Diseases of salivary glands

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A Sialadenitis Inflammatory disorders of the major salivary glands are usually the result of bacterial or viral infection but occasionally sialadenitis is due to other causes, such as trauma, irradiation, and allergic reactions. i) Bacterial sialadenitis Bacterial sialadenitis may present as an acute or chronic condition. a) Acute bacterial sialadenitis This uncommon disorder principally involves the parotid gland. The bacteria reach the gland from the mouth by ascending the ductal system, the main organisms involved being Streptococcids pyogenes and Staphylococcus aureus. Less commonly. Haemophilus species or members of the blackpigmented bacteroides group may be isolated. Reduced salivary flow is the major predisposing factor, and acute parotitis may occur in patients with Sjogren syndrome or following the use of drugs with xerostomic side-effects. Acute infection may also arise in immunocomptomised patients. The onset of acute sialadenitis is rapid. Clinically, it presents as swelling of the involved gland accompanied by pain, fever, malaise, and redness of the overlying skin. Pus may be expressed from the affected duct. b) Chronic bacterial sialadenitis Chronic sialadenitis is usually a non-specific inflammatory disease associated with duct obstruction, most often due to salivary calculi and law-grade ascending infection. The submandibular gland is much more commonly involved than the parotid gland. The predisposing factor may be a disorder of secretion resulting in decreased salivary flow. The symptoms of recurrent tender swelling of the affected gland are mainly related to the associated obstruction. The duct orifice may appear inflamed. Histological examination shows a. Varying degrees of dilatation of the ductal system, b. Hyperplasia of duct epithelium, c. Periductal fibrosis, d. Acinar trophy with replacement fibrosis, e. Chronic inflammatory cell infiltration.

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ii) Viral sialadenitis Mumps (epidemic parotitis) Mumps is an acute, contagious infection which often occurs in minor epidemics and is caused by a patamyxovirus. It is the commonest cause of parotid enlargement and the commonest of all the salivary gland diseases. It is most common in childhood. The virus is transmitted by direct contact with infected saliva and by droplet spread. Non-specific prodromal symptoms of fever and malaise are followed by painful swelling of sudden onset involving one or more salivary glands. The parotid glands are almost always involved, bilaterally in about 70 per cent of cases. The salivary gland enlargement gradually subsides over a period of about 7 days. The virus is present in the saliva 2 - 3 days before the onset of sialadenitis and for about 6 days afterwards. Diagnosis a) The diagnosis of murp is usually made on clinical grounds, but in atypical cases can be confirmed by the detection of IgM class antibodies and by the rise in serum antibody titre to mump virus antigens. iii) Postirradiation sialadenitis Radiation sialadenitis is a common complication of radiotherapy and there is a direct correlation between the dose of irradiation and the severity of the damage. The latter is often irreversible leading to fibrous replacement of the damaged acini and squamous metaplasia of ducts. iv) Sarcoidosis Sarcoidosis is an uncommon chronic disease of unknown cause, in which granulomas form particularly in the lungs, lymph nodes (especially the hilar nodes), salivary gjands and other sites. Clinical features a) Oro-facial features include: i) Cervical lymphadenopathy ii) Occasionally enlarged salivary glands iii) Xerostomia iv) Mucosal nodules v) Gingival hyperplasia vi) Labial swelling. b) Systemic features include: i) Erythema nodosum ii) Lymphadenopathy iii) Lung involvement

