommended drugs or treatments may not be available in the UK, such as anifrolumab. The “overarching principles” in the guidelines are very important for all doctors treating patients with lupus and can be used by doctors in the UK as they are not about specific treatments.

What do the new 2023 recommendations on lupus say?

The 2019 version had 33 recommendations, and the new 2023 guidelines have 5 overarching principles and 13 recommendations. They made some new recommendations and combined old recommendations. Because they merged or re-worded some of the old guidelines, they have presented all of the recommendations as “new” because it was impractical to give detailed descriptions of all the smaller changes.

You can read the new guidelines, and the detailed explanation of each, for free here: https://ard.bmj.com/content/annrheumdis/early/2023/10/11/ard-2023-224762.full.pdf

The 2023 EULAR Guidelines are:

Overarching principles
A	SLE requires multidisciplinary, individualised management with patient education and shared decision-making, taking into consideration the costs to patient and society.
B	SLE disease activity should be assessed at each clinic visit (the frequency depending on physician’s discretion), with evaluation of organ damage (at least annually), using validated instruments.
C	Non-pharmacological interventions, including sun protection, smoking cessation, healthy, balanced diet, regular exercise and measures to promote bone health are important to improve long-term outcomes.
D	Pharmacological interventions are directed by patient characteristics, type and severity of organ involvement, treatment-related harms, comorbidities, risk for progressive organ damage and patient preferences.
E	Early SLE diagnosis (including serological assessment), regular screening for organ involvement (especially nephritis), prompt initiation of treatment aiming at remission (or low disease activity if this is not possible), and strict adherence to treatment are essential to prevent flares and organ damage, improve prognosis and enhance quality of life.
Individual recommendations
1	Hydroxychloroquine is recommended for all patients, unless contraindicated, at a target dose of 5 mg/kg real body weight/day, but individualised based on risk for flare and retinal toxicity.
2	Glucocorticoids, if needed, are dosed based on the type and severity of organ involvement, and should be reduced to maintenance dose of ≤5 mg/day (prednisone equivalent) and, when possible, withdrawn; in patients with moderate-to-severe disease, pulses of intravenous methylprednisolone (125–1000 mg per day, for 1–3 days) 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, azathioprine or mycophenolate) and/or biological agents (eg, belimumab or anifrolumab) should be considered.
4	In patients with organ-threatening or life-threatening disease, intravenous cyclophosphamide should be considered; in refractory cases, rituximab may be considered.
5	Treatment of active skin disease should include topical agents (glucocorticoids, calcineurin inhibitors), antimalarials (hydroxychloroquine, chloroquine), and/or systemic glucocorticoids as needed, with anifrolumab, belimumab, methotrexate, or mycophenolate, considered as second-line therapy.
6	In active neuropsychiatric disease attributed to SLE, glucocorticoids and immunosuppressive agents for inflammatory manifestations and antiplatelet agents/anticoagulants for atherothrombotic/antiphospholipid antibodies (aPL)-related manifestations should be considered.
7	For acute treatment of severe autoimmune thrombocytopenia, high-dose glucocorticoids (including pulses of intravenous methylprednisolone), with or without intravenous immunoglobulin G, and/or rituximab, and/or high-dose intravenous cyclophosphamide, followed by maintenance therapy with rituximab, azathioprine, mycophenolate, or cyclosporine, should be considered.
8	Patients with active proliferative lupus nephritis should receive low-dose (EuroLupus) intravenous cyclophosphamide or mycophenolate and glucocorticoids (pulses of intravenous methylprednisolone followed by lower oral doses); combination therapy with belimumab (either with cyclophosphamide or mycophenolate) or calcineurin inhibitors (especially voclosporin or tacrolimus, combined with mycophenolate) should be considered
9	Following renal response, treatment of LN should continue for at least 3 years; patients initially treated with mycophenolate alone or in combination with belimumab or a calcineurin inhibitor should remain on these drugs, whereas mycophenolate or azathioprine should replace cyclophosphamide for those initially treated with cyclophosphamide alone or in combination with belimumab
10	In patients at high risk for kidney failure (defined as reduced glomerular filtration rate, histological presence of cellular crescents or fibrinoid necrosis, or severe interstitial inflammation), high-dose (National Institutes of Health regimen) intravenous cyclophosphamide 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
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; low-dose aspirin (75–100 mg/day) should be considered in patients with SLE/without APS with high-risk aPL profile.
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