By Dr. C. Dennehey M.D.
University of California,
Irvine Medical Center,
Division of Rheumatology
Sjogrens syndrome is characterized by inflammation of the exocrine glands, which results in a variety of symptoms including dry eyes and dry mouth and other symptoms depending on which glands are affected. The first description of Sjogrens Syndrome was made in 1888 in a 42-year-old farmer with parotid gland swelling. Sjogrens Syndrome can occur in conjunction with SLE or can be considered “primary” meaning without any other associated disease. When Sjogrens Syndrome is associated with another disease such as SLE, the treatment includes treatment of the underlying disease such as SLE as well as treatment of Sjogrens Syndrome.
Sjogrens Syndrome symptoms may vary depending on the glands involved. The exocrine glands that are usually involved are the parotid glands, minor salivary glands, the lacimal glands that produce tears, and the pancreatic glands. If any or all the glands are affected, the symptoms can include dry mouth, dry eyes and abnormal digestion.
If the lacimal glands are affected, the glands become inflamed and are unable to produce adequate tears to lubricate the eyes. The danger with this is the development of corneal abrasions and ulcers. Which could lead to blindness if untreated. Your doctor may test for Sjogrens Syndrome of the lacrimal glands by doing an “schirmers test”. This involves putting a piece of filter paper under the lower eyelid and measure how many tears are produced and absorbed by the filter paper. If the filter paper absorbs more than 8mm, that is considered normal tear production, and if less than 8mm are produced, then this is considered abnormally low tear production. If you are found to have an abnormal tear production, you need to be seen by an ophthalmologist to be evaluated for corneal ulcerations that can occur from lack of tears.
If the parotid glands or the minor salivary glands of the lips are affected, then the predominate symptom is a perpetually dry mouth. Your physician will ask you if you can eat a cracker without a glass of water. Normally, people are able, Sjogrens Syndrome patients cannot. The sequela of this problem is the development of multiple cavities in the teeth, oral ulcerations, poor dentition, swallowing difficulties, and possible gastric reflux exacerbation secondary to the loss of the antibacterial and acidic neutralization activity of the saliva. Your physician may test your salivary gland function with the “saxon” test, which measures whole mouth saliva production. The patients are asked to chew a gauze sponge for 2 minutes: the difference in the weight of the sponge before and after chewing represents the amount of saliva produced. In general, a change in weight of 2.75 grams or more in the gauze represents normal saliva production.
If the pancreatic exocrine glands are affected, this can affect digestion with fats being unable to be digested and subsequent bowel difficulties including diarrhea and foul smelling stools.
Treatment of Sjogrens Syndrome includes treating the primary disorder such as SLE, which usually involves immunosuppressive drugs. Treatment also includes treating the specific symptoms. For dry eyes, there are a number of eye drops on the market ranging from simple tear solution to thick methylcellulose solutions that are used during the night to protect eyes while sleeping. Surgically, the tear ducts can be plugged to prevent drainage of any tears that are produced and subsequently keep the eyes lubricated.
Dry mouth symptoms are treated primarily through frequent sips of water. There is a medication available “pilocarpine” that stimulates saliva production.
Also, sucking on sugarless hard candies promoted saliva production. These patients must keep meticulous care of their teeth, and visit the dentist frequently.
Digestion difficulties can sometimes be alleviated with the addition of pancreatic enzymes to the diet.
The diagnosis of Sjorgens Syndrome is suspected by clinical symptoms as mentioned above and the diagnosis is confirmed by specific blood tests for auto antibodies called “SSA” and “SSB” and also specific pathological findings from a minor salivary lip gland biopsy.
Anti-Ro/SSA and anti-La/SSB auto antibodies are “Ana’s” or antinuclear antigen antibodies that have been detected in high frequency in patients with Sjogrens Syndrome. “Anti-Ro” was named after the original patient in whom the auto antibodies were identified. When anit-Ro/SSA antibodies are found in patients with SLE, (about 50%), these patients can have a variant of SLE called “subacute cutaneous lupus”. Women who have the SSA/SSB auto antibodies and become pregnant have an increased risk of developing a congenital heart block in the baby and need to be followed closely by the obstetrician and rheumatologist.
The significance of Sjogrens Syndrome is that rarely Sjogrens Syndrome may progress to a lymphoma. Those patients with Sjogrens Syndrome should be carefully monitored clinically for any evidence of development of lymphoma (a type of blood cancer).
Other rare manifestations of Sjogrens Syndrome, which can also occur in SLE, are nervous system inflammation and vasculitis.
Generally, the indication for testing for the SSA/SSB antibodies are in woman with SLE who are pregnant, women who have a history of giving birth to a child with heart block or myocarditis, patient with dry eyes, dry mouth or tear gland enlargement, and in patients with unusual SLE symptoms such as vasculitis or central nervous system symptoms.