Sjögren’s syndrome ( /ˈʃoʊɡrɪnz/ SHOH-ɡrinz), 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 and the average age of onset is late 40s, although Sjögren’s occurs in all age groups in both women and men. It is estimated to strike as many as 4 million people in the United States alone making it the second most common autoimmune rheumatic disease.
Sjögren’s syndrome 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, scleroderma, primary biliary cirrhosis etc. (Secondary Sjögren’s syndrome).
The hallmark symptom of the disorder is a generalized dryness, typically involving dry mouth and 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 of IL-1RA in CSF, an interleukin 1 antagonist, suggesting that there was first increased activity in the interleukin 1 system, then an auto-regulatory up-regulation of IL-RA in attempts to reduce the successful binding of Interleukin 1 to its receptors. It is likely that Interleukin 1 is the marker for fatigue, however IL-1RA increases are observed in the CSF and is associated with increased fatigue through cytokine induced sickness behavior.[5] 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.
Dental carePreventive dental treatment is also necessary (and often overlooked by the patient), as the lack of saliva associated with xerostomia (dry mouth) creates an ideal environment for the proliferation of bacteria that cause dental caries (cavities). Treatments include at-home topical fluoride application to strengthen tooth enamel and frequent teeth cleanings by a dental hygienist. Existing cavities must also be treated, as cavities that extend into the tooth can not be effectively treated through teeth cleaning alone, and are at a high risk of spreading into the pulp of the tooth, leading to the loss of vitality and need for extraction or root canal therapy. This treatment regimen is the same as that used for all xerostomia patients, such as those undergoing head and neck radiation therapy which often damages the salivary glands, as they are more susceptible to radiation than other body tissues.
Unfortunately, many patients, not realizing the need for dental treatment, do not see a dentist until most of their teeth are beyond the point of restoration. It is not uncommon for a dentist to see a xerostomia patient with severe, untreatable caries in almost every tooth. In severe cases, the only viable treatment may be to extract all of the patient’s teeth and treat with prosthetics such as dentures and/or implants
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