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Group 3 Sjogrens Syndrome

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PRINCIPLES OF MEDICINE has been found that some patients present only with

CASE PRESENTATION dry eyes and dry mouth (sicca complex or primary
Sjögren’s syndrome), while others also develop
GUSTILO, NICOLE systemic lupus erythematosus, polyarteritis nodosa,
LACSON, COLEENE polymyositis or scleroderma, as well as rheumatoid
LERIO, FRANCINE arthritis (secondary Sjögren’s syndrome). As Sjögren
LIU, GRACE pointed out, cases of xerostomia and arthritis without
keratoconjunctivitis sicca have been observed.
I. CASE ❖ Sex: Women are much more likely to have Sjogren's
syndrome
A 48-year-old woman presents to the clinic with itching eyes, ❖ Rheumatic disease: It's common for people who have
dryness of mouth, difficulty swallowing, loss of sense of Sjogren's syndrome to also have a rheumatic disease
taste, hoarseness, fatigue and swollen parotid glands. She such as rheumatoid arthritis or lupus.
reports increasing joint pain over the past 2 years. She ❖ Ocular effects of Sjogren’s syndrome:
complains of frequent mouth sores. Laboratory tests reveal: - Failure of tear secretion
positive antinuclear antibodies and rheumatoid factor - Dried secretions stick to conjunctiva and cornea
levels of 70 U/ml. A parotid gland biopsy reveals inflammatory - Failure of clearance of foreign particles from the
infiltrates in the interlobular connective tissue with damage cornea and conjunctiva
to acinar cells and striated ducts. - Gritty sensation in the eyes
- Keratinisation and loss of goblet cells in
II. DIFFERENTIAL DIAGNOSIS conjunctiva
- Abrasions, ulcers and inflammation
- Risk of impairment or loss of sight
RULE IN RULE OUT
❖ Oral effects of Sjorgen’s syndrome:
- Discomfort
SYSTEMIC - DRY MOUTH - BUTTERFLY WING
LUPUS - DRY EYES LIKE RASH ON THE - Difficulties with eating or swallowing
ERYTHEMATOS - JOINT PAIN FACE - Disturbed taste sensation
US (SLE) - MOUTH SORE - Disturbed quality of speech
- Predisposition to infection
RHEUMATOID - DRY EYES - MORNING ❖ In radiographs:
ARTHRITIS - DRY MOUTH STIFFNESS Sialographs demonstrate the formation of punctate,
- JOINT PAIN - INFLAMMATION cavitary defects which are filled with radiopaque contrast
INVOLVING SMALL media. These filling defects have been said to produce a
JOINTS OF HANDS,
‘cherry blossom’ or ‘branchless fruit-laden tree’ effect
WRIST AND FEET
- JOINT radiographically.
DEFORMITIES
Revised international classification criteria for
SARCOIDOSIS - DRY EYES - NIGHT SWEATS sjogren’s syndrome:
- PAROTID - GRANULOMAS IN
GLAND SALIVARY GLANDS I. Ocular symptoms: a positive response to at least on of
ENLARGEMENT - COUGH UP BLOOD the three validated questions
- JOINT PAIN
1. have you had daily, persistent troublesome dry
eyes for more than 3 months?
2. Do you have a recurrent sensation of sand or
III. IMPRESSION (DIAGNOSIS) WITH gravel in the eyes?
SUPPORTING FACTS 3. Do you use tear substitutes more than three times
​ jogren's syndrome
S a day?

II. Oral symptoms: a positive response to at least one of


three validated questions
1. Have you had a daily feeling of dry mouth for
more than 3 months?
2. Have you had recurrent or persistently swollen
salivary glands as an adult?
3. Do you frequently drink liquids to aid in
swallowing dry foods?

