Systemic lupus erythematosus

Definition:

• SLE is a chronic autoimmune disease characterized by the production of autoantibodies leading to systemic inflammation affecting multiple organ systems.

Epidemiology:

• More prevalent in women, especially during childbearing years.
• Higher incidence and severity in African, Hispanic, and Asian populations compared to Caucasians.

Pathophysiology:

• The exact cause is unknown, but involves a combination of genetic, environmental, and hormonal factors.
• Autoantibody production (e.g., antinuclear antibodies – ANAs) leads to the formation of immune complexes that deposit in tissues, causing inflammation and damage.

Clinical Features:

• Symptoms vary widely but can include:
  • General: Fatigue, fever, weight loss.
  • Skin: Malar rash (butterfly rash), discoid rash, photosensitivity.
  • Musculoskeletal: Arthralgia, arthritis.
  • Renal: Lupus nephritis.
  • Neurological: Seizures, psychosis.
  • Hematological: Anemia, leukopenia, thrombocytopenia.
  • Cardiovascular: Pericarditis, myocarditis.
• Disease course can be characterized by periods of remissions and exacerbations.

Diagnosis:

• Based on clinical criteria and laboratory findings.
• ANA testing is highly sensitive but not specific.
• Other antibodies: Anti-dsDNA, anti-Smith, anti-phospholipid antibodies.
• Complement levels (C3, C4) can be decreased.
• Renal biopsy may be necessary in cases of lupus nephritis.

Management:

• No cure; treatment aims to control symptoms and prevent organ damage.
• NSAIDs and hydroxychloroquine for mild symptoms.
• Corticosteroids and immunosuppressants for severe organ involvement.
• Regular monitoring for disease activity and medication side effects.
• Multidisciplinary approach involving rheumatology, nephrology, dermatology, neurology, etc.

Prognosis:

• Variable; modern treatments have significantly improved outcomes.
• Important to monitor for complications such as infections, cardiovascular disease, and renal failure.