Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ONGOING

joint symptoms and serositis

1st line – hydroxychloroquine

Back
ONGOING
|
joint symptoms and serositis
1st line – 

hydroxychloroquine

Hyydroxychloroquine is recommended for all patients with SLE unless contraindicated.[47]

The beneficial effects of hydroxychloroquine in SLE include the reduction of constitutional symptoms, and reduced musculoskeletal and mucocutaneous manifestations.[101] Guidance recommends that patients who are in long-standing remission may lower their dose, although no studies have formally addressed this strategy.[47] 

Concerns exist regarding the development of retinal toxicity with hydroxychloroquine therapy.[102][103] Risk factors include duration of treatment, dose, chronic kidney disease, and pre-existing retinal or macular disease.[103] Retrospective case-control study data suggest that risk of toxic retinopathy is low for doses below 5.0 mg/kg of real body weight for up to 10 years.[102] 

Ophthalmological screening (by visual field examination and/or spectral domain-optical coherence tomography) is recommended at baseline, after 5 years, and yearly thereafter in the absence of risk factors for retinal toxicity.[47]

Can be used in combination with non-steroidal anti-inflammatory drugs and/or corticosteroids if required.[115][116]

Treatment with hydroxychloroquine needs to be sustained, but withdrawal during remissions should be considered.

Primary options

hydroxychloroquine: 200-400 mg/day orally given in 1-2 divided doses, maximum 5 mg/kg/day (base)

Plus – lifestyle changes and psychological therapies

Back
ONGOING
|
joint symptoms and serositis
Plus – 

lifestyle changes and psychological therapies

Treatment recommended for ALL patients in selected patient group

LIestyle changes include dietary advice, smoking cessation, sun protection, exercise, and psychological therapy.

Exposure to ultraviolet light may exacerbate or induce systemic manifestations of SLE.[83] Patients with SLE should be advised to avoid excessive sun exposure and to use a broad-spectrum sunscreen.[84] 

No dietary measures have been shown to alter the course of SLE. However, the late complications of premature cardiovascular disease should be borne in mind. Patients should be advised to maintain an ideal body weight for their height and reduce salt intake if hypertension due to renal disease is present. General advice includes eating at least 5 servings of fruit or vegetables per day, replacing saturated fats with monounsaturates and polyunsaturates, and increasing the amount of oily fish eaten; a diet rich in polyunsaturated fatty acids should be recommended.[85] Standard advice for the amount of alcohol per week for men and women should be given. 

SLE is associated with inadequate levels of serum vitamin D compared with the general population.[86][87][88] In patients with SLE, vitamin D supplements reduce disease activity; increase serum levels; and improve levels of inflammatory markers, fatigue, and endothelial function.[88][89][90]

Some evidence suggests that omega-3 fatty acid supplementation may reduce SLE disease activity.[89][91] 

Herbal preparations should be avoided. They can interact adversely with pharmacological agents and may cause harm.

Patients with stable SLE should be advised to avoid a sedentary lifestyle and to undertake supervised exercise.[85] In these patients, adherence to exercise guidelines should be encouraged to maintain optimum cardiovascular fitness. This should include ≥30 minutes of moderate physical activity ≥5 times per week; patients are advised to stop exercising if they experience pain or discomfort. 

Patients who smoke should be encouraged to stop. Evidence suggests smoking is associated with more active disease, and a significant reduction in the therapeutic effect of hydroxychloroquine.[44][96][97] Smoking cessation reduces the risk of atherosclerotic vascular disease. 

SLE has a significant impact on health-related quality of life, and has been shown to increase suicidal ideation and suicide attempts.[92][93] Literature reviews suggest that psychological interventions such as psychotherapy, cognitive behavioural therapies (CBT), psychoeducation, and mindfulness-based CBT, as adjuncts to medical therapy, improve fatigue, depression, pain, and quality of life for patients with SLE.[94][95]

Consider – non-steroidal anti-inflammatory drug

Back
ONGOING
|
joint symptoms and serositis
Consider – 

non-steroidal anti-inflammatory drug

Additional treatment recommended for SOME patients in selected patient group

Non-steroidal anti-inflammatory drugs (NSAIDs) are frequently used as a first-line measure in SLE to control joint stiffness as well as musculoskeletal and serosal pain. Naproxen may be the preferred first-line agent owing to the rare occurrence of aseptic meningitis with ibuprofen.[98][99][100] 

Blood pressure should be monitored and NSAIDs should be avoided in patients with hypertension or renal disease.

If long-term NSAID therapy is indicated, Helicobacter pylori eradication and the need for gastroprotection are considered.

Patients who require an anti-inflammatory and who are at high risk of gastrointestinal ulceration should be given a cyclo-oxygenase-2 (COX-2) inhibitor (e.g., celecoxib) if they are at low cardiovascular risk.

Primary options

naproxen: 500 mg orally twice daily when required, maximum 1500 mg/day

Secondary options

celecoxib: 100-200 mg orally twice daily

Consider – corticosteroid

Back
ONGOING
|
joint symptoms and serositis
Consider – 

corticosteroid

Additional treatment recommended for SOME patients in selected patient group

Used when non-steroidal anti-inflammatory drugs (NSAIDs) and hydroxychloroquine are inadequate.

Pulses of intravenous methylprednisolone are recommended to provide immediate therapeutic effect in SLE and enable the use of a lower starting dose of oral corticosteroids.[47] 

The recommended dose and route of administration depends on the type and severity of organ involvement, but for chronic maintenance treatment the dose should be minimised to <7.5 mg/day and, when possible, withdrawn.[47] 

The long-term adverse effects of corticosteroid therapy are well documented, and patients should be counselled regarding risk of hypertension and atherosclerotic disease, hyperglycaemia, potential skin changes, infection, mood disorders, disorders of bone and muscle (e.g., osteoporosis, osteonecrosis, myopathy), and ophthalmological effects (e.g., cataracts, increased ocular pressure, exophthalmos). Caution is advised with corticosteroid use in patients with upper gastrointestinal symptoms, especially if also taking NSAIDs.

The lowest possible dose to control symptoms should be used for the shortest period of time.

Can be used in combination with NSAIDs and/or hydroxychloroquine if required.[115][116]

Primary options

methylprednisolone sodium succinate: 250-1000 mg intravenously once daily for 3 days

OR

prednisolone: 5-60 mg orally once daily

More

Consider – immunosuppressant

Back
ONGOING
|
joint symptoms and serositis
Consider – 

immunosuppressant

Additional treatment recommended for SOME patients in selected patient group

The addition of immunosuppressive agents (such as methotrexate, azathioprine, or mycophenolate) should be considered for the treatment of patients with organ-threatening disease, patients not responding to hydroxychloroquine (alone or in combination with corticosteroids), and patients unable to reduce the corticosteroid dose below the acceptable dose for chronic use.[47]

Early initiation of immunosuppressive agents can expedite the tapering/discontinuation of corticosteroids.[47] 

Methotrexate can be a helpful addition in patients taking oral corticosteroids for arthritis/arthralgia.[117] Patients taking methotrexate should have regular haematological and liver function testing. Methotrexate use may increase the risk of infection. Abnormal haematological and/or liver function results may necessitate reduction in prescribed dose.[117] Folinic acid or folic acid (depending on local guidelines) is given to counteract the folate-antagonist action of methotrexate.

