Treatment for Systemic Lupus Erythematosus (SLE)
Hydroxychloroquine is recommended for all patients with SLE at a dose not exceeding 5 mg/kg real body weight as the cornerstone of therapy, with additional immunomodulatory agents and glucocorticoids tailored to disease severity and organ involvement. 1, 2
First-Line Treatment
- Hydroxychloroquine (HCQ) should be prescribed for all SLE patients without contraindications, serving as the backbone of treatment due to its ability to reduce disease activity, prevent flares, and improve survival 1, 2, 3
- The recommended dose of HCQ is not to exceed 5 mg/kg real body weight to minimize risk of retinal toxicity 1, 2, 4
- Regular ophthalmological screening should be performed at baseline, after 5 years, and yearly thereafter 5, 2
- Glucocorticoids (GC) can be used at doses appropriate to the severity of organ involvement 1, 2:
- During chronic maintenance treatment, glucocorticoids should be minimized to less than 7.5 mg/day (prednisone equivalent) and, when possible, withdrawn completely to prevent organ damage 1, 6, 7
Second-Line Treatment
- Immunomodulatory/immunosuppressive agents should be added for patients not responding to HCQ alone or to facilitate glucocorticoid tapering 1, 2:
- Methotrexate: particularly effective for skin and joint manifestations 2
- Azathioprine: suitable for maintenance therapy, particularly in women contemplating pregnancy 2
- Mycophenolate mofetil: effective for renal and non-renal manifestations (except neuropsychiatric disease) 5, 2
- Cyclophosphamide: reserved for severe organ-threatening disease (especially renal, cardiopulmonary, or neuropsychiatric) 5, 2
Biological Therapies
- For persistently active or flaring disease despite standard therapy, add-on belimumab should be considered 1, 6, 2
- Rituximab may be considered in organ-threatening, refractory disease 1, 6, 5, 2
Organ-Specific Treatment Approaches
Lupus Nephritis
- Kidney biopsy is essential for diagnosis and treatment planning 2
- Induction therapy options include mycophenolate mofetil and cyclophosphamide 5, 2
- Maintenance therapy includes mycophenolate mofetil or azathioprine 2
Neuropsychiatric Lupus
- Treatment depends on the underlying pathophysiological mechanism 2:
Hematological Manifestations
- For significant thrombocytopenia: moderate/high doses of glucocorticoids in combination with immunosuppressive agents 2
- For refractory cases: rituximab or cyclophosphamide 2
Monitoring and Prevention of Complications
- Regular assessment of disease activity using validated indices is recommended 6, 5, 2
- Monitor complement levels and anti-dsDNA antibodies to assess disease activity 6, 5, 2
- Screen for infections, which are a common complication of immunosuppressive therapy 6, 5, 2
- Assess and minimize risk factors for comorbidities, including atherosclerosis, hypertension, dyslipidemias, diabetes, and osteoporosis 2
- In patients with antiphospholipid antibodies, low-dose aspirin may be considered for primary prevention of thrombosis 2
Treatment Goals
- Achieve remission or low disease activity in all organ systems 1, 6, 2
- Prevent disease flares 1, 2, 8
- Minimize glucocorticoid exposure to reduce adverse effects 1, 6, 2, 7
- Improve long-term survival and quality of life 6, 2, 3
Clinical Pearls and Pitfalls
- Discontinuation of hydroxychloroquine significantly increases the risk of disease flares (2.5 times higher) 8
- Early initiation of immunomodulatory agents can expedite the tapering/discontinuation of glucocorticoids 1, 6
- Patients with higher blood concentrations of hydroxychloroquine tend to have less active disease 3, 9
- Pulse methylprednisolone is more effective and less toxic than increasing oral doses of prednisone for moderate-severe flares 7