Understanding Sjogren’s Syndrome

Q. What is Sjögren’s syndrome and what causes this disease?

A. Sjögren’s syndrome (SS) is an autoimmune disease where the body attacks itself. Some examples of more familiar autoimmune diseases include systemic lupus erythematosus and rheumatoid arthritis. In the case of lupus, the body attacks the kidneys; with rheumatoid arthritis, the body attacks the joints. With SS, the body attacks the secretory glands, most commonly the salivary glands and the tear-producing lacrimal glands. As a result, there is diminished saliva and tear production. As dental professionals, we need to be aware of the symptoms and treatments available for SS because once the salivary glands cease to produce saliva, preventable oral health problems can occur.

Researchers have speculated for years that Sjögren’s may be caused by a viral etiology (i.e., Epstein-Barr virus).1 Determining the actual cause of autoimmune diseases, however, is still a big mystery.

PATIENT PROFILE

Q .Who is the most likely to develop Sjögren’s syndrome?

A SS is often diagnosed in the peri-menopausal female. The ratio of females to males with SS is 9:1. We are finding SS more often in younger people and in males with increased frequency, most likely due to greater awareness. And certainly dental professionals are in a unique position to recognize individuals with dry mouth and to immediately initiate preventive strategies.

Q. How prevalent is Sjögren’s syndrome?

A. In the United States, SS is estimated to affect 2-4 million people.1 It is also considered the second most common autoimmune disease after rheumatoid arthritis.1 Previously, it was thought that very few people suffered from SS but this line of thinking is changing. We are finding that it is more common than previously thought.

TREATMENT

Q. How is Sjögren’s syndrome treated and can it be cured?

A. There is currently no cure for SS. However, palliative treatments are available.

In this population, the protective effects of saliva is diminished as the amount of saliva produced by the salivary glands decreases. Saliva is important as it can physically wash away deleterious bacterial, viral, and fungal populations. It contains various proteins, enzymes and bioactive particles that can protect the oral cavity, initiate digestion and maintain homeostasis. When not enough saliva is present the protective effect is diminished and can result in increased oral infections, increased caries, and altered taste sensation. Fungal infections do not have the classic, white cheesy curd-type appearance. Rather, the appearance is erythematous and can affect the tongue, the oral mucosa, and corners of the lip (angular cheilitis). Patients often complain of a burning sensation and sensitivity to acidic/spicy foods. These symptoms are easily treatable with an antifungal medication. Most oral antifungal medications, however, contain sugar. Dental professionals need to explain to patients that they shouldn’t go to sleep with the medication in their mouths since the sugar will contribute to caries risk.

To address the risk of caries in these patients, dental professionals and patients need to think beyond just keeping their teeth clean. Diet and fluoride treatment are also important factors in reducing the risk of caries. I advise SS patients to use self-applied fluoride products, either in a tray or by brushing on a neutral fluoride gel or dentifrice. We will also consider application of a 5% sodium fluoride varnish. Prescription medication is also available to stimulate salivary flow (Cevimeline or Pilocarpine). The use of sugarfree hard candies or gum can also stimulate salivary flow. SS patients often experience severe oral dryness at night. The use of artificial salivas and lubricants* may be useful for increasing and individuals comfort at night.

Q. Why do Sjögren’s syndrome patients experience decay in atypical places?

A. SS patients experience decay in some very interesting places. We observe caries in cervical/root surfaces and also on incisal cusp tips. These are areas of the tooth that are typically considered accessible and cleansable with a toothbrush. We are not really sure why the demineralization process occurs here, but we can speculate.

We know that when the pH goes below a certain critical pH on the tooth surface, the tooth starts to demineralize. Theoretically, SS patients may not have enough saliva to maintain the correct pH level so their dentinal/enamel surfaces are spending a greater amount of time in an environment that favors demineralization over remineralization.

