Treatment of Systemic Lupus Erythematosus (SLE)

Foundation Therapy (All Patients)

The American College of Rheumatology recommends hydroxychloroquine as the cornerstone of therapy for all SLE patients, reducing disease activity, preventing flares, and improving survival 1, 2
Hydroxychloroquine is mandatory for all SLE patients unless contraindicated, at a dose not exceeding 5 mg/kg of actual body weight per day (typically 200-400 mg daily) 1, 2
Ophthalmological screening is required at baseline, after 5 years of therapy, and yearly thereafter to monitor for retinal toxicity 2
Low-dose glucocorticoids (prednisone ≤7.5 mg/day or equivalent) can be added when clinically indicated, but the goal is to minimize to <7.5 mg/day and withdraw when possible to prevent organ damage 1, 2, 3
Photoprotection with sunscreens should be used to prevent cutaneous flares 2
Low-dose aspirin should be considered for patients with antiphospholipid antibodies, those receiving corticosteroids, or those with cardiovascular risk factors 2
Calcium and vitamin D supplementation is indicated for all patients on long-term glucocorticoids 2, 4

For mild disease, start with hydroxychloroquine plus low-dose glucocorticoids (if needed) 3, 5
For acute flares, pulses of intravenous methylprednisolone (500-1000 mg) provide immediate effect and enable lower starting doses of oral glucocorticoids 2
Methotrexate is the preferred first choice due to cost and availability for musculoskeletal and skin manifestations 1, 3, 5
Belimumab should be added for patients with inadequate response to standard therapy (hydroxychloroquine + glucocorticoids ± immunosuppressants) 1, 2

Kidney biopsy is essential before initiating therapy to confirm diagnosis and guide treatment planning 1, 2
Mycophenolate mofetil or low-dose intravenous cyclophosphamide are the first-line options for induction therapy 1, 2, 3
Mycophenolate mofetil or azathioprine should be used for long-term maintenance 1, 2

Belimumab should be considered when standard therapy (hydroxychloroquine + glucocorticoids ± immunosuppressants) is inadequate 1, 2
Rituximab can be considered for organ-threatening disease refractory to or with intolerance/contraindications to standard immunosuppressive agents 1, 2
Anifrolumab is FDA-approved for moderate-to-severe extrarenal SLE 2
Voclosporin is FDA-approved for lupus nephritis 2

Use validated activity indices (SLEDAI, BILAG) at each visit 2
Monitor anti-dsDNA, C3, C4, complete blood count, creatinine, proteinuria, and urine sediment regularly 2
SLE patients have a 5-fold increased mortality risk compared to the general population 2, 4
Screen for infections, cardiovascular disease, hypertension, diabetes, dyslipidemia, atherosclerosis, osteoporosis, avascular bone necrosis, and malignancies (especially non-Hodgkin lymphoma, lung cancer, hepatobiliary cancer) 2, 4

Hydroxychloroquine, azathioprine, prednisolone, and low-dose aspirin are safe medications during pregnancy 2
Mycophenolate mofetil, cyclophosphamide, and methotrexate must be avoided during pregnancy 2
Patients with lupus nephritis and antiphospholipid antibodies have higher risk of preeclampsia and require closer monitoring 6

Never withhold hydroxychloroquine unless there is a clear contraindication—non-adherence is associated with higher flare rates and mortality 2
Avoid prolonged high-dose glucocorticoids (>7.5 mg/day prednisone equivalent) as they increase irreversible organ damage risk 1, 2
Do not delay immunosuppressive therapy in organ-threatening disease—early aggressive treatment prevents irreversible damage 2, 3
Always perform kidney biopsy before treating lupus nephritis—treatment decisions depend on histological classification 1, 2

The European League Against Rheumatism recommends screening for HIV, HCV/HBV, tuberculosis, and CMV before immunosuppression, and vaccination with inactivated vaccines such as influenza and pneumococcus, especially when SLE is inactive 7
Infection risk assessment should include monitoring for severe neutropenia, severe lymphopenia, and low IgG levels 7

The European League Against Rheumatism suggests non-pharmacological management should be tailored, person-centered, and participatory, including patient education and support, physical exercise programs, smoking cessation interventions, avoidance of cold exposure, and psychosocial interventions 8

All patients should be assessed for adequate calcium and vitamin D intake, regular exercise, and smoking habits, with screening and follow-up according to existing guidelines for postmenopausal women and patients on steroids 7

Cancer screening is recommended according to guidelines for the general population, including cervical smear tests 7