Transcript Sjogren-Parotitis

Sialadenitis/Parotitis/Sjogren’s
Vincent Steniger, D.M.D
4-17-08
Outline

Case presentation
 Sialadentitis/ Overall Salivary Gland
Infections
 Parotitis
 Sjogren’s Syndrome
Chief Complaint

53 y/o white, male patient presents with a chief complaint as
follows:
– “Would like my broken front teeth fixed”
– “Would like to check on my swollen glands”

Interpretation of Chief Complaint:
– #7 and #8 Fractured
– Presents with right Parotid Gland swelling, negative
pain, tenderness
History of Present Illness

Current Medical
Conditions:
– Diabetes Type II (1995)
– Asthma (1994)
– Hx of Submandibular
Salivary Stones and
Swelling and resection of
SM glands in 1987
– Hx of both R & L Parotid
Swelling, sometimes with
Pain
– GERD (2007)

Current Medications:
– Glucotrol (5mg/day) (Stimulates
pancreas to secrete insulin.
Sulfonoylurea  Blocks K+ in Islet
Cells leading to greater increase in
Insulin Secretion)
– Prevacid

(H Pump Inhibitor)
– Multivitamin
– Zyrtec
 Antihistamine
– Mucinex
 Expectorant draws water from the
lungs aiding in getting rid of phlegm
– Oasis Mouth Spray
 Essential oils, Glycerol
Past Medical Hx (SHH)

HEENT
Endocrine:
– Diabetes Type II
– SM Glands Removed (1987)
– Hx of R and L Parotid Swelling
– Pt wears glasses
– Seasonal Allergies

and Pain possible Sjogren’s
– Hx of minor gland above the L.
eye removed (1999) (Lacrimal)
CV System:
– Pt denies Prosthetic Valve,
Congenital Heart Disease,
RF, Endocarditis

Renal:
– Hx of Proteinurea

Gastro-intestinal/Hepatic:
– GERD
– Gall Bladder removal (1984)
Respiratory:
– Asthma
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Dermatological: Ø
Infectious:
– Denies HIV/AIDS, Hep A, B, C,
Urinary/Reproductive: Ø
TB


Autoimmune: Ø
Neoplastic: Ø
EOE/ IOE

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Very Swollen Right Parotid
for which the pt says “this is
actually normal, it gets much
bigger than this”
No Skin Lesions
Lips were of healthy color
Left Parotid Gland WNL
Scar can be seen across the
neck from wear the SM
glands were resected
Scar above left eye where
gland resected
+Sialomegally
-Lymphadenopathy
-Thyromegally

Heavily Restored Dentition
 Multiple Amalgam Fillings
 Missing #’s 1-5, 16, 17, 19,
20, 29, 31, 32
 A few areas of
staining/possible caries
 Mild Gingivitis/
Recession/Moderate
Periodontitis (Some
Mobility)
 Noticeably dry oral cavity
 Class I Occlusion
Sialadentitis
 What
is Sialadentitis?
– Simply inflammation of the salivary glands
– Can be due to a number of factors including:
 Mumps infection
 Coxacki Virus
 Parainfluenza
 Systemic Disease
Sialadentitis: Etiology

May be infectious:
– May be caused by bacterial or viral infections
 May be non-infectious:
– May be caused by systemic disease such as Sjogren’s or
Sarcoidosis or even by radiation therapy
 May be Post-Surgical:
– Called “Surgical Mumps”
– Pt kept without fluids and given atropine  causes
xerostomia predisposing to inflammation
 May be Pharmacological:
– Drugs causing xerostomia
 May be architectural:
– Block of the salivary gland due to a stone
Parotitis