Q. So fluoride can help prevent the low pH problem.

A. Fluoride does not specifically prevent the consequences of low pH, but it does play a role in helping a tooth remineralize.

Q. Does Sjögren’s syndrome affect other parts of the body besides the oral cavity?

A. Definitely, as it is a systemic disease. SS affects tear production, which creates problems in the eye area similar to those experienced by people with allergies. The eyes are often red, itchy, and they could be painful. Patients often have achy joints and experience fatigue. A percentage of the patients may have a thyroid problem, kidney disease, lung disease, gastric reflux, dry skin or peripheral neuropathy.

Q. Is Sjögren’s syndrome most often diagnosed in a physician’s office when patients are seeking treatment for these other problems or do they often come to the dentist office first?

A. Both. The Sjögren’s Syndrome Foundation did a survey that found that the average time from onset of symptoms to diagnosis is more than 6 years [see www.sjogrens.org/syndrome/diagnosis/html]. As noted above, SS is a systemic disease that can affect many organ systems. An individual may be seeing many different specialists for the specific organ system affected (i.e., endocrinologist, pulmonologist, gastroenterologist, ophthalmologist, general internist, rheumatologist, neurologist) and until someone recognizes that common theme of dry eyes or dry mouth, the final diagnosis may not be achieved. And certainly dental professionals are in a unique position to identify someone who is experiencing dry mouth as the result of not making enough saliva.

Q. What are the initial indicators that a patient has salivary dysfunction?

A. Patients with salivary dysfunction usually present with an oral cavity that looks dry or parched. You can stick a mirror on their cheek/mucosa and it sticks. Another sign to look for is a lack of pooling of saliva in the floor of the mouth because this is the exit of the submandibular sublingual salivary glands.

Dental professionals are also very aware that in certain patients it is not necessary to frequently use the saliva ejector—there is little to no saliva to suck out. This patient may have difficulty swallowing a cracker without additional fluid and feel that they have do not have enough saliva. Another indicator that dental professionals should look for is cervical and incisal cusp tip caries. Patients may also complain of intraoral burning, sensitivity to spicy foods, and taste alterations. Adults who have continuing problems with decay may also have salivary dysfunction.

RESEARCH

Q. Is there any new research being done on Sjögren’s syndrome?

A Yes, the Sjögren’s Syndrome Clinic at UCSF, along with clinics located in 5 other countries ( Argentina, China, Denmark, Japan, and the United Kingdom), are currently working on a wonderful project that is funded by the National Institutes of Health. It’s a project where we do an evaluation for individuals complaining of dry eyes, dry mouth or individuals who have already been diagnosed with Sjogren’s syndrome at no charge and then catalog the data retrieved and samples provided. We’ve had people fly out from 35 different states plus Canada, Mexico, India, and England. We hope to understand this disease better by compiling complete patient histories, their tissue samples, saliva samples, and tear samples. The data and clinical samples will be made available to researchers to do genetic analyses and to look into the causes and the pathogenesis of the disease. This is probably the biggest stimulus to research in the area of Sjögren’s syndrome in the history of the disease.

Q. How many people will you include in the study?

A. We hope to get as many as possible, thousands would be nice. We have been funded for 5 years and we’re preparing to obtain additional funding for subsequent years. Please see our website at http://sicca.ucsf.edu or call (415) 476-0535 for further information.

REFERENCES

1. Clinical Manifestations and Early Diagnosis of Sjögren Syndrome Stuart S. Kassan, MD; Haralampos M. Moutsopoulos, MD, FRCP(Edin) Arch Intern Med. 2004;164:1275-1284. (http://archinte.ama-assn.org/cgi/content/abstract/164/12/1275)

*Biotene Dry Mouth Products, Laclede Inc, Rancho Dominguez, Calif

*Oasis Mouthrinse and Mouth Spray, GlaxoSmithKline, Moon Township, Pa

From Dimensions of Dental Hygiene. November 2007;5(11): 24, 26.