Degenerative conditions (Table 42.6)
Sjogren’s
syndrome
Sjogren’s syndrome is an autoimmune condition causing progressive
destruction of the salivary and lachrymal glands. In 1933 Sjogren first
described the association of keratoconjunctivitis sicca (dry eyes) and Xerostomia
(dry mouth). Shortly thereafter he noted that these symptoms
frequently
Females
are affected more often than males in the ratio of 10:1. Typically they are
middle aged. The presenting complaint is usually of the underlying connective
tissue disorder and only later does the patient become aware of a gritty feeling
in the eyes due to dry eyes or of dry mouth. Occasionally there is enlargement
of the parotid glands bilaterally and even more rarely the enlarged parotids are
painful (Fig. 42.13). Superinfection of the mouth with Candida albicans is
frequent. Less frequently the patient develops bacterial sialadenitis due to
ascending infection from the mouth. The condition does not invariably progress
to total xerostomia and for any individual patient it is nor possible to predict
the outcome. The characteristic features of the condition are progressive
lymphocytic infiltration, acinar destruction and proliferation of duct
epithelium of all salivary and lachrymal tissue.
The
diagnosis is often based on the characteristic history. No laboratory
investigation is pathognomonic of either primary or secondary Sjogren’s
syndrome. However, the following investigations are usually undertaken:
1. Sialography reveals the
progressive damage from punctate sialectasis to total parenchymal destruction
leaving no more than a grossly dilated duct (Fig.
42.14).
2. Labial salivary gland biopsy can be misleading particularly if only
one minor gland is harvested. The characteristic lymphocytic infiltration is
focal and a single gland may not show the changes. A minimum of three glands
should be submitted to the pathologist.
3. Estimation of salivary flow may be unhelpful as the normal
variation in flow rates makes the interpretation of the results difficult.
4. Viral staining of the cornea with
rose Bengal and examination of the cornea with a slit-lamp is a very sensitive
assessment of a dry eye.
5. Autoantibody screen. See
Table
42.7.
6. Blood tests usually show a moderately raised erythrocyte sedimentation rate (ESR) and a mild microcytic anaemia (the anaemia of chronic disease).
The
management of Sjogren’s syndrome must be symptomatic. No known treatment
modifies or reverses the xerostomia and keratoconjunctivitis sicca. Artificial
tears are essential to protect the cornea. For the dry mouth various artificial
saliva preparations are available but often the patient prefers to use frequent
drinks and learns to carry a bottle of water with them at all times. If patients
are to use
‘Benign’
lymphoepithelial lesion
The term ‘benign lymphoepithelial lesion’ was coined by Godwin in 1952.
Use of the word ‘benign’ to describe the lesion is misleading as
approximately 20 per cent of patients with benign lymhoepithelial lesion or
Sjogren’s syndrome ultimately develop lymphorna. Histologically it is not
possible to distinguish benign epithelial lesions from Sjogren's syndrome.
Both are characterised by lymphocytic infiltration, acinar atrophy and ductal
epithelial proliferation. Indeed they may well be manifestations of the same
condition.
Clinically
benign lymphoepithelial lesion presents as diffuse swelling of the parotid. The
swelling is firm and often painful. In 20 per cent of cases the parotid swelling
is bilateral. Eighty per cent of patients are female and most are
Mikulicz’
syndrome
In 1888 Mikulicz described benign, asymptomatic, symmetrical
enlargement of the lacrimal and salivary glands. His original publication
described a series of patients who clearly had a variety of different
conditions. Benign lymphoepithelial lesion, Sjogren’s syndrome, lymphoma,
lymphocytic leukemia, sarcoid and Sialosis can all present in this way. The
term Mikulicz’ syndrome is nor helpful and should nor be used (Fig. 42.15).
Xerostomia
A complaint of dry mouth is common. It seems to be particularly
frequent in postmenopausal women who also complain of a burning tongue or
mouth. Normal salivary flow decreases with age in both men and women. The
situation is further confused as patients with Sjogren’s syndrome are
frequently unaware of having a dry mouth and patients who complain of dry mouth
frequently have normal salivary flow rates. The most common causes of xerostomia
in order of frequency are:
• chronic anxiety states and depression;
• dehydration;
• drugs — many drugs have been implicated in causing xerostomia
as an undesirable side effect (Table 42.8);
• salivary gland diseases as described earlier.
Xerostomia
can be difficult to treat. Treatment is aimed at the relief of symptoms and the
avoidance or control of complications. Frequent sips of water help most
patients. Artificial salvias are nor well accepted but their lubricant
properties may be particularly useful at meal times. Cholinergic drugs such as
pilocarpine can be tried but their side effects — diarrhoea and pupillary
dilatation often outweigh any benefit.
Rampant
caries and destructive periodontal disease are major complications due to oral
infection. Meticulous oral hygiene and the weekly use of topical fluoride are
essential. There is a high incidence of oral candidiasis and antifungal drugs are
necessary.
Sialorrhoea
(Table 42.9)
Some drugs and painful lesions in the mouth increase salivary flow
rates. In normal health this is rarely noticed as the excess saliva is swallowed
spontaneously. ‘False ptylism’ is more common and is a well-recognised
delusional symptom or