Definition:
– Inflammation of the Parotid Gland

May be infectious or non-infectious
 Common Causes:
– Mumps
– Sjogren’s Syndrome
– Bacterial infection of parotid gland  usually Staph.
aureus
– Blocked salivary duct
– Stone in salivary duct
Parotitis: Symptoms
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Pain/ Tenderness of the Parotid Glands
Enlargement of the Parotid Glands
Infectious parotitis
– Acute bacterial parotitis: The patient reports progressive painful swelling of the
gland; chewing aggravates the pain.
– Acute viral parotitis (mumps): Pain and swelling of the gland last 5-9 days.
Moderate malaise, anorexia, and fever occur. Bilateral involvement is present in
most instances.
– HIV parotitis: Nonpainful swelling of the gland occurs; otherwise, patient is
asymptomatic.
Parotitis in tuberculosis: Chronic nontender swelling of one parotid gland occurs, or a
lump is noted within the gland. Symptoms of tuberculosis are found in some cases.
Sjogren’s Syndrome: Recurrent or chronic swelling of one or both parotid glands with
no apparent cause is noted. It is frequently associated with autoimmune disease.
Discomfort is modest in most cases and is related to dry mouth and eyes.
Recurrent parotitis of childhood: Repetitious episodes of unilateral or bilateral mumpslike episodes in a young child are indicative.
Sarcoidosis: Chronic nontender swelling of parotid gland occurs
Overall Treatment for Parotitis

Acute:
– Antibiotics
– Rehydration stimulating salivary flow
– Possible IND

Chronic:
– Eliminate causative agent:

–
–
–
–
Get rid of salivary stone/ other blockage
Warm Compresses
Sialogogues
Possible surgical resection
Ligation of the duct in hopes of atrophy
Mumps
Mumps: (Viral Parotitis)

Acute sialadenitis caused by RNA virus
–

Paramyxovirus
Other viruses causing salivary gland
infection:
–
–
–
Cytomegalovirus
Coxsackieviruses
Echovirus
Mumps: Clinical Features

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Transmitted via airborne droplet
Mainly effects the parotid gland
Mainly effects children between the ages of 5-18
Has a 2-3 week incubation period
Clinically:
– Will see rapid swelling of the parotids bilaterally
– Acute pain when salivating
Clinical Features- continued

When looking at the patient:
– The ear lobe is elevated due to glandular enlargement

There may be a purulent discharge from the
parotid duct but it is clear and unremarkable
 Blood Work:
– As the acini become infected the salivary amylase leaks
into the interstitium and is absorbed in the blood stream
raising the serum amylase levels
Mumps: Histopathology

There is infiltration with plasma cells and
the lymphocytes
 The ductal lumens contain desquamated cell
debris and leukocytes
Mumps: Treatment

There no effective antiviral therapy
available for the treatment of mumps.
 Analgesics and antipyretics are given to
control pain and fever
 Liquid diet with vitamins
 Bed rest
Sjogren’s Syndrome
Sjogren’s Syndrome

It is a group of autoimmune conditions with a
marked predilection for woman, it has an intense T
lymphocyte – mediated autoimmune process in
salivary and the lacrimal glands as on of its most
prominent component
 Sjogren’s syndrome exhibits T cells infiltration
and replaces the glandular parenchyma
Sjogren’s Syndrome

Sjogren’s Syndrome:
– objective evidence of keratoconjunctivitis sicca
– characteristic pathologic features of the salivary
glands
– 2 out of 3 of:


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recurrent chronic idiopathic salivary gland swelling
unexplained xerostomia
connective tissue disease
Sjogren’s Syndrome: Who is effected?

Sjogren’s Syndrome can be Primary or Secondary:
– Primary  the syndrome is second to nothing

Only effects the salivary glands and the lacrimal glands
– Secondary  Sjogren’s secondary to something like
Rheumatoid Arthritis, SLE or Scleroderma

0.5-1% of the population is effected
 Age ranges for Sjogren’s  20-40 years
 9:1 women effected more
Sjogren's Syndrome: Age of Onset
The frequency distributions of ages at onset of symptoms
& at diagnosis of primary Sjogren's syndrome
45
% OF PATIENTS
40
Onset
At diagnosis
35
30
25
20
15
10
5
0
1-10 11-20 21-30 31-40 41-50 51-60 61-70 71-80 81-90
AGE
Sjogren’s Syndrome: Clinically

