• Sjögren syndrome is a chronic inflammatory disease that affects salivary, lacrimal and other exocrine glands.
• Sjögren syndrome is the expression of an autoimmune process that results principally in dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia) owing to lymphocyte-mediated destruction of lacrimal and salivary gland parenchyma.
• Primary Sjögren syndrome (sicca complex): only dry eyes and dry mouth.
• Secondary Sjögren syndrome: primary Sjögren + systemic lupus erythematosus, polyarteritis nodosa, polymyositis, scleroderma or rheumatoid arthritis.
• The specific cause of this syndrome is unknown, numerous immunologic alterations indicate a disease of great complexity.
• This syndrome appears to be of autoimmune origin that may be limited to exocrine glands, or it may extend to include systemic connective tissue disorders.
• Viruses, particularly retroviruses and Epstein–Barr virus, have been implicated in the aetiology of Sjögren syndrome, but none are proven causes.
• Sjögren syndrome occurs in all ethnic and racial groups. The peak age of onset is 50 years, and 90% of cases occur in women.
• Typical features are dryness of mouth and eyes as a result of hypofunction of the salivary glands and lacrimal glands.
• Painful, burning sensation of the oral mucosa.
• Other secretory glands involved in dryness are of the nose, larynx, pharynx, tracheobronchial tree and the vagina.
• The chief oral complaint in Sjögren syndrome is xerostomia, which may be the source of eating and speaking difficulties.
• These patients are also at greater risk for dental caries, periodontal disease and oral candidiasis because of dry mouth.
• Parotid gland enlargement, which is often recurrent and symmetric, occurs in approximately 50% of patients.
• A significant percentage of these patients also present with complaints of arthralgia, myalgia and fatigue.
• There is an increased risk of lymphoreticular malignancy developing in the primary form, the relative risk is estimated to be approximately 44 time that in the general population. An interesting associated sign is a decrease in serum immunoglobulin levels accompanying or preceding the malignant change.
• In the sicca complex, there is parotid gland enlargement that is usually absent in secondary Sjögren syndrome.
• The salivary component of Sjögren syndrome maybe assessed by sialochemical studies, nuclear imaging of the glands (scintigraphy), contrast sialography, flow rate analysis, and a minor salivary gland biopsy.
• Sialochemistry studies have shown increased levels of IgA, potassium and sodium in the saliva.
• The most commonly used and most reliable method of assessing salivary alteration in this syndrome currently is a labial salivary gland biopsy.
• Nuclear medicine techniques using a technetium pertechnetate isotope and subsequent scintiscanning can yield functional information relative to the uptake of the isotope by salivary gland tissue.
• Contrast sialography aids in detecting filling defects within the gland being examined.
• A punctate sialectasia is characteristic in individuals with Sjögren syndrome. This finding reflects significant ductal and acinar damage.
• Other laboratory findings commonly found in primary and secondary Sjögren syndrome include mild anaemia, leukopenia, eosinophilia, an elevated ESR, and diffuse elevation of serum immunoglobulin levels.
• In addition, numerous autoantibodies maybe found, including rheumatoid factor, antinuclear antibodies, and precipitating antinuclear antibodies such as antiSjögren syndrome-A (SS-A) and antiSjögren syndrome-B (SS-B) in association with both primary and secondary Sjögren syndrome.
• Patients who have SS-B antibodies are more likely to develop extra-glandular disease.
• HLA-DR4 antigen is often identified in patients with secondary Sjögren syndrome; antigens found in patients with the primary form are often HLA-B8 and HIA-DR3 types.
• Three types of histological patterns are seen in the major salivary glands:
a. Intense lymphocytic infiltration of the gland replacing all the acinar structures.
b. Proliferation of the ductal epithelium and myoepithelium to form ‘epimyoepithelial islands’.
c. Atrophy of the glands following the lymphocytic infiltration.
• Similar changes were seen in the accessory salivary glands in the lips.
• Sjögren syndrome and the complication of the sicca component are best managed symptomatically.
• Artificial saliva and artificial tears are available for this purpose.
• Preventive oral measures are extremely important relative to xerostomia. Scrupulous oral hygiene, dietary modification, topical fluoride therapy and remineralizing solutions are important in maintaining oral and dental tissues.
