What are the steps for diagnosing and managing Systemic Lupus Erythematosus (SLE)?

From the Guidelines

Diagnosing and managing Systemic Lupus Erythematosus (SLE) requires a comprehensive approach involving clinical evaluation, laboratory testing, and individualized treatment, with hydroxychloroquine as the cornerstone therapy for mild disease, and corticosteroids and immunosuppressants for moderate to severe disease, as recommended by the 2019 EULAR update 1.

Key Steps in Diagnosis and Management

• Diagnosis begins with recognizing the clinical features of SLE, including malar rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neurologic disorder, hematologic disorder, immunologic disorder, and antinuclear antibodies.
• Laboratory tests should include complete blood count, comprehensive metabolic panel, urinalysis, complement levels (C3, C4), and autoantibody testing (ANA, anti-dsDNA, anti-Smith) 1.
• If SLE is suspected, referral to a rheumatologist is recommended for confirmation and management.
• Treatment depends on disease severity and organ involvement, with the goal of achieving remission or low disease activity and preventing flares 1.

Treatment Options

• For mild disease, hydroxychloroquine 200-400 mg daily is recommended, at a 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 to severe disease, corticosteroids such as prednisone 0.5-1 mg/kg/day may be needed initially, with gradual tapering to less than 7.5 mg/day (prednisone equivalent) and, when possible, withdrawal 1.
• Immunosuppressants including methotrexate (15-25 mg weekly), azathioprine (1-2.5 mg/kg/day), mycophenolate mofetil (1-3 g/day), or cyclophosphamide may be added for organ-threatening disease.
• Belimumab, a biologic agent, can be considered for patients with active disease despite standard therapy.

Monitoring and Prevention

• Regular monitoring is essential, including clinical assessment every 3-6 months, laboratory tests to monitor disease activity and medication side effects, and screening for complications.
• Sun protection, cardiovascular risk management, bone health maintenance, and vaccination against pneumococcal and influenza infections are important preventive measures.
• Patient education about the chronic nature of SLE, medication adherence, and recognizing flare symptoms is crucial for successful management.

From the Research

Diagnosis of Systemic Lupus Erythematosus (SLE)

• The diagnosis of SLE depends on characteristic clinical features and laboratory test results, including skin rash, joint symptoms, and oral ulcers 2.
• A high index of initial suspicion of SLE is critical, and SLE should be suspected in any patient presenting with an unexplained disease process involving two or more organ systems 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, 4.

Diagnostic Criteria for SLE

• The diagnostic criteria for SLE include clinical manifestations such as skin lesions, arthritis, renal disorder, neurologic disorder, hematologic changes, and others 4.
• Serum anti-nuclear antibody, anti-ds-DNA antibody, and anti-Sm antibody are important biomarkers of SLE patients 4.
• The Systemic Lupus Collaborating Clinics proposed the SLICC criteria for SLE in 2012, which includes new knowledge of autoantibodies and the importance of low complement 4.

Management of SLE

• The management of SLE includes lifestyle changes such as avoidance of ultraviolet light, prevention of comorbidities, and use of immunomodulators and immunosuppressants 5.
• Hydroxychloroquine is the cornerstone of treatment because it reduces disease flares and other constitutional symptoms 3.
• 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 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.

Treatment Goals and Strategies

• The main goal of treatment is the achievement of remission or low disease activity, and comorbidities due to both disease activity and treatment adverse effects necessitate vigilant prevention and management strategies 6.
• Tailoring treatment options to achieve remission, while balancing treatment-related comorbidities, are priority areas of SLE management 6.
• New upcoming agents and regimens are currently under study, including belimumab, anifrolumab, and voclosporin 6, 5.