Executive Summary: The 2026 British Society for Rheumatology guideline for the management of children, young people and adults with systemic lupus erythematosus

Graphical Abstract

Graphical Abstract.

This is the executive summary of ‘British Society for Rheumatology guideline for the management of children, young people and adults with systemic lupus erythematosus.’ For the full guideline, please see https://doi.org/10.1093/rheumatology/keag224.

Background

Systemic lupus erythematosus (SLE) is a complex, life-long autoimmune disease that causes inflammation and subsequent damage to multiple organs including skin, joints, the kidneys, brain, lungs, and heart if not adequately treated. It can present at any age with 15–20% of affected people developing symptoms in childhood. SLE is uncommon; ∼1 in 1000 adults in the UK have this condition [1]. Despite advances in therapy, adults with SLE still die on average 25 years earlier than otherwise expected [2], with mortality rates of up to 10% within 10 years of juvenile-onset SLE (JSLE) diagnosis [3]. Furthermore, health disparities are significant in SLE and affect outcomes. For example, JSLE has a more aggressive presentation and disease course compared with adult-onset SLE [4, 5], and people of Black African and South Asian ancestry are associated with more severe lupus and poorer outcomes compared with people of European ancestry [1, 6, 7]. Early diagnosis, empowering individuals to manage their condition, instituting appropriate immunosuppression swiftly and limiting toxicity from treatment is key to mitigating organ damage and mortality from SLE, and improving quality of life.

Rationale for updating guideline

The first British Society for Rheumatology (BSR) guideline for the management of adult SLE in 2017 [8] was comprehensive and an important step forward to harmonize the management of adults living with SLE across the UK. Updating the guideline is now required to reflect current best practice by incorporating all the available high-quality evidence and significant advances in the management of SLE, particularly in the field of lupus nephritis. This guideline builds on and widens the scope from the 2017 BSR SLE guideline by including guidance on non-pharmacological approaches, the management of cutaneous lupus erythematosus (CLE) and delivery of care to harmonize care within the UK system and define auditable standards.

In line with other BSR guidelines and equality considerations, this updated guideline includes consideration of people of all ages, sex, ancestral and sociodemographic factors that are relevant in the management of SLE.

Objectives

The objective of this life-course guideline is to produce comprehensive, patient-focused, evidence-based, and expert consensus recommendations for the management of people with SLE. It covers the diagnosis, assessment, monitoring, non-pharmacological and pharmacological management, and the delivery of care in the UK.

Methods and rigour of development

This guideline was developed in line with the BSR Guidelines Protocol (version 5.4, November 2023) using Grading of Recommendations, Assessment, Development and Evaluation (GRADE) [9] and Appraisal of Guidelines for Research and Evaluation II (AGREE II) [10] methodologies. The scope of the guideline was agreed by the Guideline Working Group (GWG) to address 13 key questions [11].

Based on these key questions, a literature search was undertaken (Supplementary material, S2 and S3, available at Rheumatology online), eligible papers were reviewed, draft recommendations developed and the GRADE process followed to summarize the level of the evidence (LoE) of each proposed recommendation into A (high), B (moderate), C (low) or D (very low). The strength of recommendation (SoR) for each recommendation was graded as either strong = 1 or weak = 2. Strength of agreement (SoA) was determined by the working group responses. Only recommendations with a SoA ≥80% were included. In this executive summary, we provide the recommendations followed by the SoR (1 or 2), LoE (A, B, C or D) and mean SoA score across the GWG in percentages.

The final draft of the guideline was submitted to the BSR Guidelines Steering Group for stakeholder and internal review and feedback.

Key guidance

There are 102 recommendations. Please refer to the Full Guidance for rationale and evidence supporting each statement.

(A) Diagnosis: what clinical and serological features should prompt consideration of a diagnosis of SLE in primary or secondary care? 

