- 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.
- 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.
- 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
- further Ix
sjogrens_syndrome.txt · Last modified: 2017/07/07 06:54 by 127.0.0.1