Slide 14 Diseases of Salivary Glands I

8/3/2019 Slide 14 Diseases of Salivary Glands I

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Dent 356-11

Diseases of Salivary Glands IDevelopmental AnomaliesSialadenitisObstructive & Traumatic Lesions

Sjgren Syndrome

Dr. Rima SafadiFrom Dr. Huda Hammad Lectures


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Salivary Glands: Major


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Salivary Glands: MajorParotid Gland


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Salivary Glands: MajorSubmandibular Gland


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Salivary Glands: MajorSublingual Gland


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Salivary Glands: Minor


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Salivary Glands: Minor


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Developmental Anomalies of

Salivary Glands

Rare.

Aplasia of one or more major SG is rare.

Atresia of one or more major SG ducts.


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Salivary Glands

Aplasia of parotidgland may be

associated with otheranomalies, e.g.

- mandibulofacialdysostosis

- aplasia of lacrimalglands

- hemifacial microsomia.


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Salivary Glands

Heterotopic SGtissue: e.g. Stafnes

idiopathic bonecavity.

Accessory parotid

tissue within cheek ormasseter muscle isrelatively common.


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Sialadenitis

Bacterial:AcuteChronicRecurrent parotitis

Viral:MumpsCytomegalic inclusion disease

Postirradiation

Sarcoidosis

Sialadenitis of minor glands


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Acute Bacterial Sialadenitis

Uncommon, mostly affects parotid.

Ascending infection from mouth.

Mainly Streptococcus pyogenes&Staphylococcus aureus.

Less commonly Haemophilusspecies ormembers of the black-pigmentedBacteroidesgroup.


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Predisposing factors:

1. Reduced salivary flow, e.g. Sjgren syndrome,drugs with xerostomic side effect.

2. Used to be a common postoperativecomplication in debilitated, dehydrated patientsfollowing abdominal surgery.

3. Immunocompromized patients.

4. Acute exacerbation of chronic sialadenitis,usually submandibular gland.


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Clinical features:

1. Rapid onset.

2. Swelling of involved gland.

3. Pain, fever, malaise.

4. Redness of overlying skin.

5. Pus may be expressed fromduct.


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Chronic Bacterial Sialadenitis

In major glands, it is usually non-specific,associated with duct obstruction (salivarycalculi) & low-grade ascending infection.

Submandibular > parotid gland.

Disorder of secretion & decreased salivaryflow may also predispose to it.


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Clinical features:

1. Usually unilateral.

2. Recurrent, tender swelling ofaffected gland related toobstruction.

3. Duct orifice may appearinflamed.

4. In acute exacerbations theremay be purulent or saltydischarge.


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Histopathology:

- varying degrees of ductaldilatation.

- hyperplastic ductalepithelium.

- periductal fibrosis.

- acinar atrophy &

replacement by fibroustissue.

- chronic inflammatoryinfiltration.


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Sialographydemonstrates ductobstruction, glandulartissue destruction, &

ductal dilatation.

Progressive chronicinflammation ofsubmandibular gland mayresult in almost complete

replacement ofparenchyma by fibroustissue, which may bemistaken for a tumor

(chronic sclerosingsialadenitis).


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Recurrent Parotitis

Rare disorder of children or adults.

Rarely, adult form may follow on from childhoodtype, but mostly due to persistence ofpredisposing factors such as calculi or ductstrictures.

Possible etiology of childhood type:

1. Abnormally low secretion rate.2. Immaturity of immune response.3. Congenital abnormalities of ductal system.


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Recurrent Parotitis

Clinical features:

1. Unilateral or bilateral.

2. Recurrent painful swelling of parotid.

3. Pus may be expressed from orifice.

4. Often resolves spontaneously by early adulthood.

5. Repeated infection may cause irreversible damage tomain duct, predisposing to duct obstruction and furtherepisodes in adult life.


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Mumps (Epidemic Parotitis)

An acute, contagious infection which oftenoccurs in minor epidemics.

Caused by a paramyxovirus.

Commonest cause of parotid enlargement.

