Systemic Lupus Erythematosus (SLE): Overview, Manifestations, and Management

Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease characterized by loss of self-tolerance, autoantibody production (especially anti-dsDNA, anti-Smith), immune complex deposition, and inflammation affecting virtually any organ.

Clinical Spectrum

• Constitutional: Fatigue (90%), fever, weight loss.
• Mucocutaneous: Malar rash (butterfly), discoid lesions, photosensitivity, oral/nasal ulcers, alopecia.
• Musculoskeletal: Arthralgia/arthritis (non-erosive, symmetric, small joints).
• Renal (lupus nephritis): Proteinuria, hematuria, casts, rising creatinine—major morbidity/mortality driver.
• Neuropsychiatric: Headache, cognitive dysfunction, seizures, psychosis, stroke, transverse myelitis.
• Serositis: Pleuritis, pericarditis.
• Hematologic: Leukopenia, lymphopenia, thrombocytopenia, autoimmune hemolytic anemia.
• Cardiovascular: Accelerated atherosclerosis, valvular disease (Libman-Sacks), myocarditis.
• Antiphospholipid syndrome (APS): Thrombosis, pregnancy morbidity (associated, not exclusive to SLE).

Diagnosis

2019 EULAR/ACR classification criteria (weighted score ≥10 with positive ANA ≥1:80). Clinical judgment remains paramount.

Management Principles

• Hydroxychloroquine (HCQ) for all (unless contraindicated): reduces flares, thrombosis, damage, mortality.
• Glucocorticoids: Bridge for flares; taper to lowest dose; avoid long-term high-dose.
• Immunosuppressants (organ-threatening): MMF, azathioprine, cyclophosphamide, rituximab, belimumab, voclosporin, anifrolumab.
• Cardiovascular risk reduction: Statin, BP control, smoking cessation.
• Vaccinations: Inactivated vaccines OK; avoid live vaccines if immunosuppressed.
• Pregnancy planning: Pre-conception counseling; continue HCQ; manage nephritis/APS.

Multidisciplinary care (rheumatology, nephrology, dermatology, OB/GYN) improves outcomes.