Importance of timely referral to secondary or tertiary care for diagnosis

1. People suspected to have SLE should be referred to secondary or tertiary care for diagnosis. Those with a strong suspicion of a new diagnosis of SLE should be prioritized as a 3-week wait target (1C, 96.4%).

   Classification criteria for SLE

2. SLE should be diagnosed by clinicians experienced in its management and requires a combination of clinical, laboratory and immunological features attributable to SLE (2B, 98.3%).

3. Current classification criteria (SLICC 2012 or EULAR/ACR 2019) may be used as a guide but not as diagnostic criteria (2C, 97.7%).

   ANA and beyond

4. Antinuclear antibody (ANA) test should only be performed where there is reasonable clinical suspicion of SLE or related diseases. ANAs are present in 95–99% of people with SLE, so a negative test makes SLE unlikely (results can fluctuate so a negative test does not rule out SLE where there have been past positives) (2B, 95.2%).

5. ANA test should be supplemented by tests for anti-dsDNA antibodies, extractable nuclear antigen antibodies and C3/C4 complement levels since the presence of anti-dsDNA antibodies (2B), low complement levels (2C), and/or anti-Smith (Sm) antibody (2C) is highly supportive of SLE diagnosis in people with relevant clinical symptoms (SoA 97.4%).

6. In children and young people with symptoms suggestive of SLE but negative ANA, monogenic lupus and complement deficiencies should be considered (2C, 98.7%).

7. Antiphospholipid antibodies (aPLs) and lupus anticoagulant (LAC) should be tested in all people with SLE at diagnosis (2B). A positive test requires confirmation on two occasions at least 12 weeks apart (2B) (SoA 98.0%).

   (B) Assessment: what is the best way to assess people with SLE? 

   Clinical assessment

8. Clinical manifestations in people with SLE should be assessed to determine if they are due to disease activity, damage, drug toxicity, infection, thrombosis or the presence of comorbidities (2B, 98.1%).

9. People with SLE should be assessed for manifestations in all systems and relevant clinical examination and investigations performed (2C, 98.9%).

   Disease activity and validated clinical tools

10. The use of validated disease activity tools such as BILAG-2004 and SLEDAI-2K is recommended (2B, 97.0%).

11. Validated instruments should be used to categorize disease activity as absent, mild, moderate and severe, with the occurrence of flares (2C, 96.5%).

12. Urinalysis and blood pressure should be performed at every visit (2C, 97.2%).

    Complications of SLE

13. In people with SLE with unexplained fever, especially when associated with falling blood counts and high ferritin, evolving HLH/MAS should be considered together with other differentials such as infection or flare. Investigate as per the GIRFT guidance, including calculation of the H-score (2C, 97.1%).

14. Cognitive impairment affecting daily activities, decisions (and schooling in JSLE), should be assessed in collaboration with a neurologist, neuropsychologist or other experienced professionals (2C, 96.0%).

    (C) Monitoring: What is the best way to monitor people with SLE? 

15. Those with active disease should be reviewed at least every 1–3 months (2C), with blood pressure (1A), urinalysis (1A) and renal function including proteinuria estimation (1A), anti-dsDNA antibodies (2B), complement levels (2C), inflammatory markers (2C), full blood count (2C) and liver function tests (2D) forming part of the assessment, and further tests as necessary (2D) (SoA 95.0%).

16. Body weight should be checked at each visit, and for children and young people with SLE, height should also be checked at each visit to monitor linear growth (2C, 97.0%).

17. People with SLE with stable low disease activity or in remission can be monitored less frequently. The interval will vary according to age, initial severity, duration of remission and therapy changes, but should be no longer than 12-monthly (adults) or 6-monthly (children) (2D, 97.4%).

18. People with SLE should have a systematic assessment of disease damage every 12–24 months. The SLICC/ACR damage index may be used (2C, 96.8%).

19. Immunosuppressive therapy and biologics may lead to toxicities. Close monitoring of drugs by regular laboratory tests and clinical assessment should be performed in accordance with drug monitoring guidelines (2D, 99.1%).