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Diagnosis and Management a) Specialist referral is indicated if this multisystem disease is suspected. b) In over 50% of patients with bilateral hilar lymphadenopathy. c) Typical granulomas which are non-caseating, contaim multinucleated giant cells and are surrounded by lymphocytes. d) Lymphadenopathy can be revealed by chest radiography or gallium scan. Galllium is taken up by macrophages in the granulomas; a scan may show uptake in involved lymph nodes. e) Patients with only minor symptoms of sarcoidosis often reguire no treatment. f) Corticosteroids are used if there is active disease of the lungs or eyes, cerebral involvement, or other serious complications such as hypercalcarmia. B Obstructive and traumatic lesions Duct obstruction and trauma are important factors in the aeriology of a number of salivary gland diseases, such as chronic sialadenitis in major glands. Duct obstruction may be due to a blockage within the lumen or result from disease in or around the duct wall, such as fibrosis or neoplasia. It can involve any part of the ductal tree. Obstruction to the duct orifice is usually due to chronic trauma. i) Salivary calculi (sialoliths) Salivary calculi cause obstruction within the duct lumen and can occur at any age, but are most common in middle-aged adults. Calculi may form in ducts within the gland or in the main excretory duct, The submandibular gland is most frequently involved, accounting for about 70 - 90 per cent of cases. The parotid gland is the next most commonly involved. The typical signs and symptoms of calculi associated with major glands are pain and sudden enlargement of the gland, especially at meal times when salivary secretion is stimulated. The reduction in salivary flow predisposes to ascending infection and chronic sialadenitis. The calculi may be detected by palpation and on radiographs, and may be round or ovoid, rough or smooth, and vary considerably in size. They are usually yellowish in colour and comprise mainly calcium phosphates. Aetiology and pathogenesis a) They are largely unknown. b) It is generally thought that they form by deposition of calcium salts around an initial organic nidus which consist of altered salivary cucins together with desquamated epithelial cells and microorganisms. c) Successive deposition of inorganic and organic material would produce a lamellated calculus.

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C Sjogren syndrome Sjogren syndrome is a chronic autoimmne disease characterized by lymphocytic infiltration and acinar destruction of lacrimal and salivary glands, leading to dry eyes and dry mouth. The syndrome may occurs in association with another autoimmune disease, most frequently rheumatoid arthritis or systemic lupus erythematosis. i) Primary Sjogren syndrome - the combination of dry mouth (xerostomia), and dry eyes (xerophthalmia or keratoconjunctivitis sicca); ii) Secondary Sjogren syndrome - the triad of xerostomia, xerophthalmia, and an autoimmune connective tissue disease (usually rheumatoid arthritis). Primary Sjogren syndrome is also known as the sicca syndrome. In addition to xerosromia and xerophthalmia, Sjogren syndrome call present with a wide spectrum of clinical features involving abnormalities of other exocrine glands and a variety of extraglandular manifestations. Sjegren syndrome predominantly affects middle-aged females. Clinical features i) Xerostomia may be associated with difficulty in swallowing and speaking, increased fluid intake, and disturbances of taste. ii) In addition, it predisposes to oral candidosis, bacterial sialadenitis, and dental caries. iii) The oral mucosa appears dry, smooth, and glazed; lingual changes may be prominent, the dorsum of the tongue often appearing red and atrophic and showing varying degrees of fissuring and lobulation. iv) Salivary gland enlargement is very variable. The enlargement is usually bilateral, predominantly affects the parotid glands, and is; seldom painful. Lacrimal gland enlargement is uncommon. Histological examination of involved major glands shows i) Lymphocyric infiltration, initially around intralobular ducts which eventually replaces the whole of the affected lobules. ii) Acinar atrophy iii) Ductal epithelium may show proliferation. iv) The hypoplasia of the duct lumen leading to islands of epithelial tissue in a sea of lymphocytes. These appearances are described by the term benign lymphoepithelial lesion i) The benign lymphoepithelial lesion is characteristic but not pathogenomic of Sjogren syndrome. ii) It also develops in the sialadenitis associated with hepatitis C virus infection, and in HIV-associated salivary gland disease.

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Other investigations useful in assessing the degree of salivary and involvement include estimation of parotid salivary flow rates, which are usually reduced, and sialography, which shows varying degrees of sialectasis. The detection of anti-Ro and anti-La antibodies is another important investigation in establishing a diagnosis of Sjogren syndrome. Although the immunological and histopathological findings in Sjogren syndrome support an autoimmune pathogenesis, genetic factors are thought to be important in increasing the susceptibility of an individual to external environmental factors. Malignant change usually occurs late in the course of the disease and may be associated with increased swelling of the affected gland.