Commonest of all salivary gland diseases.
http://rds.yahoo.com/S=96062857/K=mumps+disease/v=2/SID=w/l=II/R=26/SS=i/OID=38a29ee264bbdfa8/SIG=1g9c0l4f4/EXP=1121856959/*-http:/images.search.yahoo.com/search/images/view?back=http://images.search.yahoo.com/search/images?p=mumps+disease&ei=UTF-8&fl=0&imgsz=all&fr=sfp&b=21&h=134&w=150&imgcurl=www.nald.ca/CLR/sick/graphics/page41c.GIF&imgurl=www.nald.ca/CLR/sick/graphics/page41c.GIF&size=2.9kB&name=page41c.GIF&rcurl=http://www.nald.ca/CLR/sick/page41.htm&rurl=http://www.nald.ca/CLR/sick/page41.htm&p=mumps+disease&type=gif&no=26&tt=82&ei=UTF-8


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Clinical features:

1. Most common in childhood.

2. Transmitted by direct contact with infected saliva anddroplet spread.

3. Incubation period of 2-3 weeks.

4. Prodromal symptoms of fever & malaise.
http://rds.yahoo.com/S=96062857/K=mumps+disease/v=2/SID=w/l=II/R=26/SS=i/OID=38a29ee264bbdfa8/SIG=1g9c0l4f4/EXP=1121856959/*-http:/images.search.yahoo.com/search/images/view?back=http://images.search.yahoo.com/search/images?p=mumps+disease&ei=UTF-8&fl=0&imgsz=all&fr=sfp&b=21&h=134&w=150&imgcurl=www.nald.ca/CLR/sick/graphics/page41c.GIF&imgurl=www.nald.ca/CLR/sick/graphics/page41c.GIF&size=2.9kB&name=page41c.GIF&rcurl=http://www.nald.ca/CLR/sick/page41.htm&rurl=http://www.nald.ca/CLR/sick/page41.htm&p=mumps+disease&type=gif&no=26&tt=82&ei=UTF-8


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Clinical features,contd:

5. Sudden onset ofpainful swelling ofone or more salivary

glands, mostly parotid(70%).


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Clinical features, contd:

6. Occasional involvement

of submandibular andsublingual glands, butrarely without parotidalso.

7. Enlargement graduallysubsides over a week.


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Clinical features, contd:

8. Virus is present in saliva 2-3 days before onset,and 6 days after.

9. Occasional involvement of other organs inadults; testes, ovaries, CNS.

10. Orchitis is the most common complicationaffecting 20% of affected adult males.


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Diagnosis is usually clinical, but can beconfirmed by detection of IgM antibodies

and by rise in serum titer to mumps virusantigens within the 1st week.

Immunity after an attack in long-lastingand recurrent infection is rare.


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Cytomegalic Inclusion Disease(Salivary Gland Inclusion Disease)

CMV is a member of the herpesvirus group.

Infection is common in humans worldwide.

Most primary infections are asymptomatic.

It can cause severe disseminated disease in neonatesand immunocompromised hosts e.g. transplant and HIV-

infected patients.

May be associated with xerostomia in HIV infection.


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Cytomegalic Inclusion Disease(Salivary Gland Inclusion Disease)

Histopathology:1. Salivary gland involvement is

usually an incidentalhistological finding.

2. Large, doubly contouredowl-eye inclusion bodieswithin nucleus or cytoplasmof duct cells of parotid gland.

3. In disseminated disease,similar inclusions are found inkidneys, liver, lungs, brain, &other organs.
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Postirradiation Sialadenitis

A common complication or radiotherapy.

Direct correlation between dose of irradiation andseverity of damage.

Damage is often irreversible leading to:a. fibrous replacement of damaged acinib. squamous metaplasia of ducts.

In less severe cases, some degree of function mayreturn after several months.

Serous acini are more sensitive than mucous acini.


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Sarcoidosis

May affect parotid andminor salivary glands.

Parotid involvement

presents as persistent,often painlessenlargement.

May be associated withinvolvement of lacrimalglands in Heerfordtsyndrome.


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Sialadenitis of Minor Glands

Often an incidental & insignificant finding.

May however be of diagnostic significance, suchas in sarcoidosis & Sjgren syndrome.

Most frequently seen in association with mucousextravasation cysts & nicotinic stomatitis.


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Histopathology:

- Varying degrees of ductaldilatation.

- Hyperplastic ductal epithelium.

- Periductal fibrosis.

- Acinar atrophy & replacement byfibrous tissue.

- Chronic inflammatory infiltration.


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Very rarely may present with multiple mucosalswellings associated with cystic dilatation ofducts and chronic suppuration (stomatitisglandularis).

Most commonly on the lips, probably as anacute exacerbation of a chronic form associatedwith obstruction or reduction in salivary flow

(cheilitis glandularis).


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Obstructive & Traumatic Lesions

Salivary calculi (sialoliths)

Necrotizing sialometaplasia

Mucoceles


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Salivary Calculi (Sialoliths)

Most common inmiddle-aged adults.

May form in ductswithin the gland, or in

main excretory duct.