20. The presence of aPLs is linked to thrombotic events, organ damage, and adverse pregnancy outcomes (2B). If previously negative, aPLs should be re-evaluated before pregnancy, major surgery, or following a new severe manifestation or vascular event (2D, 96.7%).

21. Anti-Ro/SSA and anti-La/SSB antibodies are associated with neonatal lupus (including congenital heart block) and should be re-assessed prior to pregnancy, and pre-pregnancy counselling provided (1A, 98.5%).

    (D) Treatment target: what is the role of a treatment target in the management of SLE? 

22. Early diagnosis, regular screening for organ or system involvement, prompt initiation of treatment, and regular assessment of adherence is recommended in the management of SLE (2C, 98.8%).

23. The primary treatment goal should be to achieve remission, or if remission is not possible, Lupus Low Disease Activity State (or cLLDAS in JSLE), following a treat-to-target (T2T) approach (2C, 97.4%).

    (E) Non-pharmacological approach: what non-pharmacological interventions are supported by evidence and/or expert consensus to inform a BSR guideline? 

    General principle: education and adherence

24. People with SLE and their parents/carers should be offered age, culture and language-appropriate education and self-management support as part of routine care (1C, 96.9%).

25. Clinicians should promote and monitor adherence. For example: discuss the importance of adherence on disease control and long-term outcomes, monitor prescriptions and drug levels, practice shared decision-making on treatment and route of administration (1D, 98.0%).

    Lifestyle measures

26. People with SLE should be advised at diagnosis and more frequently as necessary to use high sun protection factor (SPF), broad-spectrum UV-A and UV-B sunscreen (1B) and to consider safe sun exposure strategies such as reduced sun exposure and the use of protective clothing (1D) (SoA 96.9%).

27. In people with SLE, smoking habits should be assessed, and people guided to cessation strategies (1C, 98.2%).

28. Physical activity should be encouraged as appropriate to disease status in adults (1B) and children (1C) with SLE. This will include tailored advice for some patients (SoA 97.0%).

    Dietary measures and supplementation

29. A healthy, balanced diet, as recommended for people without SLE, should be recommended for people with SLE. More specific advice may be required for those with LN (1D, 96.8%).

30. Clinicians should recommend that people with SLE take a daily vitamin D supplement throughout the year, particularly in autumn and winter (1B). Where deemed appropriate, vitamin D levels should be tested and additional supplementation recommended if below the normal range (1C) (SoA 96.6%).

    Cardiovascular comorbidities, vaccination and bone health

31. People with SLE are at increased risk of cardiovascular comorbidities, therefore assessment and management of modifiable risk factors (e.g. hypertension, dyslipidaemia, diabetes, high BMI and smoking) should take place at baseline and at least annually, using appropriate risk assessment tool where applicable (1B, 96.6%).

32. The importance of receiving eligible vaccinations should be emphasized at least annually. Additionally, when boosters or new vaccinations are recommended, this guidance should be reinforced (1C, 98.4%).

33. Annual assessment and management of bone health should be performed including diet, exercise, lifestyle factors and medications. Vitamin D status and bone density should be assessed where indicated (2C, 97.6%).

    Fatigue and psychological support

34. Health professionals should incorporate assessment of fatigue severity, impact and coping strategies alongside disease activity assessment during clinical consultations (1D, 95.4%).

35. Psychological support should be available to people with SLE, whenever possible as part of the multidisciplinary team (MDT) (1C, 96.7%).

36. Specialist psychology care should be available for specific problems associated with SLE in adults (1A) and children (1C, 96.3%).

37. All healthcare professionals assessing people with SLE should know how to signpost towards and/or refer to mental health services where there is an identified need for support (1D, 97.8%).

    (F) Antimalarials in SLE: General recommendations and what is the best evidence for monitoring therapies? 

