From the Guidelines
Systemic Lupus Erythematosus (SLE) is diagnosed using specific clinical and laboratory criteria, and treatment depends on disease severity and organ involvement, with the goal of controlling inflammation, preventing organ damage, and improving quality of life while minimizing medication side effects. The diagnostic criteria for SLE include at least 4 out of 11 clinical and laboratory findings, such as malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neurologic disorder, hematologic disorder, immunologic disorder, and positive ANA 1.
Treatment Options
Treatment options for SLE vary depending on the severity of the disease and the organs involved. For mild SLE, hydroxychloroquine (200-400mg daily) is often used as the foundation of therapy, combined with NSAIDs for pain and inflammation 1. Corticosteroids like prednisone (5-60mg daily) are used for disease flares, with the goal of tapering to the lowest effective dose. For moderate to severe disease, immunosuppressants such as methotrexate (7.5-25mg weekly), azathioprine (50-150mg daily), mycophenolate mofetil (1-3g daily), or cyclophosphamide (for severe nephritis) may be added 1. Biologics like belimumab (10mg/kg IV monthly) may be used for patients with active disease despite standard therapy 1.
Lifestyle Modifications
Lifestyle modifications are essential for managing SLE, including sun protection, regular exercise, and smoking cessation 1. Patients should also receive regular monitoring of disease activity, medication side effects, and preventive care, including vaccinations, bone density testing, and cardiovascular risk assessment 1.
Recent Updates
Recent updates in the management of SLE include the introduction of new drugs, such as anifrolumab, an anti-type 1 interferon receptor inhibitor, which was approved in 2021 for the treatment of moderate-to-severe extrarenal SLE 1. The 2019 update of the EULAR recommendations for the management of SLE also provides guidance on the use of hydroxychloroquine, glucocorticoids, and immunomodulatory agents, as well as the management of specific organ manifestations, such as cutaneous, neuropsychiatric, hematological, and renal disease 1.
Key Principles
Key principles in the management of SLE include individualized treatment, patient involvement, and a focus on reaching and maintaining remission or low-disease activity, as well as preventing organ damage and improving quality of life 1. Treatment should be tailored to the specific needs of each patient, taking into account their disease severity, organ involvement, and other factors, such as cost and availability of medications 1.
From the FDA Drug Label
Hydroxychloroquine sulfate is indicated for the treatment of chronic discoid lupus erythematosus and systemic lupus erythematosus in adults.
The diagnostic criteria for Systemic Lupus Erythematosus (SLE) are not explicitly stated in the drug label. However, treatment options for SLE include hydroxychloroquine sulfate. Key points about the treatment of SLE with hydroxychloroquine sulfate:
- It is indicated for the treatment of SLE in adults.
- The label does not provide information on the diagnostic criteria for SLE. 2
From the Research
Diagnostic Criteria for SLE
- The diagnosis of SLE is a clinical diagnosis that is made after excluding alternative diagnoses, as there are no uniformly accepted diagnostic criteria for SLE 3.
- The 2019 European Alliance of Associations for Rheumatology (formerly the European League Against Rheumatism)/American College of Rheumatology classification criteria are an estimated 96.1% sensitive and 93.4% specific for SLE, and include both clinical factors and immunologic measures 4.
- Clinical factors that may be indicative of SLE include fever, cytopenia, rash, arthritis, and proteinuria, which may be indicative of lupus nephritis 4.
- Immunologic measures that may be indicative of SLE include SLE-specific autoantibodies, such as anti-dsDNA, anti-SS(A), anti-SS(B), anti-Smith, and anti-neuronal DNA, and low complement levels 4, 5.
Treatment Options for SLE
- The primary goal of treatment is to achieve disease remission or quiescence, defined by minimal symptoms, low levels of autoimmune inflammatory markers, and minimal systemic glucocorticoid requirement while the patient is treated with maintenance doses of immunomodulatory or immunosuppressive medications 4.
- Hydroxychloroquine is standard of care for SLE and has been associated with a significant reduction in mortality 4.
- Treatments in addition to hydroxychloroquine are individualized, with immunosuppressive agents, such as azathioprine, mycophenolate mofetil, and cyclophosphamide, typically used for treating moderate to severe disease 4.
- Biologic therapies, such as belimumab, voclosporin, and anifrolumab, may also be used to treat SLE, particularly in patients with active disease or lupus nephritis 6, 4.
- Other treatment options, such as rituximab, may be used in patients with severe SLE who do not respond to other medications 6.
- Symptomatic presentations, such as psychiatric and mood disorders, seizures, and inflammatory responses, may be treated with antipsychotics, antidepressants, anxiolytic medications, antiepileptic drugs, and immunosuppressants, respectively 5.