Subjective and Objective Findings:
 Subjective:
– Xerostomia
– Salivary Gland Enlargement

Objective:
–
–
–
–
–
–
Stomatitis
Oral Ulcers
Cracked, “crocodile skin” tongue
Carious Teeth
Parotid Gland Enlargement
Certain Tests can be done
“Crocodile Skin” Tongue, Carious Teeth
Tests and Studies: Scintigraphy

Scintigraphy (Nuclear Medicine)  administer radioactive substance
in order to show the physiology and state of the biological process:
Scintigraphy
Moderate
Marked
Normal
diagnosis
involvement involvement
Degree of
None
Mild
Severe
xerostomia
Salivary flow
1.60
0.42
0.00
rate (ml/5min/gland)
Scintigraphy: Continued

With Sjogren’s Syndrome there will be
delayed uptake and concentration of marker
as well as delayed excretion
Tests and Studies: Schirmer’s Test


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A test of whether the eye has enough tears to keep moist
Procedure:
– Piece of filter paper inserted for several minutes
(usually 5) and moisture recorded
<5 ml in 5 minutes is characteristic of Sjogren’s Syndrome
Tests and Studies: Serology
Autoantibodies
Rheumatoid factors
(Igs)
Cryoglobulins (type
% positive
80
30
II)
Ro/SSA
La/SSB
a-fodrin
60
30
95
Tests and Studies: Salivary Gland Biopsy

A lip biopsy, if positive for Sjogren’s will
show lymphocytes clusters and glandular
destruction due to inflammation
Tests and Studies: Salivary Flow Rate

Stimulated
 Unstimulated
Sjogren’s Syndrome: Exclusion Criteria
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Prior head and neck irradiation
Pre-existing lymphoma
Acquired immunodeficiency disease (AIDS)
Hepatitis C infection
Sarcoidosis
Graft-versus-host disease
Sialoadenosis
Drugs (neuroleptic, anti-depressant, anti-hypertensive,
parasympatholytic)
Sjogren’s Syndrome: Differential Diagnosis

HIV, HCV infection

Sarcoidosis

Amyloidosis

Lipoproteinemia

Chronic graft-versus-host disease

Lymphoproliferative disorders
Sjogren's Syndrome
Algorithm for the diagnosis
Dry mouth
Dry eyes
or
Salivary gland
enlargement
or
Raynaud’s phenomenon
Purpura
Renal tubular acidosis
If any positive
Eye & salivary
gland tests
Serology
If positive
Sjogren's Syndrome
Sjogren’s Syndrome: Pathophysiology

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Insult may start with a bacterial or viral infection. The
peptides and antigens associated with the bacterial or viral
infection along with autoantigens (self being recognized as
foreign) are associated with HLA II complex that gets
expressed on CD4+ T Cells. Once the HLA is expressed,
there is release of cytokines and further T Cell activation
After the initial attack of the CD4+ T Cells, B cells enter
the gland and make autoantibodies, including, in many
cases Anti-SS-A (Ro) and Anti-SS-B (La) (only Sjogren’s
Syndrome) and in some cases Rheumatoid Factor. Via
autoimmunity, and antigens being expressed to CD4+ T
cells, the acini of the gland are destroyed
Pathophysiology: Continued
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Multifactorial disease
SS is sometimes called autoimmune epithelitis in which
there is apoptosis of epithelial cells leading to degradation
products and leading to antinuclear autoantigens to the
immune system
Molecules within the TNF family play a big role in the
polyclonal activation of B Cells. This, in turn leads to
autoantibodies
There is known inhibition of healthy glands and/or the
muscarinic receptors (via antibodies) and also abnormal
function of aquaporins leading to poor function of
remaining healthy glandular structure
There is prolonged/permanent activation of autoreactive B
cells favoring oncogenic activity and possible development
of B Lymphoma
Parotitis/Sjogren’s: Histology

Acute Parotitis:
– See inflammatory infiltrate (neutrophils) around the
ductal system and acini
– Destruction of epithelial tissues