III. Ocular signs: objective evidence of ocular involvement


defined as a positive result to at least one of the following
​Sjogren's syndrome typically occurs in people with one or two tests:
more known risk factors, including: 1. Shrimer’s I test, performed without anaesthesia
(less or equal to 5 mm in 5 min)
❖ Age: Sjogren's syndrome is usually diagnosed in 2. Rose Bengal score or other ocular dye score
people older than 40. (more or equal to 4 according to van Bijsterveld’s
❖ A triad consisting of keratoconjunctivitis sicca, scoring system)
xerostomia and rheumatoid arthritis. Subsequently, it
The Van Bijsterveld score is determined by adding up the
Opening of the Buccal Floor of Floor of buccal
combined score (from 0 to 3; 0 is normal and 3 is the
ducts cavity buccal cavity cavity at the
highest level of staining) of the central and two outer
across the at the sides sides of lingual
sections. The maximum score per eye is therefore 9. A
maxillary of lingual frenulum
score of 4 or higher is abnormal.
2nd molar frenulum
IV. Histopathology: minor salivary glands focal lymphocytic
sialoadenitis, with a focus score more or equal to 1. Type of cells Serous Mixed Mostly mucous

V. salivary gland involvement: objective evidence of


salivary gland involvement defined by a positive result to at Type of acini Serous Sero-mucous Mostly mucous
least one of the following diagnostic tests: acini acini acini
1. unstimulated whole salivary flow (less or equal to
1.5ml in 15 min) Specific duct Stenson’s Wharton’s Bartholin’s duct
2. parotid sialography duct duct
3. salivary scintigraphy
Duct types 3 types 3 types 3 types
VI. antibodies in the serum to Ro/SS-A or La/SS-B intercalated intercalated intercalated,
antigens, or both , striated and striated striated and
and ducts excretory ducts
IV. ANATOMY OF THE ORGAN excretory
INVOLVED(GROSS AND MICROSCOPIC ducts

Nerve supply Glossophar Lingual nerve Lingual nerve


yngeal CN V) and CN V) and
nerve (CN facial nerve facial nerve
IX), (CN VII) (CN VII)
trigeminal
nerve (CN
V)

Amount of 25% 60% 5%


saliva
production

Size of glands largest intermediate smallest

Minor salivary glands:


SALIVARY GLANDS - Scattered throughout the oral submucosa, with
the exception of the gingiva and anterior hard
palate
- Not encapsulated and each gland possesses 1
duct draining directly into the oral cavity
- Predominantly mucinous apart from the purely
serous von Ebner glands of the dorsal tongue
that surround the circumvallate papillae

GROSS ANATOMY
Salivary glands are exocrine glands meaning that the Normal:
produced secretions are released via ducts into the
adjacent external or internal epithelia Parotid gland:

Parotid Submandibu Sublingual


gland lar gland gland

Developmental Ectodermal Endodermal Endodermal


origin

Submandibular gland:
Serous ● Dominant component in the
acini parotid gland and von Ebner
glands of the tongue
● Polygonal shaped cells with
dense, intracytoplasmic,
basophilic zymogen enzyme
containing granules
● Cells are periodic acid-Schiff
(PAS) reagent positive

Sublingual gland:
Mucous ● Dominant component in the
acini minor salivary glands of the
palate and base of tongue
● Round cells filled with mucin
and containing a basally
located, condensed nucleus
● Positive for mucicarmine,
alcian blue and PAS with
diastase resistance (DPAS)
Lacrimal gland:

Mixed ● Dominant component in the


seromuc submandibular (serous
ous acini predominance) and sublingual
(mucous predominance)
glands, minor salivary glands
of the lip, cheek and anterior
tongue
Abnormal: ● Mucous acini with crescent
shaped caps (demilunes) of
Swelling of parotid gland: serous cells

❖ 3 main types of ducts are intercalated, striated or


interlobular:

Intercalat ● Smallest caliber


ed ducts ● Emerge from the acinar unit
● Lined by a single layer of cuboidal
Lacrimal gland enlargement: epithelium
● Contain reserve cells that
regenerate the terminal secretory
unit

