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«Sjögrenʼs Syndrome Textbook by Stone, Casal, and Moutsopoulos Chapter: Therapy of Oral and Cutaneous Dryness Manifestations in Sjögrenʼs Syndrome ...»

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Sjögrenʼs Syndrome Textbook

by Stone, Casal, and Moutsopoulos

Chapter: Therapy of Oral and Cutaneous Dryness Manifestations

in Sjögrenʼs Syndrome

Robert I. Fox, M.D, Ph.D.*

Carla M. Fox, R.N.*

Rheumatology Clinic

Scripps Memorial Hospital and Research Foundation

La Jolla, California

Address correspondence to:

Robert I. Fox, M.D., Ph.D.

Chief, Rheumatology Clinic

9850 Genesee Ave, #910

La Jolla, California USA 92037

RobertFoxMD@mac.com

http://www.robertfoxmd.com *Acknowledgements:

Our thanks for the wonderful contributions to patient care provided over many years to our patients from the Scripps Oral Health Clinic (headed by Dr. John Weston) and dedicated Oral Hygienists including Laurie Powell and Joanne Snyder, as well to our Dermatology associates including Alice Liu and Judith Kopersky, our Gynecology associates including John Willems, and our Urology associates including John Naitoh.

Key Words Sicca symptoms Xerostomia Oral lubricant Cholinergic agonist Mechanical stimulation of saliva Dirunal variation of saliva flow Burning Mouth Syndrome Xerosis Vaginal dryness ABSTRACT Treatment of oral dryness in the Sjögrenʼs syndrome patient remains unsatisfactory. A variety of methods including mechanical stimulation of the oral mucosa with sugar-free lozenges, mechanical devices, topical lubricants to facilitate chewing-swallowing-speaking, and oral agents to increase salivation by augmenting cholinergic function are currently used.

New approaches in early clinical trial include:

1. agents that impede water loss across the mucosal membrane by sodium-linked channels; and

2. stimulation of water transport directly across the mucosal membranes by purogenic stimulated water transporters.

Rheumatologists must recognize that a dry mouth is not necessarily a painful mouth and that oral candidiasis is a frequent treatable cause of increased symptoms.

This erythematous candida infection often occurs under the dentures in the SS patient in a location that is not part of the normal rheumatologic exam.

Additionally, when prescribing medications for the SS patient, potential anticholinergic side effects and normal diurnal variation of salivation must be considered.

In many SS patients, the symptoms of dryness are out of proportion to either clinical exam or results of salivary scintography. This may reflect the importance of mucosal mucins to reduce the viscosity of movement of tongue and membranes. Also, the painful mouth may result from “burning mouth” syndrome, which includes a spectrum of causes from localized neuropathy to depressive syndromes.

Although specific dental aspects are best addressed by the Oral Medicine Health team, the rheumatologist frequently will be asked by the patient about their additional particular oral needs due to both their dryness and their systemic medications. Particularly, recommendations regarding fluoride treatments, agents to improve calcification, the use of cosmetic dentistry and implants are important and expensive topics to the patient.

Also, simple instruction about their particular needs at the time of surgery (such as the ability to use oral lubricants while they are water intake restricted) and attention to methods of intubation in order to preserve teeth/dentures may be save expensive reconstructive surgery at a later date.

Other dryness complaints of the SS patient include dry skin (xerosis) and vaginal dryness. Although these are best evaluated by the dermatologist and gynecologist, the rheumatologist must be familiar with the treatments used by other specialists.

I. Background Sjögrenʼs syndrome (SS) is characterized by dry eyes and dry mouth. This chapter will concentrate on the treatment of dry mouth and the particular needs that the patient can perform for themselves and the special attention by the dentist and oral hygienist required as a consequence of dryness.

The importance of oral dryness extends far beyond simple difficulty in eating certain foods or increased frequency/expense of dental problems. Women socialize around eating, and thus, the inability to eat the same foods with family and friends impact on their overall quality of life, as measured by the “Oral Health Quality»\Assessment Scales.” Increasingly, women engage in “working” breakfast, lunch and dinner meetings, as well as the need to give oral presentations. All of these activities are profoundly impacted by dry mouth and difficulty with talking due to oral dryness.

