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Salma with facial rash

Due to
1. Systemic Lupus Erythematosus
  1. Epidemiology
    1. Prevalence and incidence
      1. More in black than white
      2. More in women than men
      3. More in Hispanics
      4. More in Indians/Alaska Natives
    2. US
      1. Prevalence is 20-150/100,000 people
      2. White women "Prevalence is 164/100,000 people"
      3. African American women "Prevalence is 406/100,000 people"
    3. In Europe & North America "Prevalence is 24/100,000 people"
    4. In North America, South America, Europe, & Asia "Incidence is 1-25/100,000 people"
    5. UAE "2012"
      1. Incidence was 2.1/100,000 people
      2. Prevalence was 103/100,000 people
    6. Death rates
  2. Connective Tissue Disorder
    1. Rheumatoid arthritis
      1. Symmetric joint involvement
        Joints of the hands, feet, wrists, elbows, knees and ankles
      2. Morning stiffness lasting > 1 hour
      3. Women (30 - 60 years)
    2. Sjögren's syndrome
      1. Destruction of the salivary and lacrimal glands
      2. Women (40 to 60 years)
      3. Type 4 hypersensitivity
    3. Scleroderma \ Systemic sclerosis
      1. Fibroblast activation and excess collagen deposition
    4. Polymyositis
      1. Progressive symmetric proximal muscle weakness
    5. Dermatomyositis
      1. Gottron’s sign
        Symmetric erythematous eruption over knuckles
      2. Heliotrope rash
        Reddish eruption on upper eyelids
      3. Shawl sign
        Erythematous rash over neck, upper chest and shoulders
    6. Mixed connective tissue disease
      1. Autoimmune mediated tissue damage with mixed features of SLE, scleroderma, polymyositis
    7. Psoriatic arthritis
      1. Joint inflammation in patients with psoriasis
      2. HLA-B27 & environmental factors
  3. Risk factors
    1. Gender "Women"
    2. Age "15 - 44"
    3. Race or ethnicity
    4. Family history "5% - 13% chance"
    5. Genetic factors
  4. Pathogenesis
    1. Failure to maintain self-tolerance
    2. Tissue injury due to immune complex deposition
    3. Autoantibodies "Type II hypersensitivity"
    4. Immunofluorescence "IgG"
      1. Deposits of immunoglobulin
      2. Granular pattern "Kidney"
  5. Differential Diagnosis
    1. Adult-onset Still disease
    2. Behcet syndrome
    3. Chronic fatigue syndrome
    4. Endocarditis
    5. Fibromyalgia
    6. HIV infection
    7. Inflammatory bowel disease
    8. Lyme disease
    11. Reactive arthritis
    13. Sarcoidosis
    15. Thyroid disease
  6. Clinical manifestation
    1. Tachypnea
    2. Cough
    3. Photosensitivity
    4. Pleuritis/ Pleural effusion
    5. Weight loss
    6. Fatigue
    7. Fever & Increase infection
    8. Arthritis
    9. Emotional lability
    10. Hematological disorder
    11. Neurologic disorder
    12. Raynaud’s phenomenon
    13. Pericarditis
    14. Vasculitis
    15. Rash
      1. Erythematous
      2. Butterfly
    16. Lupus nephritis
      1. Proteinuria
      2. Hematuria
  7. Types
    1. Drug induced
      1. Anti-histone antibodies
      2. No involvement of kidney or brain
      3. Sulfasalazine, Hydralazine, Isoniazid, Procainamide / Phenytoin, Statins
      4. Symptoms: Arthralgia - myalgia - Fatigue - Serositis
    2. Neonatal lupus
      1. In fetus with mothers that have
        Anti Ro\SSA - anti La\SSB - anti U1RNP
      2. Placental transmission of maternal IgG abs
      3. Rash resembling Subcutaneous cutaneous lupus erythematosus
    3. Cutaneous lupus
      1. Acute
        Butterfly rash
      2. Subacute
        Annular or psoriasiform plaques
      3. Chronic
      4. Related to photosensitivity
    4. Chronic Discoid Lupus Erythematosus
      1. Red inflamed, coin-shaped patches of skin with a scaling and crusty appearance
      2. ANA
  8. Investigations
    1. CBC & differential
    2. Activated PTT
    3. BUN & electrolytes
    4. ESR & CRP
    5. Antinuclear antibodies, (ds) DNA, smith antigen
    6. Urinalysis
    7. Chest x-ray
    8. ECG
    9. C3, C4, CH50
    10. Anti-ro & anti-La abs
    11. Thyroglobulin abs & Thyroid peroxidase abs
    12. IgG
  9. Management
    1. Immunosuppressants
      1. General Mechanisms of Action
        Mono- and polyclonal antibodies that block T-cell surface molecules
        Antilymphocyte and antithymocyte Globulins
        Basiliximab
        Daclizumab
        Belimumab (B-cells)
        Rituximab (B-cells)
        Interference with cytokine production or action
        Disruption of cell metabolism, preventing lymphocyte proliferation
      2. Corticosteroids
        Mechanisms of action
      3. Hydroxychloroquine
        Decreases T-cell activation
      4. Antibodies
  10. Complications
    1. Blood clots / Vasculitis
    2. Pericarditis / Heart attack
    3. Stroke / Memory changes / Behavioral changes / Seizures
    4. Pleuritis
    5. Kidney inflammation / Decreased kidney function / Kidney failure
    6. Pregnancy complications & miscarriage
  11. Prognosis
    1. Clinically unpredictable
    2. 5-year survival rate (90%)
    3. 10-year survival rate (80%)

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Salma with facial rash

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	Created by Alya Almualla over 5 years ago