Striated ● Proceed from the intercalated


ducts ducts
● Intralobular
● Intermediate caliber between
intercalated and interlobular ducts
MICROSCOPIC ANATOMY ● Lined by columnar epithelium
containing basal cytoplasmic
❖ Functional unit of all salivary glands consists of striations, formed by rows of
acini and ducts mitochondria alternating with
❖ Within the major salivary glands, these functional folded cell membrane
secretory units are arranged in lobules ● Contribute to the ionic
❖ Interlobular regions include large excretory ducts composition of the saliva
and neurovascular bundles ● Drain into interlobular ducts
❖ Acini can be composed predominantly of serous
or mucous cells or can be mixed seromucous
type
Interlobul ● Located within interlobular septa
ar ducts and lined by pseudostratified
columnar epithelium
● Merge to form the major excretory
duct outside the gland, lined
distally by nonkeratinized
stratified squamous epithelium

❖ Myoepithelial cells are critical for the expulsion of saliva


➢ Contractile myoepithelial cells surround acini,
Minor salivary glands:
intercalated ducts and striated ducts
➢ Located between epithelial cells and the
basement membrane, embracing the secretory
unit with long cytoplasmic processes
➢ Generally spindle shaped with blunt ended nuclei
and elongated cytoplasmic processes

❖ Normal inclusions within the salivary glands:


➢ Presence of sebaceous glands within the parotid gland
is common and are usually found associated with
interlobular ducts or may exist as isolated cells within
the wall of intercalated or striated ducts LACRIMAL GLAND
➢ Unique to the parotid gland is the presence of
intraparotid lymph nodes, most of which are located
superficial to the facial nerve
■ Often the lymph nodes may lack a well
defined capsule and merge with the adjacent
salivary gland acini
➢ Fatty infiltration of the major salivary glands is an age
induced variation

Parotid gland: ● Secretory units/potions are surrounded by


myoepithelial cells and divided into lobes by
connective tissue
● columnar secretory cells have pale granules and
secrete antibacterial lysosome

MICROSCOPY OF SJOGREN’S SYNDROME

Changes in salivary glands:


● Polyclonal infiltration mainly by CD4 lymphocytes
● Infiltrate initially periductal
● Progressive spread of infiltrate through the
glandular tissue
● Progressive destruction of secretory acini
● Proliferation of ducts to form epimyoepithelial
islands

Submandibular gland:

Sjögren’s
syndrome, minor salivary gland expression. Note
lymphocytic focus adjacent to intact acini.

Sublingual gland:
stochastic interactions between the environment and a
susceptible host culminating in a dysregulated immune
response and exocrine dysfunction.

Environmental Factors

The strong female predominance suggests sex-specific


predisposing factors. Estrogens are considered to
contribute to autoimmunity, whereas androgens are
thought to be protective. Ovaries produce low levels of
Sjögren’s syndrome, minor salivary gland expression.
testosterone, which decrease at the time of menopause.
Note confluent lymphocytic foci without evidence of
scarring.
The other significant source of androgens is the adrenal
cortex, which produces dehydroepiandrosterone (DHEA)
and its metabolite DHEA sulfate (DHEA-S).

The key factor behind Sjögren's syndrome is systemic and


local androgen deficiency.

Immune system abnormalities

Recent studies have focused on the central role of type-I


IFNs and the more recently described B cell-activating
Diffuse lymphocytic infiltrate and the typical factor (BAFF), which may represent a link between innate
epimyoepithelial islands and adaptive immunity.