• Use of sialogogues, such as pilocarpine and cevimeline, remains of limited value, especially in long-standing Sjögren syndrome.
• The prognosis of Sjögren syndrome is complicated by an association with malignant transformation to lymphoma. This may occur in approximately 6–7% of cases, it is more common in those with only the sicca component of the syndrome.
Q. 2. Describe in detail sialography and its significance in various diseases of salivary glands.
Describe sialography in detail and write briefly on its significance in various salivary gland disorders.
Describe the procedure for sialography of parotid gland.
Describe the indications and contraindications of sialography. Describe the technique briefly.
• Sialography is a technique in which ducts and ductules of the salivary glands are demonstrated radiographically after a radiopaque liquid has been injected along them.
• First performed in 1902, sialography is a radiographic technique wherein a radiopaque contrast agent is infused into the ductal system of a salivary gland before imaging with plain films, fluoroscopy, panoramic radiography, conventional tomography or CT.
• Sialography remains the most detailed way to image the ductal system. The parotid and submandibular glands are more readily studied with this technique.
• To demonstrate—calculi, strictures, recurrent parotitis, tumours, etc.
• Salivary fistula.
• Relationship of salivary glands and ducts to surrounding structures.
• Autoimmune or radiation-induced sialadenitis.
• Active or recent infection of the gland.
• Allergy to contrast media.
• A surveyor ‘scout’ film is usually made before the infusion of the contrast solution into the ductal system as an aid in verifying the optimal exposure factors and patient positioning parameters and for detecting radiopaque sialoliths or extra glandular pathosis.
• A lacrimal or periodontal probe is used to dilate the sphincter at the ductal orifice before the passage of a cannula (blunt needle or catheter) connected by extension tubing to a syringe containing contrast agent.
• Lipid-soluble (e.g., ethiodol) or nonlipid-soluble (e.g., Sinografin) contrast solution is then slowly infused until the patient feels discomfort (usually between 0.2 and 1.5 mL, depending on the gland being studied).
• These iodine containing agents render the ductal system radiopaque.
• The filling phase can be monitored by fluoroscopy or with static films.
• The intention is to opacify the ductal system all the way to the acini. The image of the ductal system appears as ‘tree limbs’, with no area of the gland devoid of ducts.
• With acinar filling, the ‘tree’ comes into ‘bloom’, which is the typical appearance of the parenchymal opacification phase.
• The gland is allowed to empty for 5 minutes without stimulation.
• If postevacuation images suggest contrast retention, a sialogogue such as lemon juice or 2% citric acid may be administered to augment evacuation by stimulating secretion.
• Nonlipid-soluble contrast agents are preferred because of reports of inflammatory reactions subsequent to inadvertent extravasation of lipid-soluble agents.
• Visualizes ductal anatomy/blockage.
• Invasive; requires iodine-containing dye; no quantification.
Q. 3. Clinical features, differential diagnosis and management of functional disturbance of salivary glands.
Enumerate the clinical and radiological features of functional disturbances of salivary glands.
Ans. The two types of functional disorders of salivary glands are as follows:
a. sialorrhoea and
• Sialorrhoea describes increased salivary flow.
• Painful lesions or foreign bodies in the mouth.
• Drugs, e.g., anticholinesterases, insecticides and nerve agents; antipsychotics, and cholinergic agonists used to treat dementia and myasthenia gravis).
• Toxins (e.g., mercury and thallium); and rarely other causes, e.g., rabies may be implicated.
• Sialorrhoea is an uncommon subjective complaint but objective evidence is even less common, and the problem is sometimes perceived rather than real.
• Drooling is the overflowing of saliva from the mouth not usually associated with increased saliva production.
• Drooling is normal in healthy infants, but usually stops by about 18 months and is considered abnormal if it persists beyond the age of 4 years.
• Saliva soils clothing and patients may have perioral skin breakdown and infections, disturbed speech and eating and can occasionally develop aspiration related and pulmonary complications.
• Absolute quantification of saliva spill or intraoral pooling by volumetric measurement can help guide treatment. A subjective estimate can be made by counting the bibs or items of clothing soiled each day.
