What are the steps for a lupus workup and initial management?

From the Guidelines

A lupus workup begins with a comprehensive clinical evaluation, laboratory testing, and sometimes imaging studies, with initial management depending on disease severity and organ involvement, and should include hydroxychloroquine as the cornerstone therapy for mild disease, as recommended by the 2019 EULAR guidelines 1. The initial laboratory tests should include:

• Complete blood count
• Comprehensive metabolic panel
• Urinalysis
• Erythrocyte sedimentation rate
• C-reactive protein
• Antinuclear antibody (ANA)
• If ANA is positive, more specific antibody tests 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, as stated in the 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus 1. For mild disease, hydroxychloroquine 200-400 mg daily is the cornerstone therapy, providing disease control and reducing flares, as recommended by the EULAR guidelines 1. NSAIDs like ibuprofen 400-800 mg three times daily can help manage joint pain and inflammation. For moderate disease, low-dose corticosteroids such as prednisone 5-15 mg daily may be added temporarily. Severe disease with major organ involvement requires higher-dose prednisone (0.5-1 mg/kg/day) and immunosuppressants like mycophenolate mofetil (1-3 g daily), azathioprine (1-2.5 mg/kg/day), or cyclophosphamide for life-threatening manifestations, as stated in the 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus 1. All patients should receive preventive care including:
• Sun protection
• Vitamin D supplementation (1000-2000 IU daily)
• Calcium supplementation if on steroids
• Appropriate vaccinations, such as anti-pneumococcal vaccination, which is recommended for patients with SLE, as stated in the study on immunogenicity, safety, and tolerability of anti-pneumococcal vaccination in systemic lupus erythematosus patients 1. Regular monitoring of disease activity, medication side effects, and organ function is essential, with follow-up visits typically every 3-6 months for stable patients and more frequently during flares or medication adjustments. The 2019 EULAR guidelines provide updated recommendations for the management of systemic lupus erythematosus, including the use of hydroxychloroquine, glucocorticoids, immunomodulatory agents, and biologics, such as belimumab and rituximab, for the treatment of SLE, as stated in the study on the 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus 1.

From the FDA Drug Label

The FDA drug label does not answer the question.

From the Research

Lupus Workup

• The diagnosis of systemic lupus erythematosus (SLE) involves a comprehensive assessment, including accurate physical and laboratory diagnosis, monitoring of disease activity, and recording of accumulated organ morbidity 2.
• 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 3.

Initial Management

• All patients with lupus should receive education, counseling, and support 3.
• Hydroxychloroquine is the cornerstone of treatment because it reduces disease flares and other constitutional symptoms 3, 4.
• Low-dose glucocorticoids can be used to treat most manifestations of lupus, while the use of immunosuppressive and cytotoxic agents depends on the body systems affected 3.
• 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 3.

Diagnostic Approach

• The diagnostic approach for SLE includes the use of immunomodulators, immunosuppression, and targeted therapy 4, 5.
• Lifestyle changes, such as avoidance of ultraviolet light, and prevention of comorbidities, including coronary artery disease, osteoporosis, infections, and drug toxicities, are also important 4.
• New diagnostic tools and therapeutic approaches are being developed, including novel therapeutics and nonpharmacologic interventions 5.

Organ-Specific Considerations

• Liver disease is a common complication of SLE, with a 25-50% chance of developing abnormal liver tests in their lifetime 6.
• The most common cause of liver disease in SLE is drug-induced hepatitis, while mild hepatitis reflecting SLE activity is another possibility 6.
• Other liver diseases, such as thrombotic events, viral hepatitis, and autoimmune hepatitis, can also occur in patients with SLE 6.