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A dry mouth

Summary A 50-year-old lady presents to you in your hospital dental department complaining of dry mouth. Identify the cause and plan treatment. HISTORY Complaint She complains of dryness which makes many aspects of her life a misery. The dryness is both uncomfortable and renders eating and speech difcult. She is forced to keep a bottle of water by her side at all times. History of complaint She rst noticed the dry mouth about 4 or 5 years ago though it may have been present for longer. At rst it was only an intermittent problem but over the last 3 years or so the dryness has become constant. Recently the mouth has become sore as well as dry. Medical history The patient describes herself as generally t and well but has had to attend her medical practitioner for poor circulation in her ngers. They blanch rapidly in the cold and are painful on rewarming. She has also used articial tears for dry eyes for the last 2 years but takes no other medication. EXAMINATION Extraoral examination She is a well-looking lady without detectable cervical lymphadenopathy. There is no facial asymmetry or enlargement of the parotid glands and the submandibular glands appear normal on bimanual palpation. Her eyes and ngers appear normal. Intraoral examination The appearance of the patients mouth is shown in Figures 6.1 and 6.2. What do you see? How do you interpret the ndings?
The alveolar mucosa appears glazed and translucent or thin (atrophic) suggesting long-standing xerostomia. Some

Fig. 6.1 Appearance of the patients anterior teeth.

Fig. 6.2 Appearance of the patients tongue.

oral debris adheres between the teeth, again suggesting dryness, which causes plaque to be thicker and more tenacious. There are carious lesions and restorations at the cervical margins of the lower anterior teeth, indicating a high caries rate. The tongue is lobulated and ssured. Both features suggest a lack of saliva.

If you were able to examine the patient you would nd that her mouth does feel dry. Gloved ngers and mirror adhere to the mucosa making examination uncomfortable. Parts of the mucosa, especially the palate and dorsal tongue appear redder than normal. No saliva is pooling in the floor of the mouth and what saliva can be identied is frothy and thick. Small amounts of clear but viscid saliva can be expressed from all four main salivary ducts. What are the common and important causes of xerostomia and how are they subdivided?
In true xerostomia the salivary flow is reduced. The term false xerostomia describes the sensation of dryness despite normal salivary output.
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A DRY MOUTH

Type of xerostomia False

Common causes Mouth breathing Mucosal disease Psychological Drugs Dehydration Sjgrens syndrome Irradiation Neurological Developmental anomaly

What is Sjgrens syndrome and how may the condition be subclassied?

Sjgrens syndrome is a poorly understood autoimmune disorder in which exocrine glands are destroyed. In primary Sjgrens syndrome the salivary and lacrimal glands are those most affected (though there are often nonspecic systemic signs of autoimmune disease such as Raynauds phenomenon) and there is sometimes salivary gland swelling. Other exocrine glands and organs are also affected. In secondary Sjgrens syndrome there is an accompanying connective tissue disorder such as rheumatoid arthritis, systemic lupus erythematosus or mixed connective tissue disease. Other exocrine glands are less severely affected in the secondary form, the mouth is usually less dry and salivary glands are very rarely enlarged.

True

On the basis of the history and examination which cause is the most likely? Why?
Sjgrens syndrome is the most likely cause. It is the commonest single medical disorder causing xerostomia. It also causes dry eyes and predominantly affects female patients of middle age. Sjgrens syndrome is sometimes dened by the presence of dry eyes and mouth, with or without an autoimmune/connective tissue disorder. This patient meets these criteria though they are rather imprecise and further investigations would be required to conrm the diagnosis.

INVESTIGATIONS What simple test differentiates false and true xerostomia?

Measuring the whole salivary flow rate. This may be done by asking the patient to tilt their head forward to allow all saliva to flow into a graduated specimen container for 10 minutes. Although this patient is strongly suspected to have true xerostomia it would still be a useful test because it provides a baseline reading against which disease severity and progression may be judged.

Which causes have you excluded and why?

