Sjögren's syndrome (pronounced /??o??r?nz/ shoh-grinz in English), also known as "Mikulicz disease" and "Sicca syndrome",[1] is a systemic autoimmune disease in which immune cells attack and destroy the exocrine glands[2] that produce tears and saliva.
It is named after Swedish ophthalmologist Henrik Sjögren[3] (1899–1986), who first described it.
Nine out of ten Sjögren's patients are women[4][5] and the average age of onset is late 40s, although Sjögren's occurs in all age groups in both women and men.[citation needed] It is estimated to affect as many as 4 million people in the United States alone, making it the second most common autoimmune rheumatic disease.[4][5]
Sjögren's syndrome can exist as a disorder in its own right (primary Sjögren's syndrome) or may develop years after the onset of an associated rheumatic disorder, such as rheumatoid arthritis, systemic lupus erythematosus, scleroderma, primary biliary cirrhosis etc. (secondary Sjögren's syndrome).[
Signs and symptoms
The hallmark symptom of Sjögren's syndrome is a generalized dryness, typically including xerostomia (dry mouth) and xerophthalmia (dry eyes), part of what are known as sicca symptoms. In addition, Sjögren's syndrome may cause skin, nose, and vaginal dryness, and may affect other organs of the body, including the kidneys, blood vessels, lungs, liver, pancreas, peripheral nervous system (distal axonal sensorimotor neuropathy) and brain.
Sjögren's syndrome is associated with increased levels in Cerebrospinal fluid (CSF) of IL-1RA, an interleukin 1 antagonist. This suggests that the disease begins with increased activity in the interleukin 1 system, followed by an auto-regulatory up-regulation of IL-IRA to reduce the successful binding of interleukin 1 to its receptors. It is likely that interleukin 1 is the marker for fatigue, however, increased IL-1RA is observed in the CSF and is associated with increased fatigue through cytokine induced sickness behavior.[6] On the other hand, Sjögren's syndrome is characterized by decreased levels of IL-1ra in saliva, which could be responsible for mouth inflammation and dryness. [7] Patients with secondary Sjögren's syndrome also often exhibit signs and symptoms of their primary rheumatic disorders, such as SLE, Rheumatoid Arthritis or Systemic Sclerosis.
http://en.wikipedia.org/wiki/Sj%C3%B6gren's_syndrome