    Antimalarials

38. Hydroxychloroquine (HCQ) is recommended for all people with SLE unless contraindicated (1A), at long-term target dose of 5 mg/kg/day (based on actual body weight, usual maximum 400 mg/day) (2C). Dose can be individualized based on risk of flare (2B), risk of retinal toxicity (2C) and other adverse events (2D). Dose reduction of 50% may be needed if eGFR<30 mL/min/1.73 m² (2C) (SoA 99.1%).

39. If people with SLE suffer persistent non-serious adverse effects with HCQ, consider switching to a HCQ product made by a different manufacturer as excipients may differ (2C, 97.6%).

40. We recommend following the Royal College of Ophthalmologists (RCOphth) guidelines for HCQ with ocular toxicity monitoring at 5 years, then annually thereafter (1B for adults, 2D for JSLE). For higher risk patients including those on HCQ dose of >5 mg/kg/day based on actual body weight, on tamoxifen, those with renal impairment, and those who have taken chloroquine for greater than one year, annual screening is recommended (2B) (SoA 98.1%).

41. Pathways should be established locally to facilitate ocular toxicity monitoring in accordance with the RCOphth Guidelines (2D, 97.9%).

42. The date of commencement of HCQ and dosing should be recorded in the medical records in a way that is easily retrievable (2D, 95.4%).

43. When using a standard HCQ dose, baseline or monitoring ECGs are not required unless there are specific drug-drug interactions, especially with other therapies causing long-QT (2C, 97.0%).

    (G) Non-renal SLE: what is the best evidence for the management of inactive or low disease activity SLE? 

    Conventional immunosuppressant and glucocorticoids

44. Other treatments to be considered for the management of mild non-organ threatening disease that is refractory to HCQ include methotrexate (1B), mepacrine (2C) and azathioprine (2C) (azathioprine especially for those who may become pregnant) (2C) (SoA 97.4%).

45. Glucocorticoids (GC) including topical preparations, intra-articular or intra-muscular injections or intravenous methylprednisolone may be used depending on the type and severity of organ involvement, as a bridging therapy to settle a flare and/or while HCQ or a new or increased dose of an immunosuppressant takes effect (2C, 97.5%).

46. Long-term maintenance treatment with oral GC is not routinely recommended to treat disease activity. If used, target dose should be as low as possible aiming for ≤5 mg/day prednisolone (or equivalent) in adults (0.1 mg/kg/day, up to 5 mg/day in children) (1B, 97.4%).

    (H) Non-renal SLE: what is the best evidence for the management of moderate to severely active SLE? 

    Early use of a conventional immunosuppressant

47. In people with SLE with moderate disease activity but no evidence of organ or life-threatening disease, immunosuppression such as methotrexate (1B), mycophenolate (2B), azathioprine (2C) or ciclosporin/tacrolimus (2C), in addition to HCQ, should be started early (SoA 98.8%).

    Consideration of biological therapy

48. In people with SLE with moderate to severe non-renal disease or the inability to withdraw GC despite the use of at least one immunosuppressant, discuss biological therapy and consider clinical trials with a specialized centre MDT (follow local processes for urgent cases) (2C, 98.2%).

    Belimumab

49. Belimumab is recommended as add-on treatment in autoantibody-positive people with SLE aged 5 or over with high disease activity (i.e. defined as at least one serological biomarker [positive anti-dsDNA or low complement] and a SLEDAI score of ≥10) despite standard treatment (1A, 96.9%).

    Rituximab, anifrolumab and preference for specific biologics or other induction therapies

50. Depending on specific patient presentation and situation, rituximab (2B) or anifrolumab (1A) may be more appropriate than belimumab or clinical trials in non-renal SLE (SoA 97.1%).

51. Intravenous immunoglobulin could be considered for severe autoimmune thrombocytopaenia attributed to SLE (1B, 98.3%).

52. In people with SLE with active and non-thrombotic neuropsychiatric disease attributed to SLE, GC and intravenous cyclophosphamide (2C), rituximab (2C) or combination of cyclophosphamide and rituximab (2C) should be considered (SoA 97.6%).