Primary options

methotrexate: 7.5 mg orally/intravenously/subcutaneously once weekly on the same day of each week, increase gradually according to response, maximum 20 mg/week

OR

azathioprine: 2 mg/kg/day orally, adjust dose according to response

OR

mycophenolate mofetil: consult specialist for guidance on dose

Consider – belimumab or rituximab

Back
ONGOING
|
joint symptoms and serositis
Consider – 

belimumab or rituximab

Additional treatment recommended for SOME patients in selected patient group

Belimumab should be considered as an add-on treatment for patients who have an inadequate response to combination treatment with hydroxychloroquine and corticosteroids with or without immunosuppressive agents (where inadequate response constitutes residual disease activity not allowing tapering of corticosteroids and/or frequent relapses).[47] 

In the UK, the National Institute for Health and Care Excellence (NICE) recommends belimumab as an add-on treatment for patients with active autoantibody-positive SLE with high disease activity despite standard treatment, only if: high disease activity is defined as at least 1 serological biomarker (positive anti-double-stranded DNA or low component) and a SELENA-SLEDAI (Safety of Estrogen in Lupus National Assessment - Systemic Lupus Erythematosus Disease Activity Index) score of greater than or equal to 10; treatment is continued beyond 24 weeks only if the SELENA-SLEDAI score has improved by 4 points or more.[108]​

One Cochrane review concluded that there is moderate- to high-quality evidence that belimumab is associated with clinically meaningful benefit for patients with SLE at 52 weeks compared with placebo. Patients receiving the approved dose showed at least a 4-point reduction in SELENA-SLEDAI score.[109]  

Belimumab significantly reduced organ damage progression compared with standard care in long-term study (5-year analysis) of patients with SLE.[110] 

Rituximab can be considered for patients with organ-threatening, refractory disease or with intolerance/contraindications to standard immunosuppressive agents.[47]

Consider pre-medication to attenuate infusion- and hypersensitivity-related reactions.

Primary options

belimumab: 10 mg/kg intravenously every 2 weeks for the first 3 doses, then every 4 weeks thereafter; 200 mg subcutaneously once weekly

More

Secondary options

rituximab: consult specialist for guidance on dose

mucocutaneous disease

1st line – hydroxychloroquine

Back
ONGOING
|
mucocutaneous disease
1st line – 

hydroxychloroquine

First-line treatment of skin disease in SLE includes antimalarials (e.g., hydroxychloroquine) with or without systemic corticosteroids (starting dose dependent on the severity of skin involvement) and topical agents (e.g., corticosteroids, calcineurin inhibitors).[47] 

Hydroxychloroquine is recommended for all patients with SLE unless contraindicated.[47]

The beneficial effects of hydroxychloroquine in SLE include the reduction of constitutional symptoms, and reduced musculoskeletal and mucocutaneous manifestations.[101] Guidance recommends that patients who are in long-standing remission may lower their dose, although no studies have formally addressed this strategy.[47]

Concerns exist regarding the development of retinal toxicity with hydroxychloroquine therapy.[102][103] Risk factors include duration of treatment, dose, chronic kidney disease, and pre-existing retinal or macular disease.[103] Retrospective case-control study data suggest that risk of toxic retinopathy is low for doses below 5.0 mg/kg of real body weight for up to 10 years.[102] 

Ophthalmological screening (by visual field examination and/or spectral domain-optical coherence tomography) is recommended at baseline, after 5 years, and yearly thereafter in the absence of risk factors for retinal toxicity.[47]

Can be used alone or in combination with corticosteroids if required.[115][116] 

Treatment with hydroxychloroquine needs to be sustained, but withdrawal during remissions should be considered.

Primary options

hydroxychloroquine: 200-400 mg/day orally given in 1-2 divided doses, maximum 5 mg/kg/day (base)

Plus – topical corticosteroid or calcineurin inhibitor

Back
ONGOING
|
mucocutaneous disease
Plus – 

topical corticosteroid or calcineurin inhibitor

Treatment recommended for ALL patients in selected patient group

First-line treatment of skin disease includes the use of topical agents (e.g., corticosteroids, calcineurin inhibitors).[47] 

Topical corticosteroids of different potencies may be used in combination depending on the patient’s symptoms. Potent corticosteroids (e.g., betamethasone valerate 0.1%) and very potent corticosteroids (e.g., clobetasol propionate 0.05%) are often used to treat the trunk and limbs including the hands, as well as the scalp. Moderate-potency corticosteroids (e.g., triamcinolone acetonide 0.1% or betamethasone valerate 0.025%) are used in areas more prone to atrophy such as the face and neck. Mild-potency corticosteroids (e.g., hydrocortisone 1%) are typically reserved for the eyelids, although may prove insufficient. Scalp involvement may be treated with foam or lotion formulations.

Primary options

hydrocortisone topical: (1%) apply to affected area(s) once or twice daily

More

OR

triamcinolone topical: (0.1%) apply to the affected area(s) once or twice daily

More

OR

betamethasone valerate topical: (0.025%) apply to the affected area(s) once or twice daily

More

OR

betamethasone valerate topical: (0.1%) apply to the affected area(s) once or twice daily

More

OR

clobetasol topical: (0.05%) apply to the affected area(s) twice daily

More

OR

tacrolimus topical: (0.03%, 0.1%) apply to the affected area(s) twice daily

Plus – lifestyle changes, supportive care, and psychological therapies

Back
ONGOING
|
mucocutaneous disease
Plus – 

lifestyle changes, supportive care, and psychological therapies

Treatment recommended for ALL patients in selected patient group

For patients with mucocutaneous manifestations, effective protection from ultraviolet exposure with broad-spectrum sunscreens and smoking cessation are strongly recommended.[47] 

A thorough oral care regime is recommended for all symptomatic patients.[118] Mouthwashes (e.g., chlorhexidine), basic oral hygiene, and regular attendance at a dental practitioner are helpful in the treatment of mouth ulceration. 

Lidocaine ointment may be beneficial for the management of pain secondary to major oral aphthae.[119]

Artificial saliva preparations may be required for those with dry mouth.[118]

Hypromellose eye drops are recommended for dry eyes.

No dietary measures have been shown to alter the course of SLE. However, the late complications of premature cardiovascular disease should be borne in mind. Patients should be advised to maintain an ideal body weight for their height and reduce salt intake if hypertension due to renal disease is present. General advice includes eating at least 5 servings of fruit or vegetables per day, replacing saturated fats with monounsaturates and polyunsaturates, and increasing the amount of oily fish eaten; a diet rich in polyunsaturated fatty acids should be recommended.[85] Standard advice for the amount of alcohol per week for men and women should be given. 

