What is the appropriate workup for Systemic Lupus Erythematosus (SLE)?

From the Guidelines

Systemic Lupus Erythematosus (SLE) Workup

The appropriate workup for Systemic Lupus Erythematosus (SLE) involves a comprehensive approach, including clinical assessment, laboratory tests, and imaging studies, as recommended by guidelines such as those from the European League Against Rheumatism 1.

Initial Assessment

• Patient Assessment: Evaluate disease activity using a validated index at each visit, assess organ damage annually, and consider general quality of life, comorbidities, and drug toxicity 1.
• Cardiovascular Risk Factors: Assess smoking, vascular events, physical activity, oral contraceptives, hormonal therapies, and family history of cardiovascular disease at baseline and during follow-up at least once a year 1.

Laboratory Tests

• Complete Blood Count (CBC): To evaluate for cytopenias.
• Basic Metabolic Panel (BMP): To assess renal function.
• Urinalysis: To check for proteinuria or hematuria.
• Erythrocyte Sedimentation Rate (ESR) or C-Reactive Protein (CRP): To assess inflammation.
• Antinuclear Antibody (ANA) Test: A positive result is a criterion for SLE diagnosis.
• Extractable Nuclear Antigens (ENA) Panel: Including anti-dsDNA, anti-Sm, anti-Ro, and anti-La antibodies.
• Complement Levels (C3 and C4): To assess immune complex activation.

Imaging Studies

Imaging studies may be necessary based on clinical presentation, such as chest X-rays for suspected pulmonary involvement or renal ultrasound for suspected nephritis.

Treatment and Monitoring

• Hydroxychloroquine: Recommended in all patients with lupus, at a dose not exceeding 5 mg/kg real body weight, as per the 2019 update of the EULAR recommendations 1.
• Glucocorticoids (GC): Should be minimized to less than 7.5 mg/day (prednisone equivalent) and, when possible, withdrawn during chronic maintenance treatment.
• Immunomodulatory Agents: Such as methotrexate, azathioprine, or mycophenolate, can expedite the tapering/discontinuation of GC.
• Belimumab: Considered for add-on therapy in persistently active or flaring extrarenal disease.
• Rituximab (RTX): May be considered in organ-threatening, refractory disease.

Regular Monitoring

Regular monitoring of disease activity, medication side effects, and preventive strategies for antiphospholipid antibody status, infectious diseases, and cardiovascular risk is crucial 1. The treatment plan should be individualized based on disease severity, specific organ involvement, and patient response to therapy.

From the Research

Diagnosis and Assessment of SLE

• The diagnosis of SLE is clinical, with classification criteria often used for diagnosis, yet with significant caveats 2
• The 2019 European Alliance of Associations for Rheumatology/American College of Rheumatology classification criteria are estimated to be 96.1% sensitive and 93.4% specific for SLE 3
• Accurate physical and laboratory diagnosis, monitoring of disease activity, recording of accumulated organ morbidity, and integration of these with the patient's own perceptions of health status and quality of life are essential components of the comprehensive assessment of SLE 4

Disease Activity Measurement

• Several instruments are available to measure disease activity and damage in patients with SLE, including the SLE Disease Activity Index (SLEDAI) 5
• Organ-specific measures for cutaneous, renal, and joint manifestations are needed for a detailed assessment of disease activity in conjunction with the use of disease generic tools 5
• New tools, such as the SLEDAI-2 KG, SLE-DAS, and SLEDAI-2 K RI-50, may be helpful in assessing disease activity and recording partial improvements 5

Treatment Goals and Options

• 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 3
• Hydroxychloroquine is the standard of care for SLE and has been associated with a significant reduction in mortality 3
• 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 3
• Biologic agents, such as belimumab, voclosporin, and anifrolumab, have been approved for the treatment of SLE and may be used in combination with other therapies 3, 6