Chronic Parotitis:
– Inflammatory infiltrate in the parenchyma of the gland
(Plasma Cells and Lymphocytes)

The basic features are massive lymphoid
infiltration with atrophy of the acini, proliferation
of the cells of the small ducts that leads to
narrowing of the lumen, and finally, obliteration of
the gland
Normal
Pathological
Sjogren’s Syndrome: Systemic Manifestations
Systemic manifestations Frequency (%)
Arthralgia/arthritis
Raynaud’s phenomenon
Purpura/Vasculitis
Lung involvement
(increased liver enzymes)
Kidney involvement
(Interstitial Nephritis/Glomerulonephritis)
Liver involvement
Muscle involvement
60
30
15 (1)
10 (25)
8 (25)
5
1
Skopouli et al., Semin Arthritis Rheum. 2000, 29:296
“Evaluation of sialometry and minor salivary
gland biopsy in classification of Sjögren's
Syndrome patients”

Revista Brasileira de Otorrinolaringologia
 vol.71 no.3 São Paulo May/June 2005
 Liquidato et al
 Cohort Study
Why Do the Study?

There is no gold standard for the diagnosis of
Sjogren’s Syndrome
 In the past, biopsy and salivary flow rates were
used
 Minor Salivary Gland biopsy is the most accurate
diagnosis means but is not used as a criteria for the
diagnosis of Sjogren’s, rather it is helps confirm
the diagnosis when there is a blood test confirming
presence of Anti-SSA or Anti-SS-B
 Study wants to know if there is a less invasive way
to have an accurate diagnosis of Sjogren’s
Syndrome
Purpose of the Study

The present study aimed at assessing the role of minor
salivary gland biopsy and sialometry, either isolated or
associated, as methods used to classify Sjögren's
Syndrome based on the criteria defined by the European
Community Study Group on Diagnostic Criteria for
Sjögren's Syndrome
Materials and Methods: Subjects
– 72 patients coming to the Department of
–
–
–
–
–
Otorhinolaryngology, in Sao Paulo Brazil from 1997 to 2003
Based on a criterion showed, they were submitted into the
investigation for diagnosis
Patients split into 2 groups  those with the diagnosis of
Sjogren’s already, and those without the diagnosis of
Sjogren’s
26 pts with Sjogren’s and 46 pts not yet dx with Sjogren’s
Those with Sjogren’s were broken up into Primary or
Secondary Sjogren’s
To classify patients with primary Sjögren's Syndrome we
required the presence of 4 out of 6 items and item 4
(histopathology) or 6 (auto-antibodies) had necessarily to be
present. As to classification of patients with secondary
Sjögren's Syndrome, it required the presence of item 1 or
item 2 plus 2 other items numbered 3, 4 and 5
Materials and Methods:
Methods:

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Non-stimulated sialometry:
– Used 2 pre-weighed cotton balls
– Subjects swallowed all saliva, then cotton balls put on the
floor of the mouth for 2 minutes, then weighed again
– Anything 0.1ml/minute considered abnormal
Minor Salivary Gland Biopsy:
– Horizontal incision parallel to the vermillion border of the
lower lip
– Took about 4-6 minor salivary glands
– Histopathological findings were graded as follows:
normal gland; mild inflammatory process; moderate
inflammatory process; severe inflammatory process, and
presence of inflammatory foci
A Review…
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
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sensitivity = probability of a positive test among patients with disease
specificity = probability of a negative test among patients without
disease
positive predictive value = the proportion of patients with positive
test results who are correctly diagnosed. It is considered the physician's
gold standard, as it reflects the probability that a positive test reflects
the underlying condition being tested for
negative predictive value = the proportion of patients with negative
test results who are correctly diagnosed
Results:

As to the number of inflammatory foci found on biopsy:
– Those with Primary Sjogren’s had more than those with Secondary
Sjogren’s who had more than those with no diagnosis of Sjogren’s