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79-90% of cases involve submandibulargland.

Parotid gland is next, while only 2% affectsublingual or minor glands.

Usually unilateral, although may bemultiple in same gland.


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Clinical features:

1. When symptomatic, they cause pain & sudden

enlargement of affected gland, especially atmeal times when secretion is stimulated.

2. Reduction in flow predisposes to ascendinginfection & chronic sialadenitis.


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Clinical features,contd:

3. Calculi may bedetected bypalpation and onradiographs.


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Thought to form bygradual deposition ofcalcium salts around

an initial organicnidus which mayconsist of alteredsalivary mucins with

desquamated cellsand microorganisms.
http://www.usc.edu/hsc/dental/opfs/SG/076big.html


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Necrotizing Sialometaplasia

A relatively uncommondisorder.

May be mistaken

clinically andhistologically formalignant disease.

Most frequent on hardpalate in middle-agedpatients, especiallymales.


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Clinical features:

1. Presents mostcommonly as a deep,crater-like ulcer whichmay mimic a malignantulcer.

2. May take up to 10-12weeks to heal.

3. Ulcer may be precededby an indurated swelling.


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Histopathology:

1. Lobular necrosis.

2. Squamous metaplasia ofducts & acini.

3. Mucous extravasation.

4. Inflammatory cell infiltration.

5. Overlying palatal mucosa

shows pseudoepitheliomatoushyperplasia.

6. Features may be mistaken forSCC or mucoepidermoidcarcinoma.


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Etiology unknown, but ischemia leading toinfarction of salivary lobules is most widelyaccepted theory.

In some patients there may be history oftrauma, including local anesthetic injection

and previous surgery.


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Sjgren Syndrome

A chronic autoimmune disease.

Characterized by lymphocytic infiltration and acinardestruction of lacrimal and salivary glands.

Classified into:1. Primary Sjgren or sicca syndrome : dry mouth

(xerostomia) and dry eyes (xerophthalmia orkeratoconjunctivitis sicca)

2. Secondary Sjgren syndrome : xerostomia,xerophthalmia, & an autoimmune CT disease, usuallyrheumatoid arthritis.


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Sjgren Syndrome: Clinical Features

Rheumatoid arthritis is the most common CT diseaseassociated with 2ry SS.

Other autoimmune diseases that may be associated

include:*1. Systemic lupus erythematosus.

2. Systemic sclerosis.

3. Primary biliary cirrhosis.

4. Mixed CT disease.

* Not in textbook.


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Unless stated otherwise, the general termSjgren syndrome (SS) is used toencompass both types.

Both 1ry & 2ry SS exhibit a widespectrum of clinical features associated

with widespread involvement of otherglands and other tissues.


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European criteria for diagnosis of SS relate to:

i. Ocular symptoms.

ii. Ocular signs.

iii. Oral symptoms.iv. Salivary gland function.

v. Labial salivary gland histology.

vi. Ro and La autoantibodies.

4 of the 6 criteria need to be fulfilled.


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SS predominantly affects middle-agedfemales (9:1 F:M ratio).

The most common symptoms are relatedto xerostomia and xerophthalmia.

In general, ocular & oral manifestationsare more severe in 1ry SS.


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Xerostomia may beassociated with:

1. difficulty in swallowing &speaking

2. increased fluid intake

3. disturbances of taste

4. soreness & redness ofmucosa associated withcandidosis

5. rapidly progressive caries6. acute bacterial sialadenitis.


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Oral mucosa appearsdry, smooth, andglazed.

Dorsum of tongueoften appears red andatrophic with variable

degrees of fissuringand lobulation.


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Keratoconjuctivitissicca manifests as:

1. dryness of eyes

2. conjunctivitis

3. gritty, burningsensation.


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Salivary glandenlargement is variable.

30% of patients givehistory of enlargement.

Only 15% present withenlargement.

Usually bilateral.

Predominantly affectsparotid glands.

Seldom painful.


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Lacrimal glandenlargement isuncommon.

Although clinicalinvolvement of minorsalivary glands is

uncommon, they areoften involvedmicroscopically.
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Sjgren Syndrome: Histopathological Features

Major glands:

1. Lymphocytic infiltration,

initially aroundintralobular ducts,eventually replacing thewhole affected lobules.

2. 20% B cells, 80% Tcells, mostly T-helper.