    Remission induction in organ-threatening or life-threatening non-renal SLE

53. For remission induction of people with SLE with organ-threatening or life-threatening non-renal disease attributed to disease activity, high dose GC with intravenous cyclophosphamide (2C), rituximab (2C) or combination of cyclophosphamide and rituximab (2C) should be considered. Plasma exchange and intravenous immunoglobulin could be considered (2D) (SoA 97.0%).

    Maintenance therapy following remission induction for organ-threatening or life-threatening non-renal disease attributed to SLE

54. Following remission induction for organ-threatening or life-threatening non-renal disease attributed to SLE, maintenance therapy with belimumab (1A), anifrolumab (1A) mycophenolate (1B), rituximab (2C), azathioprine (2C), methotrexate (2C) or ciclosporin/tacrolimus (2C) should be considered (SoA 98.3%).

    (I) Organ-specific approach: what is the best evidence for the management of cutaneous lupus erythematosus in SLE? 

55. In people with SLE with cutaneous manifestations, consider drug-induced lupus as a cause (2C, 97.4%).

56. Scarring and/or refractory cutaneous involvement in SLE should be managed in a timely manner in conjunction with dermatologists (1D, 98.3%).

57. Offer very potent/potent topical GC as a first-line therapy in cutaneous involvement (non-facial) attributed to SLE, for up to 4 weeks (1B, 95.9%).

58. Consider topical GC on the face with appropriate dermatology guidance (2C, 94.0%).

59. Offer topical calcineurin inhibitors in cutaneous involvement (non-facial) attributed to SLE for up to 4 weeks and longer if agreed with dermatologists (1B, 96.8%).

60. Offer very potent/potent topical GC and/or calcineurin inhibitors as an adjuvant to systemic therapy when there is widespread cutaneous involvement in SLE (1B, 96.2%).

61. Consider concomitant short-term systemic GC alongside other systemic and topical therapies, especially in people with SLE at high risk of scarring with appropriate guidance by dermatologists (2D, 94.9%).

62. In refractory cutaneous SLE, optimize hydroxychloroquine by adding mepacrine (2B) and refer to dermatology to consider other therapeutic options, for example, intralesional GC (2D), retinoids (2C), dapsone (2C), clofazimine (2C), thalidomide (2C), lenalidomide (2C) or ciclosporin (2C) (SoA 95.2%).

63. For refractory cutaneous involvement in SLE, the best evidence is for anifrolumab and this should be used as first-line biologic if available (1B). If not available, other biologics can be considered as per management of non-renal SLE (2B) (SoA 97.0%).

    (J) Organ-specific approach: what is the best evidence for the general management of lupus nephritis? 

    Overall care of people with lupus nephritis

64. All people with lupus nephritis should be managed in combined services with rheumatology and renal expertise for use of adjunctive therapies in addition to immunosuppression, and to ensure optimal management of acute kidney injury and CKD. Consult the KDIGO, EULAR, ACR and SHARE guidelines as appropriate (2D, 97.8%).

    Kidney biopsy

65. A timely kidney biopsy should be included in the initial diagnosis of lupus nephritis (2B, 98.5%).

    Proteinuria monitoring and diagnostic tests

66. For detailed guidance on monitoring for proteinuria, the role of biopsy and other diagnostic tests in adults and children, consult the KDIGO, EULAR, ACR and SHARE guidelines, respectively (2B, 98.1%).

    Initial assessment

67. Before deciding treatment, identify important prognostic factors including first episode of nephritis or a relapse, prior therapy failures, patient understanding and potential adherence concerns, histological LN class, baseline kidney function and degree of chronicity on the biopsy, presence of crescents or tubulointerstitial lesions, renal function, level of proteinuria, non-renal SLE features, relevant renal and non-renal co-morbidities, and GC toxicity (prior or future risk) (2B, 98.0%).

    (K) Organ-specific approach: what is the best evidence for active lupus nephritis induction treatment? 

