Sjogren's Syndrome

Aka: Sjogren's Syndrome, Sjogrens Syndrome, Sjogren Syndrome, Keratoconjunctivitis Sicca, Dry Eye Syndrome

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II. Epidemiology

  1. Incidence: 4 per 100,000
  2. Prevalence: 1-2 Million in United States
  3. Gender: Predominately women (93% in one study)
  4. Age of onset: Middle aged (mean age of onset 52 years old in one study)
  5. Garcia-Carrasco (2002) Medicine 81(4): 270-80 [PubMed]

III. 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

IV. Pathophysiology

  1. Environmental stimulus triggers autoimmune reaction directed at exocrine glands
  2. Postulated triggers: CMV and EBV
  3. Association with HLA-DR haplotype
  4. B-Lymphocyte mediated chronic exocrine gland destruction
    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

V. Symptoms: Most Common (93-98% of patients at presentation)

  1. Xerostomia (Dry Mouth)
    1. Frequent sips of water
    2. May impact speaking, eating and Swallowing
    3. Exam demonstrates dry mucus membranes (secondary redness and ulcers may be present)
    4. Dental Caries are more common
    5. Parotid Glands tender to touch in some cases
  2. 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
    6. Corneal clouding in severe cases

VI. Symptoms: Other Associations

  1. Gastrointestinal
    1. Dyspepsia
    2. Altered stool habits
  2. Genitourinary
    1. Renal conditions (e.g. Glomerulonephritis, Interstitial Nephritis, Renal Tubular Acidosis)
  3. Hematologic
    1. Lymphadenopathy
  4. Musculoskeletal
    1. Non-erosive Arthritis
  5. Neurologic
    1. Peripheral Neuropathy
  6. Respiratory
    1. Nasal dryness
    2. Sinusitis
    3. Chronic Cough or recurrent Bronchitis
    4. Imaging
      1. XRay may show chronic Interstitial Infiltrates
      2. CT Chest may demonstrate alveolitis or fibrosis
  7. Skin
    1. Dry Skin
    2. Cutaneous Vasculitis
    3. Raynaud's Phenomenon
  8. Constitutional and other effects
    1. Fatigue
    2. Major Depression
    3. Fever

VII. Diagnosis: Revised International Classification Criteria (4 of 6 criteria required)

  1. Lip Salivary Gland biopsy positive (see diagnostics below)
  2. Autoantibodies Anti-Ro (Anti-SSA) and Anti-La (Anti-SSB) present
  3. Ocular symptoms: One or more of the following
    1. Dry Eyes Sensation everyday for at least 3 months
    2. Recurrent Eye Foreign BodySensation (sand or gravel Sensation)
    3. Artificial tears instilled more than 3 times daily
  4. Ocular signs: One or more of the following
    1. Schirmer Test
    2. Rose Bengal Test (or other ocular dye test)
  5. Oral symptoms: One or more of the following
    1. Dry MouthSensation everyday for at least 3 months
    2. Salivary Gland swelling recurrent or persistent
    3. Dry food intake requires frequent Swallowing of liquids
  6. Oral signs: One or more of the following demonstrating Salivary Gland involvement
    1. Nonstimulated Whole Salivary Flow Collection (<1.5 ml in 15 minutes)
    2. Contrast parotid sialography demonstrates diffuse sialectasia
    3. Salivary scintigraphy with delayed uptake and decreased concentration and excretion
  7. References
    1. Vitali (2002) Ann Rheum Dis 61(6): 554-8 [PubMed]

VIII. Diagnostics: Dry Eye evaluation

  1. Schirmer's Test
  2. Rose Bengal Test
  3. Slit Lamp Exam of Cornea
    1. Decreased tear meniscus
    2. Punctate erosive keratopathy

IX. Diagnostics: Dry Mouth evaluation

  1. Nonstimulated Whole Salivary Flow Collection
  2. Minor Salivary Gland biopsy from lip (pathognomonic findings)
    1. Acinar gland lymphocytic infiltration with secondary degeneration, necrosis, atrophy
    2. Positive biopsy: one or more foci of dense inflammatory infiltrate >50 lymphs/4mm

X. Labs: Serology

  1. Rheumatoid Factor positive (32-90% positive)
  2. Antinuclear Antibody positive (55-97% positive)
    1. ANA Test Sensitivity: 48%
    2. ANA Test Specificity: 52%
    3. Positive and Negative Likelihood Ratios approach 1.0
  3. Sjogren Antibodies
    1. Autoantibody Anti-Ro (Anti-SSA) present (16-70%)
    2. Autoantibody Anti-La (Anti-SSB) present (7-50%)
    3. Increased risk of Sjogren Syndrome when antibodies present in Rheumatoid Arthritis
    4. Increased risk of cytopenias, Discoid Lupus, Lupus Nephritis when antibodies present in SLE
  4. References
    1. Garcia-Carrasco (2002) Medicine 81(4): 270-80 [PubMed]

XI. Labs: Other

  1. Erythrocyte Sedimentation Rate elevated
  2. Complete Blood Count
    1. Leukopenia
    2. Anemia
  3. Liver Function Tests abnormal

XII. Management: General

  1. Dry Eyes (Xerophthalmia)
    1. See Dry Eyes for management
  2. Dry Mouth (Xerostomia)
    1. See Dry Mouth for Management
  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

XIII. Management: Systemic Medications

  1. Dry Eyes and Dry Mouth
    1. See Muscarinic Agonists (e.g. Pilocarpine)
  2. Arthralgias
    1. NSAIDs
    2. Hydroxychloroquine (Plaquenil)
    3. Corticosteroids
  3. Monoclonal antibodies
    1. Rituximab (Rituxan): anti-CD20 - decreases B-Lymphocyte activity

XIV. Complications

  1. Non-Hodgkin Lymphoma (40 fold increased risk over general population)
  2. Dental Caries
  3. Sialadenitis
  4. Corneal Ulceration
  5. Chronic Oral Infection

XV. References

  1. Fox (2005) Lancet 366(9482): 321-31 [PubMed]
  2. Kassan (2004) Arch Intern Med 164(12): 1275-84 [PubMed]
  3. Kruszka (2009) Am Fam Physician 79(6): 465-70 [PubMed]

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