Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease characterized by the production of pathogenic autoantibodies (especially anti-ds…
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Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease characterized by the production of pathogenic autoantibodies (especially anti-dsDNA, anti-Smith) leading to immune complex deposition and inflammation in virtually any organ. SLE affects approximately 1.5 million Americans and 5 million people globally, with a 9:1 female-to-male ratio, predominantly affecting women of childbearing age. It is three times more common in Black women than in White women, and Black patients tend to have more severe disease. SLE is characterized by relapsing and remitting activity ('flares') and poses major risks of lupus nephritis, cardiovascular disease, infection, and organ damage.
This information is for educational purposes only. Always consult a qualified healthcare professional for diagnosis and personalized treatment.
Malar rash (butterfly rash): erythema over cheeks and nasal bridge, sparing nasolabial folds
Discoid rash: scarring, chronic cutaneous lesions
Photosensitivity: skin rash or flare after sun exposure
Oral/nasal ulcers (usually painless)
Arthritis: non-erosive, symmetric polyarthritis; arthralgias
Serositis: pleuritis, pericarditis (pleuritic chest pain)
Nephritis: hematuria, proteinuria, hypertension, renal insufficiency (lupus nephritis in 40–60%)
Neuropsychiatric manifestations: seizures, psychosis, cognitive dysfunction, headache
Hematologic: hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia
Constitutional: fatigue (most common complaint), fever, weight loss
SLICC 2012 or EULAR/ACR 2019 classification criteria (clinical + immunological domains)
ANA (antinuclear antibody): screening test, >95% sensitive but not specific
Anti-dsDNA: high specificity (~70–90%); correlates with disease activity and nephritis
Anti-Smith: highly specific (~99%) but low sensitivity (~25–30%)
Complement levels: low C3, C4 indicate active immune complex disease
CBC: cytopenias (anemia, leukopenia, lymphopenia, thrombocytopenia)
Urinalysis with microscopy: proteinuria, hematuria, RBC casts (nephritis)
Creatinine, eGFR for renal function
Anti-phospholipid antibodies (lupus anticoagulant, anti-cardiolipin, anti-β2GPI) — risk of APS
Renal biopsy: essential for diagnosing and classifying lupus nephritis (ISN/RPS classification I–VI)
Hydroxychloroquine (Plaquenil)
Antimalarial — cornerstone of SLE management for ALL patients unless contraindicated. Reduces flare frequency, damage accrual, thrombosis risk, and mortality. 200–400 mg/day. Annual ophthalmology monitoring for retinopathy.
Corticosteroids (Prednisone, Methylprednisolone)
For acute flares and organ-threatening disease. Minimize dose and duration (damage accrual correlates with cumulative steroid dose). IV methylprednisolone 500–1000 mg x3 days for severe flares (nephritis, CNS).
Belimumab (Benlysta)
BLyS-specific inhibitor (anti-BAFF monoclonal antibody). IV q4w or SC weekly. Reduces flares, serologic activity, and allows steroid tapering. Approved for active, autoantibody-positive SLE and active lupus nephritis.
Anifrolumab (Saphnelo)
Type I interferon receptor antagonist. IV q4w for moderate-severe SLE despite standard therapy. TULIP-1 and TULIP-2 trials. First new mechanism of action in SLE in >50 years after belimumab.
Voclosporin + MMF (Lupkynis for Lupus Nephritis)
Calcineurin inhibitor for active lupus nephritis class III/IV. AURORA 1 trial: superior complete renal response vs. MMF + placebo. Used in combination with mycophenolate mofetil.
Strict photoprotection: SPF ≥50 sunscreen, UV-protective clothing, hat — UV light triggers flares
Hydroxychloroquine adherence — most critical ongoing intervention
Avoid estrogen-containing contraceptives if anti-phospholipid antibodies positive or history of thrombosis
VTE prevention: aspirin or anticoagulation for antiphospholipid syndrome
Cardiovascular risk reduction: statin, blood pressure control (ACE/ARB for nephritis), smoking cessation
Vaccinations: pneumococcal, influenza, shingles (Shingrix), COVID-19 — avoid live vaccines if on immunosuppressants
Vitamin D supplementation (frequently deficient in SLE)
Fatigue management: regular low-impact exercise, sleep hygiene, cognitive behavioral therapy
Preconception planning: rheumatology + obstetrics co-management; avoid azathioprine/MMF in pregnancy
Prognosis
With modern therapy, 10-year survival in SLE exceeds 90% in developed countries. However, damage accrual is common: 50% of patients have irreversible organ damage within 10 years (renal, cardiovascular, neuropsychiatric). Lupus nephritis progresses to ESRD in 10–25% of patients. Cardiovascular disease (MI, stroke) is 5–10 times more common than in the general population and is the leading cause of late mortality. African American and Hispanic patients have worse renal outcomes. Early treatment, hydroxychloroquine use, and minimizing cumulative steroids improve long-term outcomes.
Systemic Lupus Erythematosus is a medical condition classified under Rheumatology. Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease characterized by the production of pathogenic autoantibodies leading to immune complex deposition and inflammation in virtually any organ. Understanding Systemic Lupus Erythematosus is essential for patients, families, and healthcare providers to ensure timely diagnosis, appropriate treatment, and optimal outcomes.
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The information on this page is for educational purposes only and is not intended as medical advice, diagnosis, or treatment. Always consult your doctor or a qualified healthcare provider with any questions about a medical condition.