Sjögren syndrome

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Background

  • Systemic autoimmune disorder
  • Lymphocytic infiltration of exocrine glands
  • Can occur in isolation or with other autoimmune diseases (e.g. rheumatoid arthritis, SLE, etc.)
  • Female:male ratio of 9:1
  • 10-40 fold increased risk of lymphoma in SS patients.

Clinical Features


Differential Diagnosis

Evaluation

Emergent

  • Not generally an ED diagnosis
  • Evaluate for emergent complications
  • Thorough physical exam to evaluate for purpura (poor prognosis in primary SS)

Non-Emergent

  • Rheumatoid factor is found in 70% of patients
  • Anti-SS-A/Ro, and SS-B/La antibodies are part of diagnostic criteria
  • Elevated ESR
  • Hypergammaglobulinemia


Management

  • Manage emergent complications
  • Ocular
    • Topical ocular cyclosporine, preservative-free artificial tears, lubricating ointments
  • Oral
    • Frequent sips of water, chewing gum, or hard candies
    • Good oral hygiene
    • Pylocarpine or Cevimeline for severe xerostomia (parasympathetic and muscarinic agonists)
  • Hydroxychloroquine for Sjögren's associated arthritis
  • Prednisone or other immunosuppressants (cyclophosphamide or azathioprine) for severe systemic symptoms
  • AVOID atropine and decongestants (decrease salivary secretions)

Disposition

See Also

External Links

References