SLE is associated with inadequate levels of serum vitamin D compared with the general population.[86][87][88] In patients with SLE, vitamin D supplements reduce disease activity; increase serum levels; and improve levels of inflammatory markers, fatigue, and endothelial function.[88][89][90] 

Some evidence suggests that omega-3 fatty acid supplementation may reduce SLE disease activity.[89][91]

Herbal preparations should be avoided. They can interact adversely with pharmacological agents and may cause harm.

Patients with stable SLE should be advised to avoid a sedentary lifestyle and to undertake supervised exercise.[85] In these patients, adherence to exercise guidelines should be encouraged to maintain optimum cardiovascular fitness. This should include ≥30 minutes of moderate physical activity ≥5 times per week; patients are advised to stop exercising if they experience pain or discomfort. 

Patients who smoke should be encouraged to stop. Evidence suggests smoking is associated with more active disease, and a significant reduction in the therapeutic effect of hydroxychloroquine.[44][96][97] Smoking cessation reduces the risk of atherosclerotic vascular disease. 

SLE has a significant impact on health-related quality of life, and has been shown to increase suicidal ideation and suicide attempts.[92][93] Literature reviews suggest that psychological interventions such as psychotherapy, cognitive behavioural therapies (CBT), psychoeducation, and mindfulness-based CBT), as adjuncts to medical therapy, improve fatigue, depression, pain, and quality of life for patients with SLE.[94][95] 

Consider – corticosteroid

Back
ONGOING
|
mucocutaneous disease
Consider – 

corticosteroid

Additional treatment recommended for SOME patients in selected patient group

Can be used when other symptom-relieving measures have failed.

Pulses of intravenous methylprednisolone are recommended to provide immediate therapeutic effect in SLE and enable the use of a lower starting dose of oral corticosteroids.[47] 

The recommended dose and route of administration depends on the type and severity of organ involvement, but for chronic maintenance treatment the dose should be minimised to <7.5 mg/day and, when possible, withdrawn.[47]

The long-term adverse effects of corticosteroid therapy are well documented, and patients should be counselled regarding risk of hypertension and atherosclerotic disease, hyperglycaemia, potential skin changes, infection, mood disorders, disorders of bone and muscle (e.g. osteoporosis, osteonecrosis, myopathy), and ophthalmological effects (e.g., cataracts, increased ocular pressure, exophthalmos). Caution is advised with corticosteroid use in patients with upper gastrointestinal symptoms, especially if also taking non-steroidal anti-inflammatory drugs.

The lowest possible dose to control symptoms should be used for the shortest period of time. Can be used in combination with hydroxychloroquine if required.[115][116]

Primary options

methylprednisolone sodium succinate: 250-1000 mg intravenously once daily for 3 days

OR

prednisolone: 5-60 mg orally once daily

More

Consider – immunosuppressant or dapsone or a retinoid

Back
ONGOING
|
mucocutaneous disease
Consider – 

immunosuppressant or dapsone or a retinoid

Additional treatment recommended for SOME patients in selected patient group

For those patients who do not respond to first-line treatment, or require high-dose corticosteroids, methotrexate, azathioprine, mycophenolate, dapsone, or a retinoid (e.g., acitretin) can be added.[47]  

Early initiation of immunosuppressive agents can expedite the tapering/discontinuation of corticosteroids.[47]

Patients taking methotrexate should have regular haematological and liver function testing. Methotrexate use may increase the risk of infection. Abnormal haematological and/or liver function results may necessitate reduction in prescribed dose.[117] Folinic acid or folic acid (depending on local guidelines) is given to counteract the folate-antagonist action of methotrexate.

Primary options

methotrexate: 7.5 mg orally/intravenously/subcutaneously once weekly on the same day of each week, increase gradually according to response, maximum 20 mg/week

OR

azathioprine: 2 mg/kg/day orally, adjust dose according to response

OR

mycophenolate mofetil: consult specialist for guidance on dose

Secondary options

acitretin: consult specialist for guidance on dose

OR

dapsone: consult specialist for guidance on dose

Consider – belimumab or rituximab

Back
ONGOING
|
mucocutaneous disease
Consider – 

belimumab or rituximab

Additional treatment recommended for SOME patients in selected patient group

Guidance suggests that belimumab and rituximab have also shown efficacy in mucocutaneous manifestations of SLE, although rituximab may be less efficacious in chronic forms of skin lupus.[47]

In the UK, the National Institute for Health and Care Excellence (NICE) recommends belimumab as an add-on treatment for patients with active autoantibody-positive SLE with high disease activity despite standard treatment, only if: high disease activity is defined as at least 1 serological biomarker (positive anti-double-stranded DNA or low component) and a SELENA-SLEDAI (Safety of Estrogen in Lupus National Assessment - Systemic Lupus Erythematosus Disease Activity Index) score of greater than or equal to 10; treatment is continued beyond 24 weeks only if the SELENA-SLEDAI score has improved by 4 points or more.[108]​

Consider pre-medication to attenuate infusion- and hypersensitivity-related reactions.

Primary options

belimumab: 10 mg/kg intravenously every 2 weeks for the first 3 doses, then every 4 weeks thereafter; 200 mg subcutaneously once weekly

More

Secondary options

rituximab: consult specialist for guidance on dose

Consider – thalidomide

Back
ONGOING
|
mucocutaneous disease
Consider – 

thalidomide

Additional treatment recommended for SOME patients in selected patient group

Thalidomide should be considered only as a rescue therapy for patients who have failed multiple previous agents due to its strict contraindication in pregnancy, the risk for irreversible polyneuropathy, and the frequent relapses on drug discontinuation.[47]

Primary options

thalidomide: consult specialist for guidance on dose

lupus nephritis

1st line – induction therapy

Back
ONGOING
|
lupus nephritis
1st line – 

induction therapy

For renal manifestations of SLE, induction therapy is required to achieve complete or partial response, followed by immunosuppression to maintain the response. An early significant drop in proteinuria (to ≤1 g/day at 6 months or ≤0.8 g/day at 12 months) is a predictor of favourable long-term renal outcome.[47] 

Mycophenolate or low-dose intravenous cyclophosphamide are recommended as initial induction treatment, as they have the best efficacy/toxicity ratio.[47][121][122] [ Cochrane Clinical Answers logo ] [Evidence C] Therapeutic regimens considered for patients at high risk for renal failure are similar, but high-dose intravenous cyclophosphamide can be used.[47]

One systematic review and meta-analysis found that mycophenolate significantly increased the level of serum complement C3 compared with cyclophosphamide.[123] Mycophenolate was also superior to cyclophosphamide with respect to secondary end points of complete remission and adverse reactions. 

Cyclophosphamide should be given with adequate fluid and mesna (a uroprotective agent) as there is a risk of uro-epithelial toxicity (e.g., haemorrhagic cystitis). Young women should be advised about the risks of amenorrhoea or premature ovarian failure with cyclophosphamide; gynaecological referral may be required for further in-depth discussion. Male patients should also be counselled regarding possible risk of infertility. The risk of amenorrhoea is lower with mycophenolate, although there are concerns about congenital malformations if it is given during pregnancy.