For example, it may be necessary to pause for a sip of water every few sentences in order to be able to spend hours on the phone or give public presentations. Tooth loss not leads to expensive and often painful dental restorations, but also may alter the nutritional intake, as patient is unable to chew or swallow particular foods.

A first step in effective management of SS is the recognition of medications with drying anti-cholinergic side effects (including over-the-counter sleeping aids), as well as consideration of diurnal variation in salivary flow rates with decrease in basal secretion at night and accompanying sleep disturbance.

Thus, medications with these side effects should not be given at a time when the effects will be additive with the decrease salivation at night. The interruption of sleep due to dryness may result from a vicious cycle of drinking more water and polyuria that leads to morning fatigue. Thus, patients might be encouraged to drink less water after dinner and use oral lubricants in order to avoid sleep disruption due to need for urination.





The treatment of dryness symptoms remains unsatisfactory, due in part to the poor correlation of patient symptoms of oral discomfort with objective measurement of salivary flow rates. There is a general misconception that dryness results from the destruction of the salivary glands, when only about 50% of the acini/ductal cells are destroyed in patients with severe dryness. This points out the importance of the inflammatory process on the neural innervation and post neural salivary gland function induced by local production of cytokines and metalloproteinases.

However, symptoms of dry mouth (like dry eyes) are dependent on a “functional circuit” where afferent nerves from the mucosal surface travel to the midbrain (salvatory nucleus) which also receives input from higher cortical regions (including taste, smell, anxiety and even auditory-- as Pavlov illustrated in his famous experiments where ringing a bell led to salivation in dogs).

This discrepancy between symptoms and signs of dry mouth is extremely important clinically. It is most apparent in those patients with “burning mouth” syndrome, where severe symptoms are not accompanied by objective evidence of oral dryness on exam or a dramatic decrease in the flow salivary flow rate by scanning technique. This emphasizes the role of the central nervous system (central sensitization) or localized neuropathy in the generation of symptoms and how the patient perceives the severity of “dryness sensation.” Patients with clinical depression, neuropathy, Alzheimer's disease (even in the presence of drooling), and multiple sclerosis frequently have complaints of dryness.

Therapeutically, treatments for dry mouth include topical agents to lubricate and facilitate swallowing and talking. Oral agents may increase the cholinergic innervation of the glands. Since limited time during the patientʼs rheumatology clinical encounter does not allow detailed patient instruction, we have used email as a highly effective means of providing instructions to the patient about where to purchase certain products and what precautions need to be taken to prevent tooth loss.

Over the past 20 years, a variety of mechanical devices placed in the mouth have been shown to stimulate basal saliva flow. This emphasizes the role of mechanical stimulation such as brushing the tongue and buccal mucosa, as well as frequent use of sugar-free lozenges.

In addition, other sicca symptoms include dryness of the skin (xerosis), nasal dryness, and vaginal dryness. The need to keep the nasal passages clear and moist will prevent mouth breathing, particularly at night, when the basal secretory rate is diminished. Also, the role of smell is closely linked to gustatory stimulation.

II. General Approach to Dry Mouth

–  –  –

Approaches to clinical management of oral sicca symptoms are generally the same for primary or secondary SS. The quality of information available from resources on the Internet or from patient support groups varies widely.

Current recommendations by the American Dental Association include:

–  –  –

Avoidance of sucrose, carbonated beverages, juices, and water with • “sweetener” additives.

Avoidance of oral irritants (e.g., coffee, alcohol, and nicotine) • Maintenance of good hydration by taking regular sips of water and • drinking sugar-free liquids (See “Fluids” below.) Use of toothpastes specifically designed for dry mouth, which lack the • detergents (such as sodium lauryl sulfate, which is the foaming agent in most toothpastes)28. These substances, that are present in many types of toothpaste, can irritate the dry mouth. 29(6, #15969).