    Intravenous glucocorticoids

68. For adults with LN requiring induction therapy, start GC therapy with 250–500 mg intravenous methylprednisolone/day for 1–3 days. For children and young people with LN, 10–30 mg/kg/day up to 500 mg total for 1–3 days (2B). Higher doses, up to 1000 mg/day, may be used for adults, children or young people under expert guidance (e.g. severe renal failure or multi-organ involvement) (SoA 98.2%).

    Oral glucocorticoids

69. Following IV methylprednisolone, adults with LN should be treated with one of the KDIGO GC regimens with the taper planned from the outset (2B, 96.3%).

70. In adults with LN without severe histology or high-risk features, and while treatment response remains acceptable, the KDIGO GC reduced-dose regimen is preferable and is especially suitable with combination immunosuppressants and/or those with high risk of GC toxicity (2B, 96.6%).

71. In adults with LN with severe histology, severe extra renal disease, not able to take combination immunosuppressants or poor initial response, the KDIGO moderate (or rarely high dose) GC regimen may be preferable (2B, 95.3%).

72. For children and young people with SLE, a similar principle should be followed for oral GC prescription in LN induction therapy (prescribing proportionate to patient characteristics and planning GC taper in advance) using weight-based dosing as appropriate (2B, 96.6%).

73. Planned GC tapering should be supervised by a physician and adjusted as appropriate to response (2B, 98.7%).

    Induction therapy: first line recommendation

74. People with SLE with new or relapsing class III or IV LN (with or without class V) should be offered combination immunosuppressant regimen including GC, HCQ and one of these combinations of immunosuppressants:

    • mycophenolic acid analogues (MPAA) and obinutuzumab (1A, 2D for JSLE);

    • MPAA and calcineurin inhibitors (CNI) (voclosporin 1A for adults, 2D for JSLE, tacrolimus 1B for adults, 2C for JSLE);

    • MPAA and belimumab (1A for adults, 2C for JSLE) (SoA 95.6%).

      Induction therapy: second line or special case recommendations

75. If the regimens in #74 are not suitable or available as remission induction for LN, then alternative immunosuppressant options with substantial evidence but lower efficacy are as follows:

    • EuroLupus dose cyclophosphamide combined with belimumab (1A);

    • MPAA combined with rituximab (2B);

    • MPAA alone (1A);

    • EuroLupus dose cyclophosphamide alone (2B) (SoA 76.5%).

      Pure class V

76. People with SLE with new or relapsing pure class V LN with proteinuria >100 mg/mmol should receive a combination immunosuppression regimen consisting of oral GC using the reduced-dose KDIGO regimen and MPAA and Calcineurin inhibitor (1A). If not appropriate or tolerated, consider alternative therapies including oral GC + MPAA + anti-CD20 therapies (2B), oral GC + MPAA + belimumab (2C) or oral GC + EuroLupus dose cyclophosphamide + belimumab (2C) (SoA 96.1%).

    Exceptionally severe lupus nephritis

77. In cases of exceptionally severe or life- or organ-threatening LN, initial therapy should be discussed with a specialist centre as the National Institute for Health (NIH) regimen of IV cyclophosphamide (2B), with or without anti-CD20 agents (2C), may be more appropriate than standard LN induction therapy initially. In such cases, the number of cyclophosphamide infusions should be limited and therapy switched to options with lower toxicity once disease activity improves (SoA 93.8%).

    Induction therapy: assessing response and switching

78. Therapy should be reconsidered early if people with LN are not meeting these treatment targets (2C):

    • at 3 months, 25% reduction in urinary protein to creatinine ratio (uPCR);

    • at 6 months, 50% reduction in uPCR;

    • at 12 months, uPCR less than 70 mg/mmol; with preserved or improving kidney function. In those with class V LN, slower reduction of proteinuria is acceptable as long as improving (SoA 95.6%).