Calcineurin inhibitors (e.g., ciclosporin) may be considered as second-line agents for induction therapy in membranous lupus nephritis, podocytopathy, or proliferative disease with refractory nephrotic syndrome despite standard-of-care within 3 to 6 months.[47] Calcineurin inhibitors may be used alone, or in combination with mycophenolate, to treat proliferative lupus nephritis.[128][129][130][131][132][133]  

For patients with refractory nephrotic syndrome, tacrolimus may be used alone or in combination with mycophenolate, as this combination is effective in disease refractory to standard therapy.[47][134]

Monitoring serum creatinine and blood levels of patients being treated with calcineurin inhibitors is essential to avoid chronic drug toxicity.[47] 

Primary options

cyclophosphamide: consult specialist for guidance on dose

OR

mycophenolate mofetil: consult specialist for guidance on dose

Secondary options

tacrolimus: consult specialist for guidance on dose

OR

ciclosporin: consult specialist for guidance on dose

OR

mycophenolate mofetil: consult specialist for guidance on dose

and

tacrolimus: consult specialist for guidance on dose

Plus – hydroxychloroquine

Back
ONGOING
|
lupus nephritis
Plus – 

hydroxychloroquine

Treatment recommended for ALL patients in selected patient group

Continued hydroxychloroquine is associated with increased remission rates in patients initially treated with mycophenolate for lupus nephritis.[126]

Primary options

hydroxychloroquine: 200-400 mg/day orally given in 1-2 divided doses, maximum 5 mg/kg/day (base)

Plus – corticosteroid

Back
ONGOING
|
lupus nephritis
Plus – 

corticosteroid

Treatment recommended for ALL patients in selected patient group

Corticosteroids are also given as part of the induction regimen in addition to background treatment with hydroxychloroquine.[124][125]  

Initial therapy with pulse doses of intravenous methylprednisolone is encouraged.[47] 

Primary options

methylprednisolone sodium succinate: 250-1000 mg intravenously once daily for 3 days

OR

prednisolone: 5-60 mg orally once daily

More

Plus – lifestyle changes and psychological therapies

Back
ONGOING
|
lupus nephritis
Plus – 

lifestyle changes and psychological therapies

Treatment recommended for ALL patients in selected patient group

Lifestyle changes include dietary advice, smoking cessation, sun protection, exercise, and psychological therapies.

Exposure to ultraviolet light may exacerbate or induce systemic manifestations of SLE.[83] Patients with SLE should be advised to avoid excessive sun exposure and to use a broad-spectrum sunscreen.[84]

No dietary measures have been shown to alter the course of SLE. However, the late complications of premature cardiovascular disease should be borne in mind. Patients should be advised to maintain an ideal body weight for their height and reduce salt intake if hypertension due to renal disease is present. General advice includes eating at least 5 servings of fruit or vegetables per day, replacing saturated fats with monounsaturates and polyunsaturates, and increasing the amount of oily fish eaten; a diet rich in polyunsaturated fatty acids should be recommended.[85] Standard advice for the amount of alcohol per week for men and women should be given. 

SLE is associated with inadequate levels of serum vitamin D compared with the general population.[86][87][88] In patients with SLE, vitamin D supplements reduce disease activity; increase serum levels; and improve levels of inflammatory markers, fatigue, and endothelial function.[88][89][90] 

Some evidence suggests that omega-3 fatty acid supplementation may reduce SLE disease activity.[89][91]

Herbal preparations should be avoided. They can interact adversely with pharmacological agents and may cause harm.

Patients with stable SLE should be advised to avoid a sedentary lifestyle and to undertake supervised exercise.[85] In these patients, adherence to exercise guidelines should be encouraged to maintain optimum cardiovascular fitness. This should include ≥30 minutes of moderate physical activity ≥5 times per week; patients are advised to stop exercising if they experience pain or discomfort. 

Patients who smoke should be encouraged to stop. Evidence suggests smoking is associated with more active disease, and a significant reduction in the therapeutic effect of hydroxychloroquine.[44][96][97] Smoking cessation reduces the risk of atherosclerotic vascular disease. 

SLE has a significant impact on health-related quality of life, and has been shown to increase suicidal ideation and suicide attempts.[92][93] Literature reviews suggest that psychological interventions such as psychotherapy, cognitive behavioural therapies (CBT), psychoeducation, and mindfulness-based CBT), as adjuncts to medical therapy, improve fatigue, depression, pain, and quality of life for patients with SLE.[94][95] 

Plus – maintenance therapy

Back
ONGOING
|
lupus nephritis
Plus – 

maintenance therapy

Treatment recommended for ALL patients in selected patient group

Once a patient has attained complete or partial response, immunosuppression is continued to maintain the response.

Mycophenolate or azathioprine are recommended first-line for maintenance therapy, and should be used in combination with corticosteroids.[47] Either treatment can be used for maintenance therapy after induction with cyclophosphamide or mycophenolate and is more effective in preserving renal function than corticosteroids alone.[135]

Calcineurin inhibitors (e.g., tacrolimus, ciclosporin) may be considered as second-line agent for maintenance therapy in membranous lupus nephritis, podocytopathy, or proliferative disease with refractory nephrotic syndrome despite standard-of-care within 3 to 6 months.[47]

One systematic review and meta-analysis of the effect of calcineurin inhibitors for the induction and maintenance treatment of lupus nephritis found that calcineurin inhibitor treatment during the maintenance period was as effective as azathioprine treatment, with a much lower risk of adverse effects.[130] 

Monitoring serum creatinine and blood levels of patients being treated with calcineurin inhibitors to avoid chronic drug toxicity is essential.[47]

Primary options

azathioprine: consult specialist for guidance on dose

OR

mycophenolate mofetil: consult specialist for guidance on dose

Secondary options

tacrolimus: consult specialist for guidance on dose

OR

ciclosporin: consult specialist for guidance on dose

Consider – belimumab or rituximab

Back
ONGOING
|
lupus nephritis
Consider – 

belimumab or rituximab

Additional treatment recommended for SOME patients in selected patient group

Belimumab is approved for adults with lupus nephritis. It should be considered as an add-on treatment for patients who have an inadequate response to combination treatment with hydroxychloroquine and corticosteroids with or without immunosuppressive agents, defined as residual disease activity not allowing tapering of corticosteroids and/or frequent relapses.[47] 

In the UK, the National Institute for Health and Care Excellence (NICE) recommends belimumab as an add-on treatment for patients with active autoantibody-positive SLE with high disease activity despite standard treatment, only if: high disease activity is defined as at least 1 serological biomarker (positive anti-double-stranded DNA or low component) and a SELENA-SLEDAI (Safety of Estrogen in Lupus National Assessment - Systemic Lupus Erythematosus Disease Activity Index) score of greater than or equal to 10; treatment is continued beyond 24 weeks only if the SELENA-SLEDAI score has improved by 4 points or more.[108]​

In a randomised double-blind trial, significantly more patients who received belimumab plus standard therapy had a renal response (43% vs. 32%; defined as ratio of urinary protein to creatinine of 0.7 or less, an estimated glomerular filtration rate that was no worse than 20% below the pre-flare value or at least 60 mL/minute/1.73 m², and no use of rescue therapy for treatment failure) compared with standard therapy alone.[127]

Rituximab can be considered for patients with organ-threatening, refractory disease or with intolerance/contraindications to standard immunosuppressive agents.[47] 

Consider pre-medication to attenuate infusion- and hypersensitivity-related reactions.

