Managing Xerostomia in Older Adults With or Without Sjögren Syndrome

Sjögren (pronounced like “show-grin”) syndrome is a systemic inflammatory disease affecting primarily the lacrimal and salivary glands. It may exist as a primary disorder (ie, primary Sjögren syndrome) or can be associated with other autoimmune diseases (ie, secondary Sjögren syndrome), such as rheumatoid arthritis, systemic lupus erythematosus, or systemic sclerosis. Sjögren syndrome is a T cell–driven autoimmune disease characterized by focal lymphocytic infiltration of the lacrimal and salivary glands. The etiology of Sjögren syndrome, although still ill-defined, is thought to be multifactorial, involving viral, neural, genetic, and environmental factors. Although the disease itself is not life-threatening, the common occurrence of dry mouth, or xerostomia, in Sjögren syndrome not only impacts quality of life but can lead to complications that increase the risks of morbidity and mortality among older adults, including candidiasis, dental caries, periodontitis, dysphagia, aspiration pneumonia, and other pulmonary infections. 

Yet, the severity of Sjögren syndrome and its oral symptoms appear to be relatively unknown and underrecognized in clinical practice. Annals of Long-Term Care: Clinical Care and Aging® (ALTC) had the opportunity to discuss xerostomia in older adults, with a focus on recognition and treatment of xerostomia in patients with Sjögren syndrome, with Driss Zoukhri, PhD, professor, department of diagnosis andhealth promotion, and Mabi Singh, DMD, MS, associate professor, department of oral pathology, oral medicine and craniofacial pain, Tufts University School of Dental Medicine, Boston, MA.

ALTC: Xerostomia occurs frequently in older adults due to a number of reasons. When patients present with dry mouth, why is it important to identify the etiology rather than simply treating it symptomatically?
Zoukhri and Singh: Salivary gland hypofunction leading to dry mouth occurs in various conditions. Attempts to identify the cause of salivary hypofunction may lead to identification of other ailments that may prevail. Some of the potential causes of salivary hypofunction are water/metabolites loss (eg, dehydration, impaired water intake, blood loss, emesis and diarrhea), renal water loss (polyuria, osmotic diuresis and protein calorie malnutrition), autoimmune diseases (Sjögren syndrome, rheumatoid arthritis, systemic lupus erythematosus, Hashimoto’s thyroiditis, HIV infections), medications, autonomic dysfunction, conditions affecting the central nervous system, psychogenic disorders, trauma, decrease in mastication, and diseases of the salivary glands (sarcoidosis, hepatitis C, liver diseases, cystic fibrosis).

Can you walk us through the process of diagnosing xerostomia in an older adult? Let’s start with the important notes from a patient’s medical history.
This would include history of any of the following: extreme fatigue, brain fog, repeated episodes of candidiasis, Raynaud’s phenomenon (a condition in which cold temperatures or strong emotions cause blood vessel spasms), waking up at night to drink water, constant sipping of water, and needing water to aid swallowing food. It’s also important to note family members with autoimmune diseases. Underlying medical ailments can be identified in a patient’s medical history. Also, recurrent mucosal infections and lesions in the oral cavity may be indicative of salivary gland hypofunction. Medical history can indeed reveal whether the patient has been through therapeutic irradiation in the head and neck area, such as any chemotherapy and surgical procedures that potentially can compromise salivary gland function.

What are some of the significant findings of xerostomia on physical examination?
These findings may include decreased output from the salivary glands; mirror sticking to oral (buccal and tongue) mucosa; frothy, thick, and viscous saliva; no salivary pooling on the floor of the mouth; fissuring or lobulated tongue; shortened papilla on tongue; altered gingival architecture; glassy appearance of the oral mucosa; and debris on palate or on teeth. Prevalence of multiple carious lesions on existing teeth and increased tooth surface loss, due to intrinsic and extrinsic erosion, and abrasions are observed in patients with salivary hypofunction.

Is laboratory testing necessary to diagnose xerostomia?
At Tufts University School of Dental Medicine, sialometry is done in all the patients. If evidence of salivary gland hypofunction is obtained and autoimmune diseases (eg, Sjögren syndrome) are suspected, then the serological tests (anti-Ro and anti-La) are done. Since serological tests are only about 60% sensitive, biopsy of minor salivary glands is requested to establish the diagnosis. Even though the minor salivary gland biopsy is considered the gold standard, there are variables that may contribute to misdiagnosis. 

Is there a gold standard therapeutic regimen for treating xerostomia? 
No, there are no evidence-based clinical practice guidelines or recommendations. The commonly used pharmacological agents used in the treatment of xerostomia are pilocarpine hydrochloride, and cevimeline hydrochloride. The most commonly used drugs to promote salivary secretion in the United States are pilocarpine hydrochloride and cevimeline. These are parasympathomimetic drugs and muscarinic agonists. The production of saliva is a result of their actions on especially M3 muscarinic receptors (and M1) and triggering the cascade of saliva production. The volume of saliva produced is dose-dependent and also depends on the remaining functional salivary glands. Because of the side effect of those agents and individual patient medical conditions, there may be limitations in prescribing them, and titration of the dosage may be necessary.