Changes in lacrimal gland:


Biopsy of a lacrimal gland with focal infiltrations of ● Interferon-α
mononuclear cells in obviously normal glandular Enhanced activity of the type-1 IFN system has
parenchyma (blue staining). been linked to multiple autoimmune diseases,
including Sjögren's syndrome.
The red staining represents surrounding mainly loose
connective tissue ● B cell-activating factor
One of the cytokines upregulated by IFNα is
BAFF. BAFF promotes B-cell survival and exists
in a membrane bound and a secreted form.
Increased levels of BAFF were previously
demonstrated in the salivary glands, saliva and
serum of Sjögren's patients. Sjögren's syndrome
T cells have a higher spontaneous production of
BAFF, and monocytes from Sjögren's syndrome
patients secrete higher levels of BAFF up on
IFNa stimulation
V. PATHOGENESIS/ PATHOPHYSIOLOGY OF
THE DISEASE
Autonomic nervous system

PATHOGENESIS Xerostomia and xerophthalmia, the cardinal Sjögren's


syndrome manifestations, are features of cholinergic
Sjögren's syndrome is considered an autoimmune disease parasympathetic ANS dysfunction, whereas
characterized by chronic inflammation involving the sympathetic cholinergic failure results in xerosis and
exocrine glands. A widely accepted model of the decreased sweating that are frequently reported by
pathogenesis of Sjögren's syndrome is that in genetically Sjögren's syndrome patients. Fatigue, another
predisposed individuals, various environmental factors prominent feature of Sjögren's syndrome, has also
such as viral infections lead to epithelial cell activation and been associated with ANS dysfunction
a protracted inflammatory response with features of
systemic autoimmunity.

Antimuscarinic receptor autoantibodies

Human Studies The major stimulus for saliva production is provided


by acetylcholine through muscarinic acetylcholine
The heterogeneity of Sjögren's syndrome suggests that the receptors of which the type 3 receptor [muscarinic 3
clinical manifestations in any individual result from receptor (M3R)] is responsible for saliva production.
Antimuscarinic acetylcholine receptor autoantibodies, cognitive impairments, such as dementia, lack of
or antibodies interfering with the other concentration, memory loss, and various psychiatric
neurotransmitters and their receptors, would provide a disorders (ranging from depression to anxiety), are often
link between autoimmunity and exocrine dysfunction noted in SS patients during clinic visits.
in Sjögren's syndrome.
VI. CLINICAL MANIFESTATIONS

PATHOPHYSIOLOGY
The two main symptoms of Sjogren's syndrome are:
The term Sjögren's syndrome refers to keratoconjunctivitis
sicca and xerostomia due to lymphocytic infiltrates of ● Dry eyes (keratoconjunctivitis sicca)
lachrymal and salivary glands. ● Dry mouth (xerostomia)

Primary Sjögren's syndrome is an autoimmune disorder


characterized by lymphocytic infiltrates and destruction of
the salivary and lachrymal glands and systemic production Some people with Sjogren's syndrome also have one or
of autoantibodies to the ribonucleoprotein particles more of the following:
SS-A/Ro and SS-B/La.
● Joint pain, swelling and stiffness
The infiltrating cells (T- and B-cells, dendritic cells) interfere ● Swollen salivary glands
with glandular function at several points: ● Skin rashes or dry skin
● Vaginal dryness
1. Destruction of glandular elements by ● Persistent dry cough
cell-mediated mechanisms ● Prolonged fatigue
2. Secretion of cytokines that activate pathways
bearing the signature of type 1 and 2 interferons Constitutional: fatigue
3. Production of autoantibodies that interfere with
muscarinic receptors Sicca symptoms: KCS and xerostomia
4. Secretion of metalloproteinases (MMPs) that
interfere with the interaction of the glandular cell
Dryness of other surfaces: nose, vagina, trachea, skin
with its extracellular matrix, which is necessary
for efficient glandular function.
Extraglandular features: arthralgia/arthritis, Raynaud’s,
lymphadenopathy, interstitial pneumonitis, vasculitis
Mechanisms:
(usually cutaneous), nephritis, lymphoma

Role of Apoptosis
Complications:

Acinar cell apoptosis is a histological feature of SS.