79. In people with LN with a partial response to the induction regimen treatment but not meeting treatment targets, expert opinion, or more specialist advice, should be used to decide on repeat biopsy and whether further induction regimen required or a change of maintenance therapy (2B, 98.4%).

80. In people refractory to the initial treatment for LN, reasons for suboptimal response should be fully re-assessed including checking adherence, optimizing doses of immunosuppressants and checking drug levels, and considering a repeat renal biopsy. Expert opinion, or more specialist advice, must be used to decide therapy (2B, 81.7%).

81. Treatment options for refractory LN include clinical trials (2B), switching to one of the other induction immunosuppressant combinations in #74 (2B), and/or restarting induction GC (2B) (SoA 96.6%).

    (L) Organ-specific approach: what is the best evidence for lupus nephritis maintenance treatment? 

    Rationale for maintenance treatment for lupus nephritis

82. Maintenance therapy should be continued for at least 3 years (1A). Longer durations may be advantageous in many people with LN and the decision should be individualized based on quality of response, prior relapses, risk associated with relapse, tolerability, biomarkers, extra-renal disease activity and patient preference (2C) (SoA 97.5%).

    Recommended maintenance treatment in lupus nephritis

83. In people with LN meeting the 12-month treatment targets after induction therapy using regimens in #74, the following treatment maintenance should be continued for at least 3 years:

    • MPAA + Obinutuzumab (1B);

    • MPAA + Belimumab (1A);

    • MPAA + CNI (1A) (SoA 96.2%).

84. In people with LN who received a different induction option to regimens in #74, combination therapies in #83 could be added for maintenance if appropriate (2B) (SoA 95.2%).

85. If rituximab and MPAA were used for induction with good response, then this combination could be continued for maintenance, individualizing retreatment interval (2C, 97.8%).

86. If combination therapy cannot be used, then MPAA should be used for maintenance therapy (1A, 97.3%).

87. If MPAA cannot be taken or pregnancy is planned, then azathioprine (or combination of azathioprine and tacrolimus) can be used for maintenance with closer monitoring for LN flare (1A, 98.3%).

    Full withdrawal of GC should be attempted following remission induction

88. Following the GC induction regimen for LN, full withdrawal of GC should be attempted; use of GC is not recommended for long-term control of disease activity after either full or partial response (2B, 97.4%).

    Management of chronic kidney disease

89. During maintenance therapy, people with LN should continue to be managed by renal services, combined with rheumatology if necessary, to ensure appropriate chronic kidney disease (CKD) management using adjunctive therapies (2B, 97.0%).

    (M) Delivery of care: how should care be delivered to optimize management of people with SLE? 

    Multispecialty/multiprofessional MDT meetings, regional specialized networks, and specialist lupus centres

90. Where there is complex multisystem disease, people with SLE should be co-managed with relevant specialties in joint clinics or if not, at least with regular multispecialty/multiprofessional MDT meetings that enable rapid communication (1D, 99.8%).

91. Every healthcare organization treating SLE should be part of a regional specialized network/regional MDT meetings so that people with SLE can receive access to additional specialized care and advice, combined with ongoing local hospital care, regardless of geography (1C). Such services are still in development for JSLE but should be used when available (SoA 98.1%).

92. All people with SLE with severe, complex or refractory disease including those with lupus nephritis, especially those being considered for biologic drugs must be discussed with and/or managed by a specialist lupus centre (1D, 98.4%).

    Shared care and a coordinated transition plan

93. Shared care within secondary care and between primary and secondary/tertiary care should be funded, supported and standardized (1D, 99.6%).

94. A coordinated transition plan including paediatric and adult specialists should be put in place to manage this high-risk period of SLE in a young person’s journey (1C, 97.9%).

    Organizing lupus care in healthcare organization

95. People with SLE should be seen in dedicated/cohorted clinics with specific clinic templates adjusted for complexity to allow adequate time for a full multisystem holistic assessment (1C, 98.6%).