Primary options

belimumab: 10 mg/kg intravenously every 2 weeks for the first 3 doses, then every 4 weeks thereafter; 400 mg subcutaneously once weekly for the first 4 doses, then 200 mg once weekly thereafter

More

Secondary options

rituximab: consult specialist for guidance on dose

neuropsychiatric lupus

1st line – immunosuppressant

Back
ONGOING
|
neuropsychiatric lupus
|
inflammatory only
1st line – 

immunosuppressant

Treatment of SLE-related neuropsychiatric disease includes immunosuppressive agents and corticosteroids for manifestations considered to reflect an inflammatory process.[47] The choice of immunosuppressive agent (e.g., azathioprine, mycophenolate, methotrexate) will depend on individual cases, as the neuropsychiatric manifestations can be varied.  

Distinction between the two pathophysiological processes (inflammatory and atherothrombotic/antiphospholipid-related manifestations) may be difficult in practice. The two processes could co-exist in the same patient. 

Cyclophosphamide can be used for severe organ-threatening or life-threatening SLE as well as rescue therapy in patients not responding to other immunosuppressive agents.[47]

Primary options

methotrexate: 7.5 mg orally/intravenously/subcutaneously once weekly on the same day of each week, increase gradually according to response, maximum 20 mg/week

OR

azathioprine: 2 mg/kg/day orally, adjust dose according to response

OR

mycophenolate mofetil: consult specialist for guidance on dose

Secondary options

cyclophosphamide: consult specialist for guidance on dose

Plus – corticosteroid

Back
ONGOING
|
neuropsychiatric lupus
|
inflammatory only
Plus – 

corticosteroid

Treatment recommended for ALL patients in selected patient group

Treatment of SLE-related neuropsychiatric disease includes corticosteroids for manifestations considered to reflect an inflammatory process.[47]

Initial therapy with pulse doses of intravenous methylprednisolone is encouraged.[47] 

Primary options

methylprednisolone sodium succinate: 250-1000 mg intravenously once daily for 3 days

OR

prednisolone: 5-60 mg orally once daily

More

Plus – lifestyle changes and psychological therapies

Back
ONGOING
|
neuropsychiatric lupus
|
inflammatory only
Plus – 

lifestyle changes and psychological therapies

Treatment recommended for ALL patients in selected patient group

Lifestyle changes include dietary advice, smoking cessation, sun protection, exercise, and psychological therapies.

Exposure to ultraviolet light may exacerbate or induce systemic manifestations of SLE.[83] Patients with SLE should be advised to avoid excessive sun exposure and to use a broad-spectrum sunscreen.[84]

No dietary measures have been shown to alter the course of SLE. However, the late complications of premature cardiovascular disease should be borne in mind. Patients should be advised to maintain an ideal body weight for their height and reduce salt intake if hypertension due to renal disease is present. General advice includes eating at least 5 servings of fruit or vegetables per day, replacing saturated fats with monounsaturates and polyunsaturates, and increasing the amount of oily fish eaten; a diet rich in polyunsaturated fatty acids should be recommended.[85] Standard advice for the amount of alcohol per week for men and women should be given. 

SLE is associated with inadequate levels of serum vitamin D compared with the general population.[86][87][88] In patients with SLE, vitamin D supplements reduce disease activity; increase serum levels; and improve levels of inflammatory markers, fatigue, and endothelial function.[88][89][90] 

Some evidence suggests that omega-3 fatty acid supplementation may reduce SLE disease activity.[89][91]

Herbal preparations should be avoided. They can interact adversely with pharmacological agents and may cause harm.

Patients with stable SLE should be advised to avoid a sedentary lifestyle and to undertake supervised exercise.[85] In these patients, adherence to exercise guidelines should be encouraged to maintain optimum cardiovascular fitness. This should include ≥30 minutes of moderate physical activity ≥5 times per week; patients are advised to stop exercising if they experience pain or discomfort. 

Patients who smoke should be encouraged to stop. Evidence suggests smoking is associated with more active disease, and a significant reduction in the therapeutic effect of hydroxychloroquine.[44][96][97] Smoking cessation reduces the risk of atherosclerotic vascular disease. 

SLE has a significant impact on health-related quality of life, and has been shown to increase suicidal ideation and suicide attempts.[92][93] Literature reviews suggest that psychological interventions such as psychotherapy, cognitive behavioural therapies (CBT), psychoeducation, and mindfulness-based CBT), as adjuncts to medical therapy, improve fatigue, depression, pain, and quality of life for patients with SLE.[94][95] 

Consider – rituximab

Back
ONGOING
|
neuropsychiatric lupus
|
inflammatory only
Consider – 

rituximab

Additional treatment recommended for SOME patients in selected patient group

Rituximab can be considered for patients with organ-threatening disease refractory or with intolerance/contraindications to standard immunosuppressive agents. Evidence of benefit in severe refractory neuropsychiatric SLE is limited to case reports.

Primary options

rituximab: consult specialist for guidance on dose

Consider – intravenous immunoglobulin (IVIG)

Back
ONGOING
|
neuropsychiatric lupus
|
inflammatory only
Consider – 

intravenous immunoglobulin (IVIG)

Additional treatment recommended for SOME patients in selected patient group

IVIG may be used as adjunctive therapy when initial treatment is inadequate, but the quality of evidence supporting its use is poor (small cohort studies).[136] IVIG can be effective in the treatment of SLE-associated peripheral neuropathies. 

Primary options

normal immunoglobulin human: consult specialist for guidance on dose

Consider – plasmapheresis

Back
ONGOING
|
neuropsychiatric lupus
|
inflammatory only
Consider – 

plasmapheresis

Additional treatment recommended for SOME patients in selected patient group

Plasmapheresis may also be considered as an adjunctive treatment.[136] The aim of the treatment is to remove circulating auto-antibodies. Recommended if there are clinical and investigative findings consistent with cerebral vasculitis, and may be used when earlier treatments are inadequate. Data from large randomised trials are lacking. 

Consider – targeted symptomatic pharmacotherapy

Back
ONGOING
|
neuropsychiatric lupus
|
inflammatory only
Consider – 

targeted symptomatic pharmacotherapy

Additional treatment recommended for SOME patients in selected patient group

Targeted symptomatic therapy is indicated according to the type of manifestation.[47] 

Antidepressants, anticonvulsants, antipsychotics, or antimigraine therapies should be prescribed on the advice of relevant specialists on an individual patient basis.