Drugs are by far the commonest cause of true xerostomia but this patient is not taking any medication. Dehydration is a common cause in elderly people who may have a habitual low fluid intake, especially when institutionalized. It also accompanies cardiac or renal failure or diuretic drugs. (The combination of drugs and disease probably explains the apparent association of xerostomia with age). These are not factors in this case. False xerostomia is very common. Those who sleep with an open mouth will have xerostomia on waking, compounded by the normal reduction in salivary secretion at night. Diseases causing oral mucosal roughness such as lichen planus or candidosis may cause a sensation of dryness but no such condition is present. False xerostomia may be a feature, sometimes a central one, in neurosis and psychosis. However, this patients mouth is genuinely dry. The history of prolonged and unremitting dryness over a period of years almost always indicates a salivary disorder and the appearance of the mucosa and the high caries rate indicate true xerostomia. Neurological and developmental causes, such as aplasia of gland or atresia of ducts, are very rare and need not be considered further until common causes have been investigated. There is no history of irradiation of the head and neck.
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When you measure the flow, the patient has a whole salivary flow rate of 0.1 ml/minute. What salivary flow rate would you consider to indicate xerostomia?
Approximately 500 ml of saliva are secreted daily, mostly during eating and drinking, and very little at night. Rates vary greatly between individuals but less than 2 ml in 10 minutes (0.2 ml/minute) unstimulated whole saliva flow is generally considered to indicate xerostomia. This patient has true xerostomia.

What further investigations are required and why is each performed?

Although a number of investigations will be required to conrm the diagnosis, the immediate problem is one of soreness. A dry mouth is not usually sore unless there is superimposed candidal infection. Smears, a saliva sample or a therapeutic trial of antifungal agent are required to exclude this possibility. The diagnosis of Sjgrens syndrome is straightforward when the clinical presentation is florid, and may then be based on history and examination alone. However, numerous investigations are required in most patients with

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Table 6.1 Investigations for patients with Sjgrens syndrome Sample Saliva Test Whole salivary flow rate Culture for candidal count Stimulated parotid flow Full blood picture Erythrocyte sedimentation rate (ESR) Immunoglobulin levels Autoantibody screen Relevance See above; differentiates false from true xerostomia To exclude superimposed candidosis Accurate estimation of maximum possible parotid salivary flow Mild anaemia is common in all autoimmune conditions and may require treatment. Relatively nonspecic but raised in inflammatory conditions, useful for monitoring their activity after treatment. Often raised in autoimmune disorders and may be markedly raised in primary Sjgrens syndrome. Autoantibodies are a frequent nding in autoimmune disease. This appears to be a partly nonspecic effect and many different autoantibodies may be seen. The exact combination in the routine screen varies between centres but usually includes rheumatoid factor, antinuclear, antithyroid, antiparietal cell and antimitochondrial antibody. Additional autoantibodies which may be seen in Sjgrens syndrome are antisalivary gland duct antibody and ssA and ssB autoantibodies (anti-Ro and anti-La) directed against extractable nuclear antigens. None of these antibodies is individually helpful in diagnosis but the presence of more than one is typical. They may help diagnosis of connective tissue disease in secondary Sjgrens syndrome and ssA and ssB may indicate patients at risk of specic complications. Antisalivary gland duct antibody is not related to either the periductal inltrates seen on biopsy or the pathogenesis of the disease. Occasionally useful to exclude unsuspected diabetes as a cause of dehydration. In established disease a sialogram almost always shows characteristic changes. Pertechnetate scintigraphy is a complex but useful test of secretion from individual glands. It is useful if sialography is not possible but involves a signicant radiation dose. Magnetic resonance imaging is useful to delineate the extent of salivary gland swelling if present. The histological appearances of salivary glands are characteristic in established disease. Biopsy of major glands is difcult but the same changes may be seen in the minor glands of the lips and cheeks provided a sufcient sample is removed (68 glands). Biopsy of the tail of the parotid is possible without signicant risk to branches of the facial nerve. It provides an excellent sample and may be useful when other techniques have failed or when other conditions need to be excluded. It may also be helpful in the diagnosis of lymphoma in swollen parotid glands. However, it is rarely performed. This measures lacrimal secretion. Narrow lter paper strips are placed with one end under the lower eyelid and the length wetted after several minutes is recorded. In practice the test is not very reproducible. It is also uncomfortable and may cause corneal abrasions when the eye is very dry and for this reason is no longer recommended. Ophthalmological examination is preferable. Examination by an ophthalmologist using a slit lamp will detect conjunctival splits and Rose Bengal staining identies dried tear secretion on the front of the eye. Though these changes are rarely helpful in diagnosis, examination and follow up are required to prevent long-term complications of dry eyes.

Blood tests

Urine Salivary gland

Glucose Sialogram Other imaging techniques

Minor salivary gland biopsy

Parotid gland biopsy

Eye

Schirmer test

Ophthalmological examination

suspected Sjgrens syndrome in whom there are just a few early signs (Table 6.1). Many investigations are possible but only the minimum required to make the diagnosis need be performed. A selection is usually necessary because every test will be negative in a small proportion of patients and none is completely specic.