3. Acinar atrophy.
http://rds.yahoo.com/S=96062857/K=sjogren+syndrome/v=2/SID=w/TID=I999_73/l=II/R=8/SS=i/OID=c21887473b387036/SIG=1kepmlk3j/EXP=1121965964/*-http:/images.search.yahoo.com/search/images/view?back=http://images.search.yahoo.com/search/images?p=sjogren+syndrome&ei=UTF-8&fr=sfp&fl=0&x=wrt&h=2000&w=2560&imgcurl=www.mor.aichi-gakuin.ac.jp/sigakubu/kiso/byouri/image/sjogren4.JPG&imgurl=www.mor.aichi-gakuin.ac.jp/sigakubu/kiso/byouri/image/sjogren4.JPG&size=170.4kB&name=sjogren4.JPG&rcurl=http://www.mor.aichi-gakuin.ac.jp/sigakubu/kiso/byouri/html/esjogrn04.html&rurl=http://www.mor.aichi-gakuin.ac.jp/sigakubu/kiso/byouri/html/esjogrn04.html&p=sjogren+syndrome&type=jpeg&no=8&tt=303&ei=UTF-8


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Major glands:

4. Proliferation of duct epitheliumto form epimyoepithelial

islands.

5. The appearance is describedas myoepithelial sialadenitisorbenign lymphoepithelial lesion.

6. Unlike lymphoma, the infiltratedoes not cross interlobular CTsepta.


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Minor glands:

1. Focal collections of lymphoidcells, initially around intralobularducts.

2. The number of foci reflects theseverity of the disease.
http://www.tmd.ac.jp/dent/opat/sakamoto.files/sjo1.jpghttp://www.tmd.ac.jp/dent/opat/sakamoto.files/sjo2.jpg


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Minor glands:

3. The semi-quantitative assessmentof this focal lymphocyticsialadenitis in labial minor salivaygland biopsies is an importantinvestigation in establishing adiagnosis, and is one of thediagnostic criteria.

4. However, since the appearance isnon-specific, it must be interpretedin the presence of clinical featuresand serological investigations.
http://www.tmd.ac.jp/dent/opat/sakamoto.files/sjo3.jpg


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Sjgren Syndrome: Investigations

Minor salivary gland biopsy.

Estimation of parotid salivaryflow rates, usually reduced.

Sialography: shows sialectasia(snowstorm pattern, cherrytree in blossom appearance).

Serological findings: anti-Ro,anti-La.


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Sjgren Syndrome: InvestigationsApproximate frequencies of serological abnormalities & autoantibodies in

primary & secondary Sjgren Syndrome

Serological abnormalities Primary

(%)

Secondary

(%)

Elevated ESR 50-100 50-100

Hypergammaglobulinemia 50-90 50-90

Immune complexes 85 Up to 50

Elevated 2 microglobulin(increased in exacerbations)

30-50 30-50

Autoantibodies

Rheumatoid factor 40-60 75-100

Antinuclear antibody 40-70 Up to 100

Anti-Ro/anti-La 80-90 20-60

Gastric parietal cell

Thyroid Variable Variable

Mitochondrial 5-30 5-30

Smooth muscle


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Sjgren Syndrome: Investigations

Although neither anti-Ro or anti-La isspecific for SS, they are diagnosticallyhelpful since they may be detected sometime before clinical picture develops.


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Sjgren Syndrome: Etiology & Pathogenesis

Strong evidence that it is autoimmune.

Genetic factors thought to be important in increasing susceptibilityto external factors which trigger the disease.

Occurs with increased frequency in patients with certain HLA classII MHC genes.

Several viruses, especially EBV have been suggested as potentialtrigger factors.

Immunological mechanisms leading to destruction of glandulartissue probably involve mainly T cells and their cytokines.

Pathogenic significance of the range of circulating autoantibodies isuncertain.


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Sjgren Syndrome: Malignant Transformation

Risk of B cell lymphomadeveloping in affected gland44 times that of generalpopulation.

Risk varies from


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Sialadenosis (Sialosis)

Non-inflammatory, non-neoplastic,recurrent bilateral swelling of salivaryglands.

Parotid glands most commonly.

Probably due to abnormalities ofneurosecretory control.


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Has been reported with:

1.Hormonal disturbances.2.Malnutrition.

3.Liver cirrhosis.

4.Chronic alcoholism.5.Various drugs.


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Histopathology:

1. Hypertrophy of serous acinar cells toabout twice their normal size.

2. Cytoplasm is densely packed withsecretory granules.


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HIV-Associated Salivary Gland Disease

HIV-related parotidenlargement may bedue to:

1. Persistent glandularlymphadenopathy.

2. Multiplelymphoepithelialcysts.

Slide 14 Diseases of Salivary Glands I

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