1st line – antiplatelet agent or anticoagulation

Back
ONGOING
|
neuropsychiatric lupus
|
atherothrombotic/antiphospholipid-related manifestations only
1st line – 

antiplatelet agent or anticoagulation

Treatment of SLE-related neuropsychiatric disease includes an antiplatelet agent/anticoagulant for atherothrombotic/antiphospholipid-related manifestations.

Distinction between the two pathophysiological processes (inflammatory and atherothrombotic/antiphospholipid-related manifestations) may be difficult in practice. The two processes could co-exist in the same patient.

Consult a haematologist for guidance on specific antiplatelet agent/anticoagulant treatment regimens.

Plus – lifestyle changes and psychological therapies

Back
ONGOING
|
neuropsychiatric lupus
|
atherothrombotic/antiphospholipid-related manifestations only
Plus – 

lifestyle changes and psychological therapies

Treatment recommended for ALL patients in selected patient group

Lifestyle changes include dietary advice, smoking cessation, sun protection, exercise, and psychological therapies.

Exposure to ultraviolet light may exacerbate or induce systemic manifestations of SLE.[83] Patients with SLE should be advised to avoid excessive sun exposure and to use a broad-spectrum sunscreen.[84]

No dietary measures have been shown to alter the course of SLE. However, the late complications of premature cardiovascular disease should be borne in mind. Patients should be advised to maintain an ideal body weight for their height and reduce salt intake if hypertension due to renal disease is present. General advice includes eating at least 5 servings of fruit or vegetables per day, replacing saturated fats with monounsaturates and polyunsaturates, and increasing the amount of oily fish eaten; a diet rich in polyunsaturated fatty acids should be recommended.[85] Standard advice for the amount of alcohol per week for men and women should be given. 

SLE is associated with inadequate levels of serum vitamin D compared with the general population.[86][87][88] In patients with SLE, vitamin D supplements reduce disease activity; increase serum levels; and improve levels of inflammatory markers, fatigue, and endothelial function.[88][89][90] 

Some evidence suggests that omega-3 fatty acid supplementation may reduce SLE disease activity.[89][91]

Herbal preparations should be avoided. They can interact adversely with pharmacological agents and may cause harm.

Patients with stable SLE should be advised to avoid a sedentary lifestyle and to undertake supervised exercise.[85] In these patients, adherence to exercise guidelines should be encouraged to maintain optimum cardiovascular fitness. This should include ≥30 minutes of moderate physical activity ≥5 times per week; patients are advised to stop exercising if they experience pain or discomfort. 

Patients who smoke should be encouraged to stop. Evidence suggests smoking is associated with more active disease, and a significant reduction in the therapeutic effect of hydroxychloroquine.[44][96][97] Smoking cessation reduces the risk of atherosclerotic vascular disease. 

SLE has a significant impact on health-related quality of life, and has been shown to increase suicidal ideation and suicide attempts.[92][93] Literature reviews suggest that psychological interventions such as psychotherapy, cognitive behavioural therapies (CBT), psychoeducation, and mindfulness-based CBT), as adjuncts to medical therapy, improve fatigue, depression, pain, and quality of life for patients with SLE.[94][95] 

Consider – immunosuppressant

Back
ONGOING
|
neuropsychiatric lupus
|
atherothrombotic/antiphospholipid-related manifestations only
Consider – 

immunosuppressant

Additional treatment recommended for SOME patients in selected patient group

Patients with SLE with cerebrovascular disease should be managed like the general population in the acute phase; in addition to controlling extra-central nervous system lupus activity, immunosuppressive therapy may be considered in the absence of antiphospholipid antibodies and other atherosclerotic risk factors or in recurrent cerebrovascular events.[47] Consult a specialist for guidance on choice of regimen.

Consider – intravenous immunoglobulin (IVIG)

Back
ONGOING
|
neuropsychiatric lupus
|
atherothrombotic/antiphospholipid-related manifestations only
Consider – 

intravenous immunoglobulin (IVIG)

Additional treatment recommended for SOME patients in selected patient group

IVIG administration has also been used in patients with SLE. The quality of evidence for use of IVIG is poor (entirely from small cohort studies). However, it may be used as an adjunctive therapy when initial treatment is inadequate.[136] IVIG can be effective in the treatment of SLE-associated peripheral neuropathies.

Primary options

normal immunoglobulin human: consult specialist for guidance on dose

Consider – plasmapheresis

Back
ONGOING
|
neuropsychiatric lupus
|
atherothrombotic/antiphospholipid-related manifestations only
Consider – 

plasmapheresis

Additional treatment recommended for SOME patients in selected patient group

Plasmapheresis may also be considered as an adjunctive treatment.[136] The aim of the treatment is to remove circulating auto-antibodies. Recommended if there are clinical and investigatory findings consistent with cerebral vasculitis, and may be used when earlier treatments are inadequate. Data from large randomised trials are lacking.

Consider – targeted symptomatic pharmacotherapy

Back
ONGOING
|
neuropsychiatric lupus
|
atherothrombotic/antiphospholipid-related manifestations only
Consider – 

targeted symptomatic pharmacotherapy

Additional treatment recommended for SOME patients in selected patient group

Targeted symptomatic therapy is indicated according to the type of manifestation.[47] 

Antidepressants, anticonvulsants, antipsychotics, or antimigraine therapies should be prescribed on the advice of relevant specialist on an individual patient basis.

1st line – immunosuppressant

Back
ONGOING
|
neuropsychiatric lupus
|
inflammatory and atherothrombotic/antiphospholipid-related manifestations
1st line – 

immunosuppressant

The combination of an immunosuppressive agent and antiplatelet/anticoagulant therapy may be considered in these patients.[47] Consult a specialist for guidance on choice of immunosuppressant regimen. 

Distinction between the two pathophysiological processes (inflammatory and atherothrombotic/antiphospholipid-related manifestations) may be difficult in practice. The two processes could co-exist in the same patient.

Plus – antiplatelet agent or anticoagulation

Back
ONGOING
|
neuropsychiatric lupus
|
inflammatory and atherothrombotic/antiphospholipid-related manifestations
Plus – 

antiplatelet agent or anticoagulation

Treatment recommended for ALL patients in selected patient group

Treatment of SLE-related neuropsychiatric disease includes an antiplatelet agent/anticoagulant for atherothrombotic/antiphospholipid-related manifestations. Consult a haematologist for guidance on specific antiplatelet/anticoagulant treatment regimens.

Plus – lifestyle changes and psychological therapies

Back
ONGOING
|
neuropsychiatric lupus
|
inflammatory and atherothrombotic/antiphospholipid-related manifestations
Plus – 

lifestyle changes and psychological therapies

Treatment recommended for ALL patients in selected patient group

Lifestyle changes include dietary advice, smoking cessation, sun protection, exercise, and psychological therapies.