• Management options range from conservative therapy to medication, radiation, or surgery, and often a combination is needed.
• Pharmacological treatment (anticholinergic drugs, e.g., atropinics such as hyoscine or ipratropium or adrenergic stimulators, e.g., clonidine) decreases salivation.
• Botulinum toxin serotype A injections may have a positive outcome.
• Persistent drooling is managed by redirecting the submandibular duct flow to the back of the mouth; or duct ligation (mainly parotid); or gland removal or neurectomy
• Xerostomia means dryness of mouth.
• Various drugs capable of causing xerostomia are as follows:
iii. Anticholinergic drugs
vi. Selective serotonin reuptake inhibitors (SSRIs)
vii. Tricyclic and heterocyclic antidepressants
viii. Atypical antidepressants
ix. Antihypertensive agents
• Muscle relaxants
• Autoimmune or systemic diseases:
• Sjögren syndrome: both primary and secondary
• Primary biliary cirrhosis
• Wegener’s granulomatosis
• Other conditions:
• Local radiation therapy
• Diabetes: both type 1 and 2
• Radioactive iodine treatment
• Human immunodeficiency virus (HIV)/acquired immunodeficiency syndrome (AIDS)
• Increased thirst and hence increased uptake of fluids especially.
• Burning and tingling sensation in the mouth.
• Difficulty in swallowing.
• Painful salivary gland enlargement.
• Swelling of salivary glands.
• Increased incidence of dental caries.
• Angular cheilitis.
• Oral infections.
• Management of the patient with xerostomia is generally directed towards palliation and requires a careful multifactorial approach, wherein local and systemic factors are considered, along with analysis of all prescription and over-the-counter medications and diet.
• Direct interventional strategies include the use of topical agents such as oral polymer-based sprays, so-called saliva substitutes, sipping of small amounts of water during the day.
• Gustatory salivary stimulation.
• Drug modification when possible.
• Elimination of caffeine-containing products.
• Chewing of sugarless candies and sugar-free gum.
• Moist sugar-free or complex carbohydrate foods.
• Elimination of alcohol-containing mouth rinses.
• Scrupulous oral hygiene.
• Topical fluoride application.
• Careful dental follow-up are required to help prevent or control dental caries.
• Oral lubricants, e.g., carboxymethylcellulose- or hydroxymethyl cellulose-based products.
• In some cases, prescription strategies include cholinergic agonists like pilocarpine and cevimeline may be helpful, as may the use of acupuncture.
Q. 4. Name the various diseases of salivary glands. Discuss clinical features, diagnosis, differential diagnosis and treatment of parotitis.
Diseases of salivary glands
• Mucus extravasation phenomenon
• Mucus retention cyst (obstructive sialadenitis)
• Maxillary sinus retention cyst/pseudocyst
• Necrotizing sialometaplasia
• Adenomatoid hyperplasia.
• Cytomegaloviral sialadenitis
• Bacterial sialadenitis
• Sjögren syndrome
• Salivary lymphoepithelial lesion
• Taste disturbances
• Mixed tumour (pleomorphic adenoma)
• Basal cell adenoma
• Canalicular adenoma
• Oncocytic tumours
• Sebaceous adenoma
• Ductal papilloma.
• Mucoepidermoid carcinoma
• Polymorphous low-grade adenocarcinoma
• Adenoid cystic carcinoma
• Clear cell carcinoma
• Acinic cell carcinoma
• Adenocarcinoma not otherwise specified.
• Carcinoma ex-mixed tumour
• Metastasizing mixed tumour
• Epimyoepithelial carcinoma
• Salivary duct carcinoma
• Basal cell adenocarcinoma
• Squamous cell carcinoma.
• Bacterial parotitis occurs both in a childhood form and in an adult form. In either form, the gland becomes swollen and painful.
• Repeated parotitis and sialectasis in a child, associated with a sialographic pattern of sialectasis.
• Prevalence (approximate): uncommon.
• Age mainly affected: usually begins in preschool children.
• Gender mainly affected: Male.
• Aetiopathogenesis: congenital or autoimmune duct defects.
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