96. Services should consider and develop ways to address potential health inequalities, particularly those faced by minoritized and underserved groups and from low socioeconomic backgrounds to ensure equitable access to optimal management (1D, 97.4%).

97. Where there are communication barriers including where interpreters are required, longer clinic appointment times are recommended (1D, 96.7%).

98. Each healthcare organization should have a lead clinician for the delivery of service for people with SLE, with recognition in job plan for this activity (1D, 96.8%).

99. Every individual with SLE should have access to a specialist nurse with understanding and expertise in management of lupus with dedicated time within their job plan, including access to a telephone and/or email advice line (1C, 97.7%).

100. People with SLE with disease flares or possible drug-related side effects should be able to receive timely advice (ideally within 1–2 working days) of contacting their hospital service (1C, 98.4%).

     Opportunity to take part in research, registry and national audits

101. All people with SLE should be given the opportunity of taking part in observational research studies, and if treated with biologic drugs, with their consent should be registered with the National BILAG Biologics Register (BILAG BR) and/or UK Juvenile SLE (JSLE) Cohort Study (1D, 98.8% [80–100]).

102. All departments should collect the required data for national audits and registries. Healthcare organizations should ensure that there is appropriate administrative support for audit participation. Clinical and operational teams should review their audit data and take any required action (1D, 97.3%).

Applicability and utility

A model audit tool is provided as Supplementary Material, available at Rheumatology online.

Supplementary material

Supplementary material is available at Rheumatology online.

Data availability

The data underlying this article are available in the article and in its Supplementary Material.

Contribution statement

M.M., E.M.D.S. and E.M.V. drafted this article. All authors contributed to developing the guideline scope, provided critical manuscript editing and revision, and approved the final submitted version.

Funding

This work was supported by the British Society for Rheumatology. There was no specific funding received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this guideline.

Disclosure statement: M.Y.M.Y. has received speaker fees from Alumis, Novartis, Roche, and CSL Vifor and consultancy fees from Aurinia Pharmaceuticals, Autolus, GSK, Novartis, and UCB. C.J.E. has received consulting fees and/or honoraria from Abbvie, Astra Zeneca, Gilead, GSK, Roche, Lilly, Otsuka, BMS and Pfizer. K.F. has received honararia from Galapagos, Abbvie, UCB and Janssen. R.G. has received honoraria from GSK. C.G. has received consultancy fees from UCB, Amgen, Alumis, Astra-Zeneca, MGP and Sanofi. B.G. has received consultancy fees from Roche and speaker fees from Johnson and Johnson. A.K. has received honoraria from Novartis, AbbVie, Janssen, Lilly, Leo and Pfizer. A.K. holds the role of Associate Editor for Rheumatology and has not peer reviewed or made any editorial decisions for this article. P.L. has received research grant funding, consulting fees and speaker fees from CSL Vifor, consulting fees and speaker fees from Astra Zeneca and consultancy fees from Pfizer. P.M.L. has received honoraria and/or grants as an investigator, speaker, and/or advisory board member from AbbVie, Almirall, Actelion, Bristol-Myers-Squibb, Celgene, Janssen, Lilly, Sanofi, Leo, Pfizer, UCB and Novartis. L.L has received consulting fees and/or honoraria from GSK, Alexion, AstraZeneca, Biogen, BMS, Kezar, Novartis, Pfizer and Otsuka. S.D.M. has received speaker fees from GSK. C.W. has received honoraria from Abbvie, AstraZeneca, Biogen, Bristol Myers Squibb, CSL Vifor, Gilead, Kyverna, Novartis, Otsuka, Roche, UCB, Viatris, and research grants paid to his employer from AstraZeneca. E.M.V. has received honoraria from Novartis, AstraZeneca, Otsuka, Roche, UCB, Aurinia, Lilly, Alumis, BMS, GSK, Pfizer and research grants paid to his employer from AstraZeneca and Sandoz. All other authors declare no competing interest related to the work described in this manuscript.