While infrequent, we caution patients that some of the fluoride-containing toothpastes may lead to brown discoloration of the teeth. Many of these “dry mouth” toothpastes are widely advertised, but have not been carefully studied in terms of their outcome for dental caries or tooth loss.

One commercially available toothpaste (i.e., Biotene®), was found to give • improved symptomatic relief in SS patients, although no change in the microbacterial composition of the biofilm was noted 29.

We also encourage our patients to use toothbrushes with features that improve effectiveness, such as inter-dental brushes (for cleaning between teeth) and electric toothbrushes.

[Please see suggestions listed in Table 3 for Dental Products] A number of basic measures are used to prevent as well as treat dry mouth in those with SS. The published evidence supporting these measures is limited, largely consisting of clinical experience and small case series in SS 19. Several older double-blind studies in SS patients demonstrated that the addition of mucins to carboxymethylcellulose (CMC) was superior to CMC alone 20-22. A small single-blinded study using a commercially available product (Oral Balance®) also showed symptomatic benefit 23.

The following measures for prevention of dryness, stimulation of secretions, dental prophylaxis, and attention to complications, should be used in all patients

with dry mouth due to SS, regardless of symptom severity:

Avoidance of medications that may worsen oral dryness, especially those • with anti-cholinergic side effects. These include over-the-counter antihistamines, cold and sleep remedies;

Avoidance of smoking and alcohol;

• Maintenance of open nasal passages to avoid mouth breathing (see • ʻNasal Drynessʼ below).

Use of room cold humidifiers, particularly at night, in areas that are dry or • windy;

Stimulation of salivary secretions, using sugar-free salivary stimulants • (e.g., chewing gums and lozenges);

Careful chewing of food before swallowing (see ʻTopical Stimulation of • Salivary Flowʼ below);

Meticulous oral hygiene and regular dental care, and avoidance of pre processed "soft" foods that may be high in sugar content (see ʻBasic Dental Careʼ below and ʻPrevention of Dental Cariesʼ below).

Recognition of oral candidiasis that may mimic or exacerbate dry mouth • symptoms (see ʻOral Candidiasisʼ below).

Various solutions --ranging from water to forms of artificial saliva-- can be used to replace oral secretions. We suggest frequent sips of water, because of convenience, low cost, and efficacy. The water does not have to be swallowed, but can be rinsed around the mouth and expectorated. Although water provides temporary moisture, it does not provide the lubricating properties that are characteristic of the mucin/water mixtures that constitute normal saliva.

Patients should be aware that too-frequent sipping of water may actually reduce the mucus film in the mouth and increase symptoms. If water consumption is excessive, especially in the evening, nocturia can occur, resulting in sleep disturbance that may worsen fatigue, cognitive difficulties, and pain that some patients experience.

We advise patients to avoid acidic beverages, which may adversely affect dental

enamel. Examples of common beverages and their relative acidity include:

–  –  –

The maintenance of pH in the oral cavity is highly important. When the pH in the oral cavity is stable, there is a decrease in the amount of demineralization that takes place. The pH and buffer capacity in the parotid saliva of individuals with Sjögren's syndrome are much lower when compared with those in normal control individuals. The buffer systems responsible for the human saliva-buffering capacity include bicarbonate, phosphate, and protein. Even a minor drop in pH can result in dental caries or damage to the teeth by erosion (Pedersen, 2005 #47).

III. Additional Needs of the SS patient from the Dentist

–  –  –

At most medical centers, and for rheumatologists in practice, communication with dentists and specialists in Oral Medicine is quite limited. However, rheumatologists need to be familiar with the terminology used by Oral Medicine in order to read the relevant literature published on SS, and to advise patients regarding particular dental procedures that may influence their medical status.

Radiation therapists have developed a close working relationship with Oral Medicine to prevent and treat oral complications, but a similar interaction for rheumatologists remain uncommon. Therefore, we will review basic terminology and approaches as a background to specific discussions listed below.

Tables 1-2 present a series of items that the rheumatologist may need to

understand in order to effectively communicate with the dentist. These items -not usually addressed in the training of internists or rheumatologists-- include:

–  –  –

Patients may require:



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