One recommendation for temporarily treating dry mouth is sipping water frequently, but this may not be the ideal solution at nighttime for elderly persons in long-term care because of the risk of falls associated with getting up at night to visit the bathroom. What do you recommend?
Big sips of water—although they help in hydrating the oral mucosa—will also wash away the protective agents in the saliva, including the proteins and mucins that give the sensation of wetness in the oral cavity. So, big and frequent sips of water are not recommended, but a small amount of water (just enough to wet and hydrate the oral mucosa) is recommended. To keep the mouth moist throughout the nighttime, breaking of vitamin E caplets in the mouth and application of oral rinses, oils, gels, sprays, and lubricants may help. If there is a problem with sleep (eg, with open mouth) or breathing problems (eg, blocked nose, deviated nasal septum), they also need to be treated to prevent xerostomia at night.

Let’s narrow our discussion to oral problems in patients with Sjögren syndrome. How prevalent is Sjögren syndrome, and why does it present more frequently in postmenopausal women than in other patient populations? 
The prevalence of Sjögren syndrome varies widely depending on the criteria for classification, but it is estimated that between 1 and 3 million people in North America suffer from this disease. Since Sjögren syndrome is a very underdiagnosed condition, it is difficult to estimate the prevalence. 

Several studies reported that androgens possess anti-inflammatory properties whereas estrogens were found to be proinflammatory. Androgen deficiency does occur during aging and after menopause. Furthermore, the serum levels of androgen precursors, hormones, and metabolites have been shown to be low in patients with Sjögren syndrome compared to controls. Therefore, postmenopausal women are at higher risk of developing Sjögren syndrome.

How is Sjögren syndrome diagnosed? 
Sjögren syndrome is considered to be the most commonly underdiagnosed autoimmune disease. It is not uncommon for there to be a delay of 5 to 8 years after symptom onset before a diagnosis is made. The diagnostic approach to Sjögren syndrome is complicated because it must include two different goals: first, assessment of the ocular and salivary components; and second, differentiation between the primary and secondary variants of the syndrome.

Are biopsies necessary?
The markers (circulatory antibodies) for Sjögren syndrome in the blood are anti-Ro/SSA and anti-La/SSB. These are only about 60% sensitive, meaning that 40% of the time, the result will be shown as negative. The biopsy of the minor salivary glands of the lower lip is still the gold standard to diagnose Sjögren syndrome. Harvesting 7 to 10 minor salivary glands and microscopic examination at different levels are necessary for proper diagnosis. Presence of 50 or more lymphocytes in 4 mm2  area is considered positive for Sjögren syndrome.

Does every patient with Sjögren syndrome experience some degree of dry mouth?
There is no direct linear correlation with the reduction of saliva and the experience of dry mouth. Some researchers have found that there could be a compensatory mechanism in the beginning stages of Sjögren syndrome. The degree of dry mouth is basically the function of extent of damage to the salivary glands and the remaining functional salivary glands. Generally, when the basal unstimulated salivary volume goes down anywhere from 50% to 75% of the original volume, experience of dry mouth occurs. In autoimmune diseases like Sjögren syndrome, the serous (watery) part of the salivary glands are destroyed first, resulting in thick, viscous, and mucous saliva, and patients may feel that there is excessive saliva in the mouth.

Besides xerostomia, what are other signs and symptoms of Sjögren syndrome?
The patient may report a sensation in the eyes like “sandy” or “gritty,” or feeling there is a foreign object in the eyes. Also, we look for dry skin, vaginal dryness, gastroesophageal reflux disease, recurrent chest infections, peripheral neuropathy, and Raynaud’s phenomenon. Fatigue is a cardinal symptom for xerostomic patients with Sjögren syndrome. Increased thirst, fissured or lobulated and coating on tongue, burning sensation of mouth, difficulty eating, swallowing, and loss of taste are other common oral symptoms. Sticking of oral tissue, especially the lips to the teeth, and the tongue to the roof of mouth, chapped lips, cracks at the corners of mouth and debris on teeth and palate are also seen, as well as increased experience of development of new dental carious lesions and recurrent carious lesions leading to failure of restorations.

In your experience, have you encountered any atypical presentations of Sjögren syndrome in an older adult?
We have diagnosed Sjögren syndrome in patients who have come for the treatments of common oral lesions (eg, lichen planus) or those who have tooth surface loss due to gastroesophageal reflux disease. Many patients who reported a strange taste and burning sensation in their mouth were diagnosed with Sjögren syndrome. Discomfort in wearing full and partial dentures also have led to the diagnosis of Sjögren syndrome. The fullness and swelling in the parotid and submandibular gland region also lead to the suspicion of Sjögren syndrome.

Given the risk of complications associated with Sjögren syndrome, it is surprising that many physicians do not take the time to test for the disease in older adults who present with oral dryness. Why do you think that is?
Sjögren syndrome is a very underrecognized condition. Since many older adults are taking one or more medications, the physicians and dentists often attribute the condition as a side effect of medications. That is one of the reasons why the diagnosis takes so long (on average, 8 years) after the first complaint of dryness in the mouth. The changes in qualitative and quantitative saliva are not typically measured in the physician’s or dentist’s office. Rather, the patients are often advised to drink or sip water as a remedy for the subjective sensation of dryness in the mouth. In our clinical practice, we find patients who are told that the problem is in their head.

How is Sjögren syndrome treated?
Treatment is basically directed toward the alleviation of the symptoms, such as dry mouth and dry eyes. Apart from using sialogogues (titrated to maximum dose) to increase the salivary flow, other potential complications of salivary hypofunction also have to be treated to reduce the risk factors in the oral cavity.

What should the provider do if the etiology of a patient’s xerostomia cannot be pinpointed?
Even if the etiology is not clear, if xerostomia is due to salivary gland hypofunction, the condition has to be treated to overcome the risk factors, decrease the occurrence of the complications of salivary gland hypofunction, and increase the quality of life of the patients.