Please confirm topic selection

Are you sure you want to trigger topic in your Anconeus AI algorithm?

Updated: Dec 28 2021

Sjogren Syndrome

  • Snapshot
    • A 60-year-old man presents to his physician’s office for fatigue, dry mouth, and dry eyes for the past year. He reports having always had a history of dry eyes that is managed with artificial tears. A year ago, he started feeling thirsty and was evaluated for diabetes, which was ruled out. He reports that he had several dental caries at his last dental appointment, despite maintaining overall good oral hygiene habits. Physical exam reveals bilateral swollen parotid glands, conjunctivitis, and dry mucous membranes. He also has dental caries. He is sent for additional workup for suspicion of an autoimmune disease.
  • Introduction
    • Clinical definition
      • an autoimmune disease of the exocrine glands
  • Epidemiology
    • Prevalence
      • relatively common (up to 5% prevalence)
    • Demographics
      • female > male
      • 40-60 years of age
      • second most common autoimmune rheumatic disease
    • Risk factors
      • family history
      • comorbid autoimmune disease
    • Pathogenesis
      • associated with HLA-DR52
      • environmental trigger, such as a viral infection, may cause
        • inflammatory destruction of exocrine glands and is characterized by aggregation of lymphocytes, primarily CD4+ T-cells and memory cells
          • the salivary and lacrimal glands are most commonly affected
      • glandular neurodegeneration from apoptosis mediated by cytokines and inflammatory cells
    • Associated conditions
      • other autoimmune diseases
        • primary biliary cholangitis
        • CREST syndrome
        • rheumatoid arthritis
        • systemic lupus erythematosus
      • marginal zone lymphoma
      • viral infections
        • hepatitis C virus
        • EBV
        • HIV
  • Presentation
    • Symptoms
      • sicca symptoms in > 95% of patients
        • ↓ saliva production causing dry mouth (xerostomia)
        • ↓ tear production causing dry eyes (keratoconjunctivitis sicca or xerophthalmia)
          • foreign body sensation
        • dyspareunia in women
      • constitutional symptoms
        • fatigue
        • weight loss
        • myalgias
        • joint pain
    • Physical exam
      • cutaneous findings
        • may have cutaneous vasculitis
          • flat or palpable purpura
          • urticarial vasculitis
        • dry skin
        • cheilitis
      • ocular findings
        • conjunctival injection
      • oral findings
        • dental caries or periodontal disease
        • dry mucous membranes
      • bilateral parotid gland enlargement
  • Imaging
    • Salivary gland scintigraphy or contrast sialography
      • indication
        • to evaluate the salivary gland function
  • Studies
    • Labs
      • positive anti-SSA (anti-Ro) (50-70% of cases)
      • positive anti-SSB (anti-La) (50-70% of cases)
        • anti-Ro/La can cross the placenta and cause neonatal heart block in infants of women with Sjogren syndrome
      • positive antinuclear antibodies (50-90% of cases)
      • possible positive rheumatoid factor (30-90% of cases)
    • Corneal staining to assess ocular damage
    • Labial salivary gland biopsy
      • indication
        • to confirm diagnosis, especially in patients with negative anti-Ro or anti-La antibodies
      • findings
        • dense inflammatory infiltrate in exocrine glands
  • Differential
    • Diabetes
    • Hepatitis C
    • Anticholinergic drug use
    • Mumps
    • Making the diagnosis
      • based on clinical presentation and laboratory studies
  • Treatment
    • Medical
      • artificial tears
        • indication
          • dry eyes
      • pilocarpine or cevimeline
        • indication
          • dry eyes or dry mouth
      • vitamin D supplementation         
        • indications
          • for all patients
          • vitamin D deficiency may increase risk of neuropathy and lymphoma 
  • Complications
    • Dental caries
    • Oral candidiasis
    • Corneal abrasion or ulceration
    • Mucosa-associated lymphoid tissue lymphoma
    • Parotid gland infection
  • Prognosis
    • Patients are at risk for non-Hodgkin lymphoma and other autoimmune diseases
1 of 0
1 of 2
Private Note

Attach Treatment Poll
Treatment poll is required to gain more useful feedback from members.
Please enter Question Text
Please enter at least 2 unique options
Please enter at least 2 unique options
Please enter at least 2 unique options