The most common complications of Sjogren's syndrome
Whether it results from glandular destruction by
involve your EYES and MOUTH.
inflammation or by malfunction of acinar and ductal
epithelial cells, apoptosis appears to affect acinar cell
Dental cavities: Because saliva helps protect the teeth
mass.
from the bacteria that cause cavities, you're more prone to
developing cavities if your mouth is dry.
Role of Autoreactive T Lymphocytes

Yeast infections: People with Sjogren's syndrome are


T cells can stimulate the production of other inflammatory
much more likely to develop oral thrush, a yeast infection in
cytokines, chemokines, and adhesion molecules to attract
the mouth.
inflammatory cells to the site of injury; in SS, this
phenomenon is probably associated with the activation of
Vision problems: Dry eyes can lead to light sensitivity,
the inflammasome.
blurred vision and corneal damage.

Role of Autoreactive B Cells


Less common complications might affect:

B lymphocytes infiltrating the salivary glands appear to be


Lungs, kidneys or liver: Inflammation can cause
marginal zone B cells, a population known to participate in
pneumonia, bronchitis or other problems in your lungs;
innate immunity, the maintenance of germinal centers in
lead to problems with kidney function; and cause hepatitis
the target tissue, and subsequent antibody production in
or cirrhosis in your liver.
SS.

Lymph node: A small percentage of people with Sjogren's


Sjogren Syndrome-Associated Neurological
syndrome develop cancer of the lymph nodes (lymphoma).
Dysfunction

Nerves: You might develop numbness, tingling and


Sensory, peripheral, cranial, and myelopathic neuropathies
burning in your hands and feet (peripheral neuropathy).
develop in approximately 20% of SS patients. In addition,
> centromere pattern, denoting staining of centromere
VII. DIAGNOSIS - LABORATORY proteins in the mitotic spindle of dividing cells

1. Blood tests The ANA test is positive in the majority of Sjögren’s


syndrome patients. However, a positive ANA test is also
Levels of different types of blood cells common in healthy individuals. With this test, a positive
result is listed as the last dilution of serum that results in
● + SS-A (Ro) and SS-B (La) antibodies: visible staining of the nucleus of the cultured human cell.
(Anti-SSA autoantibodies Typical dilutions are 1:40, 1:80, 1:160, 1:320 and 1:640.
(anti–Sjögren's-syndrome-related antigen A Positive ANA test results of 1:80 and 1:160 may be seen in
autoantibodies, also called anti-Ro) up to 15% and 5% of healthy individuals, respectively. A
negative ANA test does not exclude the diagnosis of
Sjögren’s syndrome; some of these individuals may still
These antibodies are a large family of proteins in the blood
have SS-A and/or SS-B antibodies. With the
that are capable of binding to molecular targets. These
immunofluorescent staining test, the pattern of nuclear
targets, termed antigens, are located on the surfaces of
staining is reported. Most Sjögren’s patients have either a
microorganisms, cells, and blood proteins. Each antibody
speckled or homogeneous pattern. However, some
protein has a binding site that can attach to only a very
patients may have a centromere pattern, denoting staining
specific molecular target. However, the large array of such
of centromere proteins in the mitotic spindle of dividing
antibody proteins in our blood ensures that a diverse and
cells.
large number of molecular targets can be recognized.
Binding of such molecular targets is an essential step in
our defence against infections and cancer. Cells that ● Complete Blood Count (CBC):
produce antibodies against molecular targets on our own
tissues are actively eliminated from our bodies, particularly > ↓ number of white blood cells in 15-20% patients
early in life. If this mechanism goes awry, then we may
form antibodies to self-material and be susceptible to The CBC test determines the number of red blood cells
autoimmune disease. SS-A and SS-B antibodies are (RBC), white blood cells (WBC), and platelets as well as
examples of two antibodies that bind molecular targets the level of hemoglobin in the blood. A low number of white
within our own cells. SS-A antibodies are found in blood cells (leucopenia) can be found in approximately
approximately 60-80% of Sjögren’s syndrome patients. 15-20% of Sjögren’s syndrome patients, but is usually not
SS-B antibodies are less common, being found in associated with an increased risk of infection.
approximately 30-50% of Sjögren’s syndrome patients.
SS-A antibodies thus occur commonly by themselves. ● Immunoglobulins:
However, it is very uncommon for SS-B antibodies to occur
alone. Accordingly, 20-40% of Sjögren’s syndrome patients immunoglobulin quantitation test:
may lack SS-A and/or SS-B antibodies. The finding of
SS-A and/or SS-B antibodies is not specific to Sjögren’s
> Hyperglobulinemia - ↑ IgG, IgM, and IgA
syndrome patients. These antibodies may also be found in
patients with systemic lupus and occasionally in other
The immunoglobulin quantitation test measures the levels
autoimmune diseases, such as myositis. Additionally, they
of the most common types of immunoglobulins in the
may be found in approximately 1 in 200 healthy women.
blood, known as IgG, IgM, and IgA. Some patients with
Sjögren’s syndrome have elevated levels of these
● + Antinuclear Antibody (ANA):
immunoglobulins, termed hyperglobulinemia, reflecting
overactivity of the immune system.
> Typical dilutions are 1:40, 1:80, 1:160, 1:320 and 1:640.
Positive ANA test results of 1:80 and 1:160 may be seen in
● Complement Proteins:
up to 15% and 5% of healthy individuals, respectively.