D Sialadenosis (Sialosis) It is a rare benign, non-inflammatory, non-neoplastic bilaterally symmetrical and painless enlamement of salivary glands which usually affects the parotids. Aetiology i) Many cases are idiopathic, dysfunction the autonomic innervation of the salivary glands being the unifying factor in all. ii) The main causes include: Sympathomimetic drugs such as isoprenaline; Alcohol abuse with or without accompanying liver cirrhosis; Endocrine changes, including diabetes mellitus, acromegaly, thyroid disease and pregnancy; Malnutrition in starvation, anorexia, nervosa, bulimia and cystic fibrosis. Clinical features i) Salivary gland swelling: soft, painless, generally bilateral (usually the parotids) ii) No xerostomia iii) No trismus iv) No fever. E Salivary neoplasms Aetiology The aetiology of salivary neoplasms is unclear. i) Viruses may be involved ii) Polyoma viruses iii) EpsteinBarr virus iv) Irradiation Clinical features i) Tumours of the major salivary glands mostly: Present as unilateral swelling of the parotid Are benign Are pleomorphic adenomas. Carcinoma arises in a long standing pleomorphic salivary adenoma.

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ii) The rule of nines is an approximation that states that 9 out of 10 tumours affect the parotid, 9 out of 10 are benign, and 9 out of 10 are pleomorphic salivary adenoma PSAs) iii) Intra-oral salivary gland neoplasms are: Less common than in major glands More often malignant Typically unilateral Mainly pleomorphic adenoma, but adenoid cystic carcinoma and mucoepidermoid carcinoma are relatively more common in the mouth than in the major glands. Most common in the palate but may be seen in the buccal mucosa or upper lip, and the tongue or lower lip. Malignant Potential i) Most tumours in the parotid gland are PSAs and benign. ii) Most submandibular and tumours are PSAs and benign but one-third are malignant. iii) Most sublingual gland tumours are malignant. iv) Tumours of the tongue are usually malignant - especially adenoid cystic carcinoma. v) Tumours on the lips are generally benign (pleomorphic or other adenoma) and seen in the upper lip. Classification i) Adenomas The pleomorphic salivary adenoma a) It is the most common salivary gland neoplasm, usually slow-growing and benign. b) The tumour is poorly encapsulated and parotid adenomas are in intimate relationship with the facial nerve. c) Most pleomorphic adenomas are lobulated, rubbery swellings with normal overlaying skin or mucosa but a bluish appearance if inside the mouth. d) They are not fixed to deeper tissues. e) Malignant change is uncommon but is suggested clinically by: i) Rapid growth ii) Pain iii) Fixation to deep tissues iv) Facial palsy Monomorphic adenomas have a uniform cellular structure epithelial elements. They include: a) Adenolymphoma. It is found only in the parotid and is benign, and may be bilateral. b) Oxyphil adenoma. This rare neoplasm is found virtually only in the parotid, affects mainly the elderly and is benign.

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ii) Mucoepidermoid Tumours This tumour is usually slow-growing, of low grade malignancy or is benign. iii) Acinic Cell Tumours They are very rare and usually benign, though all grades of malignancy have been reported. iv) Carcinomas Salivary carcinomas are uncommon, malignant, and usually adenoid cystic or adenocarcinomas. a) Adenoid cystic carcinoma is a slow-growing malignant tumour which infiltrates perineurally and metastasizes. b) Adenocarcinoma is a rapidly growing tumour of more malignant behaviour than adenoid cystic carcinoma c) Epidermoid carcinoma is often an undifferentiated, higly malignant tumour Diagnosis and Management of Salivary Neoplasms i) A swelling of a salivary gland, especially if localized, firm and persistent, may be a neoplasm and it would be prudent to seek a specialist opinion. ii) A long history of gradual gland enlargement suggests a benign process, while pain or facial nerve palsy is ominous and suggests carcinoma. iii) Sialography may reveal an obvious filling defect or displacement of the gland. iv) Ct scanning is a more sensitive. v) Ultrasonography has a limited application. vi) Early detection carries a good prognosis because most tumours metastasize late. vii) Some tumours, such as adenoid cystic carcinoma, invade bone and neural tissues preferentially. viii) The treatment is surgical excision; radiotherapy is sometimes an adjunct.