What is the approach to a lupus workup and treatment?

From the Guidelines

A lupus workup begins with a comprehensive clinical evaluation, laboratory testing, and sometimes imaging studies, with treatment depending on disease severity and organ involvement, and the goal of achieving remission or low disease activity, as recommended by the most recent guidelines 1.

Key Components of Lupus Workup and Treatment

• Initial laboratory tests should include complete blood count, comprehensive metabolic panel, urinalysis, erythrocyte sedimentation rate, C-reactive protein, antinuclear antibody (ANA), and if ANA is positive, more specific antibodies like anti-double-stranded DNA, anti-Smith, anti-Ro/SSA, anti-La/SSB, and antiphospholipid antibodies.
• Complement levels (C3, C4) should be measured as low levels suggest active disease.
• Hydroxychloroquine (200-400 mg daily) serves as the foundation of therapy for almost all lupus patients, reducing flares and organ damage, with a recommended dose not exceeding 5 mg/kg real body weight 1.
• NSAIDs like ibuprofen (400-800 mg three times daily) can help manage joint pain and inflammation.
• For moderate disease, corticosteroids like prednisone (starting at 0.5-1 mg/kg/day with tapering) may be needed for flares, with the goal of minimizing glucocorticoid use to less than 7.5 mg/day (prednisone equivalent) and, when possible, withdrawing them 1.
• Immunosuppressants such as methotrexate (7.5-25 mg weekly), azathioprine (1-2.5 mg/kg/day), or mycophenolate mofetil (1-3 g daily) are used as steroid-sparing agents.
• Severe lupus, especially with renal or central nervous system involvement, may require cyclophosphamide, rituximab, or belimumab.
• Sun protection, regular monitoring for disease activity and medication side effects, and addressing comorbidities like cardiovascular disease risk factors are essential components of management.
• Patient education about recognizing flares and medication adherence is crucial for successful treatment outcomes.

Recent Updates and Recommendations

• The 2023 update of the EULAR recommendations for the management of systemic lupus erythematosus emphasizes the importance of treating to target, with the goal of achieving remission or low disease activity, and provides updated guidance on the use of biologic agents, including anifrolumab and belimumab 1.
• The use of hydroxychloroquine is recommended for all patients with SLE, unless contraindicated, at a dose not exceeding 5 mg/kg real body weight, with regular monitoring for retinal toxicity 1.
• The treatment of lupus nephritis has been updated to include the use of belimumab and voclosporin, with a focus on achieving complete or partial remission 1.

From the FDA Drug Label

Hydroxychloroquine sulfate is indicated for the treatment of chronic discoid lupus erythematosus and systemic lupus erythematosus in adults.

During an exacerbation or as maintenance therapy in selected cases of: systemic lupus erythematosus

The approach to a lupus workup and treatment may involve the use of hydroxychloroquine and prednisone.

• Hydroxychloroquine is indicated for the treatment of chronic discoid lupus erythematosus and systemic lupus erythematosus in adults 2.
• Prednisone may be used as adjunctive therapy for short-term administration or as maintenance therapy in selected cases of systemic lupus erythematosus 3. Key points to consider in the treatment of lupus include:
• The use of hydroxychloroquine for the treatment of chronic discoid and systemic lupus erythematosus
• The potential use of prednisone for short-term or maintenance therapy in selected cases of systemic lupus erythematosus

From the Research

Approach to Lupus Workup

• The American College of Rheumatology has 11 classification criteria for lupus, and if a patient meets at least four criteria, lupus can be diagnosed with 95% specificity and 85% sensitivity 4
• Laboratory tests, such as ANA IIF, are effective screening assays in patients with clinical features of SLE, but false positives are common 5
• A combination of ENA and dsDNA assays will detect most patients with SLE, and ESR and CRP measurements provide useful additional information 5

Treatment of Lupus

• Hydroxychloroquine is the cornerstone of treatment because it reduces disease flares and other constitutional symptoms 4
• Low-dose glucocorticoids can be used to treat most manifestations of lupus, and the use of immunosuppressive and cytotoxic agents depends on the body systems affected 4
• Belimumab, a monoclonal antibody, has shown clinical efficacy in reducing proteinuria and improving neuropsychiatric manifestations in patients with SLE 6
• Rituximab can be an effective treatment option for patients with refractory lupus with severe or life-threatening disease, with an acceptable tolerance profile 7

Management and Monitoring

• Patients with mild disease that does not involve major organ systems can be monitored by their family physician, while patients with increased disease activity, complications, or adverse effects from treatment should be referred to a rheumatologist 4
• A combination of anti-dsDNA, C3, C4, CRP, and ESR assays provides the most useful clinical information for monitoring disease activity 5
• Regular monitoring of outwardly silent manifestations, such as renal disease, is crucial to minimize potentially devastating complications 8