Rheumatology Book

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Sjogren's SyndromeAka: Keratoconjunctivitis Sicca, Dry Eye Syndrome

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  1. Epidemiology
    1. Prevalence: 2-3 Million in United States
    2. Women predominantly affected
  2. Causes and Associated Conditions
    1. Primary: Specific autoimmune disease (Sjogren's)
    2. Secondary associations (Keratoconjunctivitis Sicca)
      1. Rheumatoid Arthritis (occurs in 25% of RA cases)
      2. Hypothyroidism (10-15%)
      3. Systemic Lupus Erythematosus
      4. Scleroderma
      5. Polymyositis
      6. Felty's Syndrome
      7. Pulmonary Fibrosis
  3. Pathophysiology: Chronic destruction of exocrine glands
    1. Lacrimal glands affected in Keratoconjunctivitis Sicca
    2. All exocrine glands affected in Sjogren's Syndrome
      1. Salivary Glands
      2. Lacrimal glands
      3. Sebaceous Glands
      4. Vaginal glands
  4. Symptoms and Signs
    1. Xerophthalmia (dry eyes): Keratoconjunctivitis Sicca
      1. Symptoms worse toward latter part of day
      2. Foreign Body sensation
      3. Painful or itching eyes
      4. Conjunctival injection
      5. KeratoConjunctivitis
    2. Other exocrine effects
      1. Xerostomia (Dry Mouth)
      2. Dry Skin
      3. Nasal dryness
      4. Sinusitis
      5. Bronchitis
    3. Constitutional and other effects
      1. Fatigue
      2. Major Depression
  5. Diagnostics
    1. Schirmer's Test
    2. Slit Lamp Exam of Cornea
      1. Punctate erosive keratopathy
  6. Labs
    1. Rheumatoid Factor positive
    2. Antinuclear Antibody positive
      1. Autoantibody Anti-Ro (Anti-SSA) present
      2. Autoantibody Anti-La (Anti-SSB) present
    3. Erythrocyte Sedimentation Rate elevated
    4. Complete Blood Count
      1. Leukopenia
      2. Anemia
    5. Liver Function Tests abnormal
  7. Management: General
    1. Dry eyes (Xerophthalmia)
      1. Sunglasses
      2. Room humidifiers
      3. Artificial tears or ointments
      4. Surgical lacrimal drainage occlusion in severe cases
    2. Dry Mouth (Xerostomia)
      1. See Dentist regularly
      2. Salivary substitutes
      3. Directly apply fluoride to teeth and in Toothpaste
    3. Dry nose
      1. Saline Nasal Spray
      2. Humidifier
    4. Dry Skin
      1. Skin moisturizing creams and ointments after shower
    5. Vaginal dryness
      1. Vaginal lubricants
      2. Estrogen Replacement Therapy
      3. Vaginal Estrogen Cream
    6. Infection
      1. Observe for Thrush or yeast Vaginitis
  8. Management: Systemic Medications
    1. Pilocarpine (for Sjogren's eye and mouth symptoms)
      1. Dose: 5 mg PO qid
      2. Causes diaphoresis and decreased night vision
    2. NSAIDs
    3. Hydroxychloroquine
    4. Corticosteroids
    5. Immunosuppressants
      1. Methotrexate
      2. Imuran
      3. Cyclosporin
      4. Cytoxan

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