Mind map

Salma with facial rash

Alya Almualla English

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