SLE (Systemic Lupus Erythematosus)

Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease characterized by a wide range of clinical manifestations and a variable course. It involves multi-organ systems and presents with periods of flares and remissions. Here’s a comprehensive overview:

Causes

  • Genetic Factors: There is a genetic predisposition to SLE, although no single gene is responsible.
  • Environmental Triggers: Sunlight (UV radiation), infections, and certain medications can trigger SLE in susceptible individuals.
  • Hormonal Factors: SLE is more common in women, especially during childbearing years, suggesting a role for hormonal factors.
  • Immune System Dysfunction: Abnormal immune response leading to autoantibody production and immune complex formation.

Diagnosis

  • History:
    • Symptoms:
      • Fatigue, fever, joint pain, and rash are common.
      • History of photosensitivity, oral ulcers, hair loss, and Raynaud’s phenomenon may be present.
    • Family History: Of SLE or other autoimmune diseases.
  • Examination:
    • Skin: Malar rash (butterfly rash), discoid rash, photosensitivity.
    • Musculoskeletal: Arthritis or arthralgia, particularly in the hands, wrists, and knees.
    • Cardiopulmonary: Pericarditis, pleuritis.
    • Neurological: Seizures or psychosis.
    • Renal: Signs of nephritis.
  • Investigations:
    • Blood Tests:
      • ANA (antinuclear antibody) test is highly sensitive.
      • Anti-dsDNA and anti-Smith antibodies are more specific.
    • Complement Levels: Low C3 and C4 levels during flares.
    • Urinalysis: Proteinuria or hematuria indicating renal involvement.
    • Imaging and Other Tests: As indicated by organ involvement (e.g., chest X-ray for pleuritis, echocardiogram for pericarditis).

Differential Diagnosis (DDx)

  • Other Autoimmune Diseases: Rheumatoid arthritis, scleroderma.
  • Infectious Diseases: Endocarditis, hepatitis C.
  • Hematologic Disorders: Leukemia, lymphoma.
  • Drug-induced Lupus: Due to certain medications like hydralazine, procainamide, or isoniazid.

Management

  • General Measures:
    • Education about the disease and its management.
    • Sun protection to avoid flares triggered by UV light.
  • Medications:
    • NSAIDs: For joint pain and serositis.
    • Antimalarial Drugs: Hydroxychloroquine for skin and joint symptoms.
    • Corticosteroids: For moderate to severe disease.
    • Immunosuppressants: Methotrexate, azathioprine, mycophenolate mofetil for more severe disease or organ involvement.
    • Biologics: Belimumab and other newer agents for refractory cases.
  • Monitoring:
    • Regular follow-up for monitoring disease activity and treatment side effects.
    • Regular checks for organ involvement, especially renal and cardiovascular systems.
  • Lifestyle Modifications:
    • Avoidance of smoking.
    • Healthy diet and exercise.
  • Prevention of Complications:
    • Vaccinations, as some are more susceptible to infections.
    • Bone health management due to steroid use.

Conclusion

SLE is a multi-faceted disease requiring a holistic approach to diagnosis and management. It necessitates a careful balance between managing acute flares and long-term treatment to prevent organ damage, emphasizing the importance of personalized care and regular monitoring.