Current Article:
– Biopsy  Sensitivity of 72%, Specificity of 84%
 Positive Predictive Value of 75%
 Negative Predictive Value of 82%
 Meaning  84% of the time, those with SS will have a positive biopsy
 Meaning  72% of the time, those without SS will have a negative biopsy
 Meaning  75% of the time, those with a positive biopsy will have SS dx
correctly
 Meaning  82% of the time, those with a negative biopsy will not have SS
– Sialometry  Sensitivity of 62%, Specificity of 52%
 Negative Predictive Value of 71%
– Biopsy Accuracy 79%
– Both Biopsy + and Sialometry +  Specificity of 95%, PPV of 86%
Conclusion

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Sialometry  higher sensitivity and specificity in Primary vs
Secondary group
– There is higher likelihood of the subject not having Sjögren's
Syndrome when sialometry is negative
Biopsy  higher sensitivity and specificity in Primary vs Secondary
group
– A subject would have 75% likelihood of having Sjögren's
syndrome when biopsy was positive and 81.6% likelihood of not
having Sjögren's syndrome when biopsy was negative
Case Study

Recurrent Parotitis as a First Manifestation
of Adult Primary Sjogren’s Syndrome
 Sugimoto et al
 The Japenese Society of Internal Medicine
January 17th, 2006
 Case Report
Case Subject

38 year-old Japanese women with 3 year history
of bilateral parotitis
 Denied dry eyes, dry mouth, trouble swallowing,
arthralgias
 Lab Tests:
– Rheumatoid Factor
– Positive for Anti-SS-A/Ro Antibodies (found in 45% of
Primary Sjogren’s Patients)
– No Anti-SS-B/La Antibodies (found in 15% of Primary
Sjogren’s Patients)
Salivary Function and Biopsy

Salivary Function:
– Unstimulated Salivary Flow  0.2ml/10 min
– Stimulated Salivary Flow  2.0ml/10 min

Labial biopsy of minor salivary glands
revealed lymphocyte infiltration and
glandular and ductal atrophy
 Diagnosed with Primary Sjogren’s
Syndrome
Conclusion

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Recurrent Suppurative Parotitis is a common first
manifestation in children and adolescents
Recurrent Parotitis being the first manifestation is rare, but
can happen
Treatment for Recurrent Parotitis:
– Because of stasis of saliva within the gland, there is
usually bacterial infection, thus antibiotics are common
 some literature promotes Antibiotic Prophylaxis to
prevent this as some patients can feel prodromal
symptoms before inflammation/infection of the gland
Point of the article:
– Consider Sjogren’s Syndrome within the differential
as the underlying cause of Recurrent Parotitis, even
in adults
Case Patient:

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Has had recurrent submandibular infection, stones, swelling to which the
glands were removed
Has had his right lacrimal gland removed because of swelling
Has now had recurrent bouts of Parotitis, some suppurative
Has constant xerostomia for which Oasis mouth spray is used
HAS NEVER BEEN WORKED UP FOR SJOGREN’S
– PCP NEVER MENTIONED IT
– ENT DIDN’T MENTION IT
– PLASTIC SURGEON BROUGHT IT UP
To Do For The Patient:
Refer to Sjogren’s specialist
 Blood tests for Anti-SS-A(Ro), Anti-SSB(La) and Rheumatoid Factor
 Stimulated, Unstimulated Salivary Flow
 Minor Salivary Gland Biopsy
 Confirm Dx of Sjogren’s Syndrome

Sjogren’s Syndrome: Treatment
Glandular  Stimulation/Replacement
 To Treat Xerostomia:

– Salivary Substitutes
– Dx and treatment of candidiasis
– Meticulous oral hygeine for prevention of caries

To Treat Xerophthalmia:
– Stimulation for tears:

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Cyclosporin A
Pilocarpine
Cimeviline
Treatment: Continued

Treatment for Salivary Gland Enlargement:
– Local moist heat
– Antibiotic Therapy
– NSAIDs
– Rule out a Lymphoma

Treatment for Peripheral Symptoms:
– Methotrexate
– Cyclosporin A
– Infliximab
– Hydroxychloroquine
– Corticosteroids