Exposure to ultraviolet light may exacerbate or induce systemic manifestations of SLE.[83] Patients with SLE should be advised to avoid excessive sun exposure and to use a broad-spectrum sunscreen.[84]

No dietary measures have been shown to alter the course of SLE. However, the late complications of premature cardiovascular disease should be borne in mind. Patients should be advised to maintain an ideal body weight for their height and reduce salt intake if hypertension due to renal disease is present. General advice includes eating at least 5 servings of fruit or vegetables per day, replacing saturated fats with monounsaturates and polyunsaturates, and increasing the amount of oily fish eaten; a diet rich in polyunsaturated fatty acids should be recommended.[85] Standard advice for the amount of alcohol per week for men and women should be given. 

SLE is associated with inadequate levels of serum vitamin D compared with the general population.[86][87][88] In patients with SLE, vitamin D supplements reduce disease activity; increase serum levels; and improve levels of inflammatory markers, fatigue, and endothelial function.[88][89][90] 

Some evidence suggests that omega-3 fatty acid supplementation may reduce SLE disease activity.[89][91]

Herbal preparations should be avoided. They can interact adversely with pharmacological agents and may cause harm.

Patients with stable SLE should be advised to avoid a sedentary lifestyle and to undertake supervised exercise.[85] In these patients, adherence to exercise guidelines should be encouraged to maintain optimum cardiovascular fitness. This should include ≥30 minutes of moderate physical activity ≥5 times per week; patients are advised to stop exercising if they experience pain or discomfort. 

Patients who smoke should be encouraged to stop. Evidence suggests smoking is associated with more active disease, and a significant reduction in the therapeutic effect of hydroxychloroquine.[44][96][97] Smoking cessation reduces the risk of atherosclerotic vascular disease. 

SLE has a significant impact on health-related quality of life, and has been shown to increase suicidal ideation and suicide attempts.[92][93] Literature reviews suggest that psychological interventions such as psychotherapy, cognitive behavioural therapies (CBT), psychoeducation, and mindfulness-based CBT), as adjuncts to medical therapy, improve fatigue, depression, pain, and quality of life for patients with SLE.[94][95] 

Consider – rituximab

Back
ONGOING
|
neuropsychiatric lupus
|
inflammatory and atherothrombotic/antiphospholipid-related manifestations
Consider – 

rituximab

Additional treatment recommended for SOME patients in selected patient group

Rituximab can be considered for patients with organ-threatening, refractory disease or with intolerance/contraindications to standard immunosuppressive agents. Evidence of benefit in severe refractory neuropsychiatric SLE is limited to case reports.

Primary options

rituximab: consult specialist for guidance on dose

Consider – intravenous immunoglobulin (IVIG)

Back
ONGOING
|
neuropsychiatric lupus
|
inflammatory and atherothrombotic/antiphospholipid-related manifestations
Consider – 

intravenous immunoglobulin (IVIG)

Additional treatment recommended for SOME patients in selected patient group

IVIG may be used as adjunctive therapy when initial treatment is inadequate, but the quality of evidence supporting its use is poor (small cohort studies).[136] IVIG can be effective in the treatment of SLE-associated peripheral neuropathies. 

Primary options

normal immunoglobulin human: consult specialist for guidance on dose

Consider – plasmapheresis

Back
ONGOING
|
neuropsychiatric lupus
|
inflammatory and atherothrombotic/antiphospholipid-related manifestations
Consider – 

plasmapheresis

Additional treatment recommended for SOME patients in selected patient group

Plasmapheresis may also be considered as an adjunctive treatment.[136] The aim of the treatment is to remove circulating auto-antibodies.  

Recommended if there are clinical and investigative findings consistent with cerebral vasculitis, and may be used when earlier treatments are inadequate. Data from large randomised trials are lacking.

Consider – targeted symptomatic pharmacotherapy

Back
ONGOING
|
neuropsychiatric lupus
|
inflammatory and atherothrombotic/antiphospholipid-related manifestations
Consider – 

targeted symptomatic pharmacotherapy

Additional treatment recommended for SOME patients in selected patient group

Targeted symptomatic therapy is indicated according to the type of manifestation.[47]

Antidepressants, anticonvulsants, antipsychotics, or antimigraine therapies should be prescribed on the advice of relevant specialist on an individual patient basis.

haematological manifestations

1st line – immunosuppressant

Back
ONGOING
|
haematological manifestations
1st line – 

immunosuppressant

Haematological manifestations that require anti-inflammatory/immunosuppressive treatment in patients with SLE include thrombocytopenia and autoimmune haemolytic anaemia.[47] 

Treatment of significant lupus thrombocytopenia (platelet count below 30,000/mm³) and autoimmune haemolytic anaemia consists of an immunosuppressive agent (e.g., azathioprine, mycophenolate, ciclosporin) as a corticosteroid-sparing agent, in combination with a corticosteroid.[47]

Primary options

azathioprine: 2 mg/kg/day orally, adjust dose according to response

OR

mycophenolate mofetil: consult specialist for guidance on dose

OR

ciclosporin: consult specialist for guidance on dose

Plus – corticosteroid

Back
ONGOING
|
haematological manifestations
Plus – 

corticosteroid

Treatment recommended for ALL patients in selected patient group

Treatment of significant lupus thrombocytopenia (platelet count below 30,000/mm³) and autoimmune haemolytic anaemia consists of moderate/high doses of corticosteroids. Initial therapy with pulse doses of intravenous methylprednisolone is encouraged.[47]

Primary options

methylprednisolone sodium succinate: 250-1000 mg intravenously once daily for 3 days

OR

prednisolone: 5-60 mg orally once daily

More

Plus – lifestyle changes and psychological therapies

Back
ONGOING
|
haematological manifestations
Plus – 

lifestyle changes and psychological therapies

Treatment recommended for ALL patients in selected patient group

Lifestyle changes include dietary advice, smoking cessation, sun protection, exercise, and psychological therapies.

Exposure to ultraviolet light may exacerbate or induce systemic manifestations of SLE.[83] Patients with SLE should be advised to avoid excessive sun exposure and to use a broad-spectrum sunscreen.[84]

No dietary measures have been shown to alter the course of SLE. However, the late complications of premature cardiovascular disease should be borne in mind. Patients should be advised to maintain an ideal body weight for their height and reduce salt intake if hypertension due to renal disease is present. General advice includes eating at least 5 servings of fruit or vegetables per day, replacing saturated fats with monounsaturates and polyunsaturates, and increasing the amount of oily fish eaten; a diet rich in polyunsaturated fatty acids should be recommended.[85] Standard advice for the amount of alcohol per week for men and women should be given. 

SLE is associated with inadequate levels of serum vitamin D compared with the general population.[86][87][88] In patients with SLE, vitamin D supplements reduce disease activity; increase serum levels; and improve levels of inflammatory markers, fatigue, and endothelial function.[88][89][90] 

Some evidence suggests that omega-3 fatty acid supplementation may reduce SLE disease activity.[89][91]

Herbal preparations should be avoided. They can interact adversely with pharmacological agents and may cause harm.

