What are the triggers to avoid and treatment options for a patient with systemic lupus erythematosus (SLE)?

Lupus Triggers and Treatment

Triggers to Avoid

Patients with SLE must avoid ultraviolet light exposure, as sunscreen use prevents development of skin lesions following photoprovocation 1, 2.

Environmental and Medication Triggers

• UV light exposure is a proven trigger for lupus flares and should be strictly avoided with consistent sunscreen use 1, 3, 2
• Cigarette smoking should be discontinued, as it increases risk of disease activity 3
• Drugs implicated in drug-induced lupus must be avoided (specific agents include procainamide, hydralazine, and certain anti-TNF biologics) 3
• Oral estrogen use has been associated with increased risk for disease flares in two RCTs and should be avoided 1
• Infections can trigger flares through activation of innate and adaptive immune systems 4

Critical Pitfall

• Hormone-replacement therapy does not increase flare risk and improves bone density better than placebo, making it acceptable when indicated 1

Treatment Options

Cornerstone Therapy

Hydroxychloroquine is mandatory for all SLE patients at ≤5 mg/kg actual body weight (typically 200-400 mg daily), as it reduces mortality and disease activity 5, 6, 7.

• The FDA-approved dosage for SLE is 200 mg once daily or 400 mg once daily (or in two divided doses) 7
• Daily doses exceeding 5 mg/kg increase the incidence of retinopathy 7
• Hydroxychloroquine should be administered orally with food or milk, and tablets should not be crushed or divided 7

Glucocorticoid Management

Minimize chronic glucocorticoids to <7.5 mg/day prednisone equivalent for maintenance therapy, as doses above this threshold significantly increase infection risk and other adverse outcomes 8, 5.

• Glucocorticoids are used for acute disease control but should be rapidly tapered once control is achieved 6
• Patients on chronic glucocorticoids require calcium and vitamin D supplementation to protect against bone mass loss 1
• Bisphosphonates demonstrate beneficial effects for bone protection in SLE patients on long-term steroids 1

Immunosuppressive Therapy

For major organ involvement (particularly lupus nephritis), use mycophenolate mofetil as first-line or low-dose intravenous cyclophosphamide combined with glucocorticoids 5.

• Maintenance therapy with mycophenolate mofetil or azathioprine for long-term disease control 5
• Cyclophosphamide should be limited to minimize gonadotoxicity, especially in patients of reproductive age 5
• The evidence for immunosuppressive agents is limited to small sample sizes, requiring judicious use while considering potential harms 1

Adjunctive Therapies

• Low-dose aspirin should be considered in adult lupus patients receiving corticosteroids, those with antiphospholipid antibodies, and those with at least one traditional cardiovascular risk factor 1
• Statins and ACE inhibitors should be considered in selected patients with increased cardiovascular disease risk 1
• Belimumab is FDA-approved for adolescent SLE patients ≥5 years of age with active disease 5

Disease Monitoring

Use at least one validated disease activity index (BILAG, ECLAM, or SLEDAI) for monitoring disease activity, as these indices predict damage and mortality 1, 6.

• Anti-dsDNA antibodies, low serum C3/C4, and anti-C1q titres correlate with disease severity and predict future flares 1, 5
• Intensification of therapy based solely on serological activity (especially rising anti-dsDNA) risks overtreating patients, though one RCT showed it prevents relapses 1

Comorbidity Prevention

SLE patients have an almost 5-fold increased risk of death compared to the general population, primarily from infections, cardiovascular disease, and malignancies 1.

• Vigilant monitoring for infections is essential, as opportunistic infections are a major cause of morbidity and mortality, particularly in patients on chronic glucocorticoids and immunosuppressive agents 8, 9
• Screen for tuberculosis and viral hepatitis at the first clinical encounter 9
• Recommend pneumococcal vaccination and yearly influenza vaccination 9
• Screen for malignancies, particularly non-Hodgkin's lymphoma, lung cancer, and hepatobiliary cancer 1, 6

Critical Management Principle

Never escalate immunosuppression in a febrile patient with new symptoms without excluding infection first—this is a potentially fatal error 8.