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sjogrens_syndrome

Sjögren's syndrome

introduction

  • also known as “Mikulicz disease” and “Sicca syndrome” is an autoimmune disorder in which immune cells attack and destroy the exocrine glands that produce tears and saliva.
  • named after Swedish ophthalmologist Henrik Sjögren (1899-1986), who first described it.
  • 90% of Sjögren's patients are women and the average age of onset is late 40s, although Sjögren's occurs in all age groups in both women and men.
  • the second most common autoimmune rheumatic disease.
  • can exist as a disorder in its own right (Primary Sjögren's syndrome) or it may develop years after the onset of an associated rheumatic disorder such as rheumatoid arthritis, systemic lupus erythematosus (SLE), scleroderma, primary biliary cirrhosis etc. (Secondary Sjögren's syndrome)

clinical features

  • the hallmark symptoms of the disorder are dry mouth and dry eyes (part of what are known as sicca symptoms)
  • may cause skin, nose, and vaginal dryness, and may affect other organs of the body, including the kidneys, blood vessels, lungs, liver, pancreas, and brain such as type I renal tubular acidosis (RTA).
  • causes increased levels of IL-1RA in CSF suggesting increased activity in the Interleukin 1 system and that this is associated with increased fatigue through cytokine induced sickness behavior
  • patients with secondary Sjögren's syndrome also have signs and symptoms associated with rheumatic disorder.
  • higher rate of non-Hodgkin lymphoma compared to both patients with other autoimmune diseases and healthy people.
  • ~5% of patients with Sjögren's syndrome will develop some form of lymphoid malignancy
  • Sjögren's syndrome in women who become pregnant has been linked to increased incidence of neonatal lupus erythematosus with congenital heart block requiring a pacemaker.

diagnostic tests

  • ANA
    • typical Sjögren's syndrome anti-nuclear antibodies (ANA) patterns are SSA/Ro and SSB/La, of which SSB/La is far more specific; SSA/Ro is associated with numerous other autoimmune conditions but are often present in Sjögren's
  • RhF
  • further Ix
sjogrens_syndrome.txt · Last modified: 2017/07/07 06:54 by gary1