Salma with facial rash

Systemic Lupus Erythematous
1. Immunopathology
  1. Presence of auto reactive B cells
    1. Lack of clearance
      1. Presence of inflammation
  2. Type 3 hypersensitivity
    1. Increased vascular permeability allows immune complexes to be deposited in the blood vessel walls
      1. This induces platelet aggregation and complement activation
        Neutrophils are attracted to the site by C3a and C5a
        Neutrophils release their lysosomal enzymes by exocytosis causing further damage to the vessel walls
  3. Type 2 hypersensitivity
    1. Autoantibodies against RBCS, WBCS and platelets
      1. Opsonize these cells and lead to their phagocytosis → Cytopenias
    2. Autoantibodies against phospholipids
      1. Secondary antiphospholipid syndrome → Hypercoagulable state
        Risk of arterial thrombosis → MI, stroke or Libman-Sacks endocarditis
        Risk of venous thrombosis
        Risk of miscarriage in females
Risk factors
1. Genetic factors
  1. Familial association
  2. HLA association
  3. Complement pathways
  4. FcγRIIb
2. Environmental Factors
  1. UV radiation
    1. Cigarette smoking
      1. Sex hormones
        Drugs
3. Immunological abnormalities
  1. Type I interferons
    1. TLR signals
      1. Failure of B cell tolerance
Signs and symptoms
1. Rash (Malar or discoid)
  1. Serositis
    1. Oral/nasopharyngeal ulcers
      1. Photoensitivity
        Immunological disorder (Anti-dsDNA, Anti-Sm)
        Neurological disorders
        ANA positive
      2. Renal disease
    2. Hematologic disorder
  2. Arthritis
Causes
1. Sunlight exposure
  1. Infections
    1. Medications
      1. Hydralazine
        INH
        Procainamides
Types
1. Systemic lupus erythematosus
  1. Cutaneous lupus erythematosus
    1. Drug-induced lupus erythematosus
      1. Neonatal lupus
        It is a rare condition that affects infants of women who have lupus and is caused by antibodies from the mother acting upon the infant in the womb
        Anti-SSA, Anti-SSB positive
    2. Red, raised, scaly non-scarring rash
Complications
1. Blood
  1. Renal
    1. Membranous nephropathy
      1. Renal failure (main cause of death)
    2. Heart
      1. Libman-Sacks endocarditis
      2. Acute pericarditis
      3. CAD
  2. ITP / IHA
  3. Anemia of chronic disease
Investigations
1. Chemistry Panel
  1. Complement
    1. ESR, CBC, GFR
      1. Urine Tests
        Urine Protein
        Creatinine Clearance:
        Urinalysis
Management
1. prevent end-organ damage
  1. prolong survival
    1. maintain disease suppression
      1. less number of flares

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Salma with facial rash

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	Created by Fatima Alkhateeb almost 6 years ago