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 submandi-
bular 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
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 un-
comfortable. 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.
2 9
6 A dry mouth
Fig. 6.1 Appearance of the patients anterior teeth.
Fig. 6.2 Appearance of the patients tongue.
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A D R Y M O U T H
Type of xerostomia Common causes
False Mouth breathing
Mucosal disease
Psychological
True Drugs
Dehydration
Sjgrens syndrome
Irradiation
Neurological
Developmental anomaly
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.
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.
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.
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.
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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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.
The results of this patients investigations are:
Salivary culture 10 000 cfu Candida sp./ml
Smear for candida Hyphae present
Red cell indices Normal
White cell count/differential count Normal
Platelets Normal
ESR 20 mm/hour
Ig levels Normal
Autoantibodies
Rose Waaler Negative
RA Iatex Negative
Antinuclear Weak positive
Antithyroid Negative
ssA Positive
ssB Positive
Urine glucose Normal
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.
A D R Y M O U T H 6
3 1
Table 6.1 Investigations for patients with Sjgrens syndrome
Sample Test Relevance
Saliva Whole salivary flow rate See above; differentiates false from true xerostomia
Culture for candidal count To exclude superimposed candidosis
Stimulated parotid flow Accurate estimation of maximum possible parotid salivary flow
Blood tests Full blood picture Mild anaemia is common in all autoimmune conditions and may require treatment.
Erythrocyte sedimentation Relatively nonspecic but raised in inflammatory conditions, useful for monitoring their activity
rate (ESR) after treatment.
Immunoglobulin levels Often raised in autoimmune disorders and may be markedly raised in primary Sjgrens syndrome.
Autoantibody screen 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.
Urine Glucose Occasionally useful to exclude unsuspected diabetes as a cause of dehydration.
Salivary Sialogram In established disease a sialogram almost always shows characteristic changes.
gland Other imaging techniques 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.
Minor salivary gland biopsy 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).
Parotid gland biopsy 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.
Eye Schirmer test 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.
Ophthalmological Examination by an ophthalmologist using a slit lamp will detect conjunctival splits and Rose Bengal
examination 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.
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A D R Y M O U T H
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.
How do you interpret these histological
appearances?
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.
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.
6
3 2
Fig. 6.3 Parotid sialogram.
Fig. 6.4 Minor salivary gland biopsy; low power.
Fig. 6.5 Minor salivary gland biopsy; high power.
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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.
A D R Y M O U T H 6
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