Patients with stable SLE should be advised to avoid a sedentary lifestyle and to undertake supervised exercise.[85] In these patients, adherence to exercise guidelines should be encouraged to maintain optimum cardiovascular fitness. This should include ≥30 minutes of moderate physical activity ≥5 times per week; patients are advised to stop exercising if they experience pain or discomfort. 

Patients who smoke should be encouraged to stop. Evidence suggests smoking is associated with more active disease, and a significant reduction in the therapeutic effect of hydroxychloroquine.[44][96][97] Smoking cessation reduces the risk of atherosclerotic vascular disease. 

SLE has a significant impact on health-related quality of life, and has been shown to increase suicidal ideation and suicide attempts.[92][93] Literature reviews suggest that psychological interventions such as psychotherapy, cognitive behavioural therapies (CBT), psychoeducation, and mindfulness-based CBT), as adjuncts to medical therapy, improve fatigue, depression, pain, and quality of life for patients with SLE.[94][95] 

Consider – intravenous immunoglobulin (IVIG)

Back
ONGOING
|
haematological manifestations
Consider – 

intravenous immunoglobulin (IVIG)

Additional treatment recommended for SOME patients in selected patient group

IVIG may be considered in the acute phase, in cases of inadequate response to high-dose corticosteroids or to avoid corticosteroid-related infectious complications.[47]

Primary options

normal immunoglobulin human: consult specialist for guidance on dose

2nd line – rituximab or cyclophosphamide

Back
ONGOING
|
haematological manifestations
2nd line – 

rituximab or cyclophosphamide

Cyclophosphamide should be considered in patients with no response to corticosteroids or patients who have relapsed.[47]

Rituximab can be considered for patients with organ-threatening, refractory disease or with intolerance/contraindications to standard immunosuppressive agents.[47] 

Primary options

rituximab: consult specialist for guidance on dose

OR

cyclophosphamide: consult specialist for guidance on dose

Consider – lifestyle changes and psychological therapies

Back
ONGOING
|
haematological manifestations
Consider – 

lifestyle changes and psychological therapies

Additional treatment recommended for SOME patients in selected patient group

Lifestyle changes include dietary advice, smoking cessation, sun protection, exercise, and psychological therapies.

Exposure to ultraviolet light may exacerbate or induce systemic manifestations of SLE.[83] Patients with SLE should be advised to avoid excessive sun exposure and to use a broad-spectrum sunscreen.[84]

No dietary measures have been shown to alter the course of SLE. However, the late complications of premature cardiovascular disease should be borne in mind. Patients should be advised to maintain an ideal body weight for their height and reduce salt intake if hypertension due to renal disease is present. General advice includes eating at least 5 servings of fruit or vegetables per day, replacing saturated fats with monounsaturates and polyunsaturates, and increasing the amount of oily fish eaten; a diet rich in polyunsaturated fatty acids should be recommended.[85] Standard advice for the amount of alcohol per week for men and women should be given. 

SLE is associated with inadequate levels of serum vitamin D compared with the general population.[86][87][88] In patients with SLE, vitamin D supplements reduce disease activity; increase serum levels; and improve levels of inflammatory markers, fatigue, and endothelial function.[88][89][90] 

Some evidence suggests that omega-3 fatty acid supplementation may reduce SLE disease activity.[89][91]

Herbal preparations should be avoided. They can interact adversely with pharmacological agents and may cause harm.

Patients with stable SLE should be advised to avoid a sedentary lifestyle and to undertake supervised exercise.[85] In these patients, adherence to exercise guidelines should be encouraged to maintain optimum cardiovascular fitness. This should include ≥30 minutes of moderate physical activity ≥5 times per week; patients are advised to stop exercising if they experience pain or discomfort. 

Patients who smoke should be encouraged to stop. Evidence suggests smoking is associated with more active disease, and a significant reduction in the therapeutic effect of hydroxychloroquine.[44][96][97] Smoking cessation reduces the risk of atherosclerotic vascular disease. 

SLE has a significant impact on health-related quality of life, and has been shown to increase suicidal ideation and suicide attempts.[92][93] Literature reviews suggest that psychological interventions such as psychotherapy, cognitive behavioural therapies (CBT), psychoeducation, and mindfulness-based CBT), as adjuncts to medical therapy, improve fatigue, depression, pain, and quality of life for patients with SLE.[94][95] 

3rd line – thrombopoietin agonist or splenectomy

Back
ONGOING
|
haematological manifestations
3rd line – 

thrombopoietin agonist or splenectomy

Thrombopoietin agonists (e.g., eltrombopag, romiplostim) or splenectomy should be reserved as last options.[47]

Primary options

eltrombopag: consult specialist for guidance on dose

OR

romiplostim: consult specialist for guidance on dose

Consider – lifestyle changes and psychological therapies

Back
ONGOING
|
haematological manifestations
Consider – 

lifestyle changes and psychological therapies

Additional treatment recommended for SOME patients in selected patient group

Lifestyle changes include dietary advice, smoking cessation, sun protection, exercise, and psychological therapies.

Exposure to ultraviolet light may exacerbate or induce systemic manifestations of SLE.[83] Patients with SLE should be advised to avoid excessive sun exposure and to use a broad-spectrum sunscreen.[84]

No dietary measures have been shown to alter the course of SLE. However, the late complications of premature cardiovascular disease should be borne in mind. Patients should be advised to maintain an ideal body weight for their height and reduce salt intake if hypertension due to renal disease is present. General advice includes eating at least 5 servings of fruit or vegetables per day, replacing saturated fats with monounsaturates and polyunsaturates, and increasing the amount of oily fish eaten; a diet rich in polyunsaturated fatty acids should be recommended.[85] Standard advice for the amount of alcohol per week for men and women should be given. 

SLE is associated with inadequate levels of serum vitamin D compared with the general population.[86][87][88] In patients with SLE, vitamin D supplements reduce disease activity; increase serum levels; and improve levels of inflammatory markers, fatigue, and endothelial function.[88][89][90] 

Some evidence suggests that omega-3 fatty acid supplementation may reduce SLE disease activity.[89][91]

Herbal preparations should be avoided. They can interact adversely with pharmacological agents and may cause harm.

Patients with stable SLE should be advised to avoid a sedentary lifestyle and to undertake supervised exercise.[85] In these patients, adherence to exercise guidelines should be encouraged to maintain optimum cardiovascular fitness. This should include ≥30 minutes of moderate physical activity ≥5 times per week; patients are advised to stop exercising if they experience pain or discomfort. 

Patients who smoke should be encouraged to stop. Evidence suggests smoking is associated with more active disease, and a significant reduction in the therapeutic effect of hydroxychloroquine.[44][96][97] Smoking cessation reduces the risk of atherosclerotic vascular disease. 

SLE has a significant impact on health-related quality of life, and has been shown to increase suicidal ideation and suicide attempts.[92][93] Literature reviews suggest that psychological interventions such as psychotherapy, cognitive behavioural therapies (CBT), psychoeducation, and mindfulness-based CBT), as adjuncts to medical therapy, improve fatigue, depression, pain, and quality of life for patients with SLE.[94][95] 

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