Antinuclear Antithyroid ssA ssB Urine glucose

Weak positive Negative Positive Positive Normal

The results of this patients investigations are:

Salivary culture Smear for candida Red cell indices White cell count/differential count Platelets ESR Ig levels Autoantibodies Rose Waaler RA Iatex 10 000 cfu Candida sp./ml Hyphae present Normal Normal Normal 20 mm/hour Normal Negative Negative

The parotid sialogram is shown in Figure 6.3. What do you see? What is your interpretation?
The sialogram shows punctate sialectasis. The major duct is seen but almost no major or minor duct branches are visible. Small round spots of contrast medium are scattered throughout the gland, apparently unconnected with the duct tree. These features have some similarities to those in chronic nonspecic sialadenitis but are much more even and affect the whole gland equally. These features are characteristic of Sjgrens syndrome.
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A DRY MOUTH

Fig. 6.5 Minor salivary gland biopsy; high power.

How do you interpret these histological appearances?

Fig. 6.3 Parotid sialogram.

The focal lymphocytic sialadenitis centred on ducts and concentric sharply dened zones of acinar atrophy surrounded by normal acini are characteristic of Sjgrens syndrome.

DIAGNOSIS What is your nal diagnosis?

The patient has primary Sjgrens syndrome. The diagnosis was suspected on the basis of history and examination, and is conrmed by the characteristic sialogram and biopsy ndings. The primary form of Sjgrens syndrome is indicated by the lack of autoimmune/connective tissue disease and the positivity for ssA and ssB autoantibodies. The presence of Raynauds phenomenon, the severity of the xerostomia and dryness of the eyes are also more consistent with the primary form. In addition the patient has candidosis which is the probable cause of the soreness.

Fig. 6.4 Minor salivary gland biopsy; low power.

The minor salivary gland biopsy is shown in Figures 6.4 and 6.5. What do you see?
The low power view shows several minor salivary glands. A minimum of 68 glands is required for reliable diagnosis and this sample is sufcient. Even at this low magnication, dark foci of inflammatory cells are visible (though they cannot be identied as such) and it can be seen that the lobular structure of the glands is largely intact. The high power view shows one gland lobule. Centrally there are three small ducts surrounded by a dense lymphocytic inltrate. The inltrate is sharply dened and within the lymphocytic focus there is complete loss of acinar cells (acinar atrophy). Around the lymphocytes there is a zone of essentially normal uninflamed mucous salivary gland.
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TREATMENT How could you contribute to the management of this patient?

Control of the underlying disease is not possible but the patient requires treatment for complications and continued follow up: Treat candidosis and follow up regularly for recurrence. Preserve what salivary secretion remains; saliva is more effective than saliva substitutes. Sip water rather than drinking it, so as to expand remaining saliva and not wash it from the mouth. Whenever possible avoid drugs which cause xerostomia.

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Maintain fluid intake. Stimulate residual salivary flow using chewing gum (sugar-free). Consider using pilocarpine in severe cases (though side-effects and an appropriate dosing regimen can be problematic). Prevent and treat dental caries Avoid sweets or overuse of citrus fruit to stimulate salivary flow. Appropriate dietary analysis, preventive advice and fluoride treatment. Treat caries. Consider using saliva substitutes though these are generally unsatisfactory and not liked by patients. Carboxymethyl-cellulose and similar starch-based liquids. Mucin-based preparations are more effective and generally better tolerated. Warn patient about, and follow up for, attacks of acute bacterial ascending sialadenitis in the major glands, which destroys residual gland function. Treat aggressively if it develops.

Ensure continued ophthalmological follow up. Inform patients general medical practitioner to ensure follow up for other complications. Involvement of other exocrine glands can lead to dry skin, dry vagina, pancreatic dysfunction and lung disease. Warn patient and follow up for development of persistent salivary gland swelling. Provide continued reassurance and care for patients with this distressing condition.

What is the signicance of the development of salivary gland swelling?

This is usually the rst sign of lymphoma development; 10% or more of patients with primary Sjgrens syndrome eventually develop lymphoma and in some cases gland swelling is the presenting sign. The lymphoma is usually a form of low grade B-cell lymphoma (MALT type) which has a slow indolent growth pattern, remains localized to the salivary glands for a long period and initially responds well to treatment. However, high grade lymphoma may also develop.

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