> ↓ levels of C4
Immunofluorescent staining test denoting:

The complement proteins, C3 and C4, are part of a group


> speckled or homogeneous pattern
of proteins that mediate tissue inflammation and damage in
certain immunologic diseases, including Sjögren’s
syndrome. Low levels of these proteins generally reflect
the ongoing utilization of these proteins in a process
triggered by the binding of immunoglobulins to molecular
targets in the serum (thereby forming immune complexes)
or to targets on cellular surfaces. Very low levels of C4 can
also be a reflection of the genetic absence of this protein.

● C-Reactive Protein (CRP):

> ↑ CRP
This test measures systemic inflammation. It is elevated Ocular surface staining to look closely at the surfaces of
most commonly in the setting of an infection or tissue your eyes for damage and dryness.
injury, such as a myocardial infarction (heart attack). In the
setting of autoimmune disease, the CRP may be elevated,
but usually to a much lesser extent than during an
infection. 3. Imaging

● Liver blood test - Sialogram.

> ↑ alkaline phosphatase, aspartate aminotransferase This special X-ray can detect dye that's injected into the
(AST), alanine aminotransferase (ALT) salivary glands in front of your ears. This procedure shows
how much saliva flows into your mouth.
There is a higher incidence of a type of liver disease known
as primary biliary cirrhosis in patients with Sjogren's - Salivary scintigraphy.
syndrome. A liver blood test (alkaline phosphatase) is
usually elevated in this disease.
This nuclear medicine test involves the injection into a vein
of a radioactive isotope, which is tracked over an hour to
● Complete Metabolic Panel: see how quickly it arrives in all your salivary glands.

> ↑ total protein level ↑ antibody

> ↓ CO2 → renal tubular acidosis 4. Biopsy

The complete metabolic panel measures the electrolytes Your doctor might also do a lip biopsy to detect the
as well as chemicals or proteins in your blood that reflect presence of clusters of inflammatory cells, which can
the function of the kidneys, liver, and immune system. In indicate Sjogren's syndrome. For this test, a sliver of tissue
Sjögren’s syndrome, the total protein level may be is removed from salivary glands in your lip and examined
elevated, reflecting the increase in antibody (or under a microscope.
immunoglobulin) levels in your blood. The serum
bicarbonate or carbon dioxide level may be low, reflecting a
disorder of the kidney that may occur in Sjögren’s
VIII. MANAGEMENT OF THE DISEASE -
syndrome, known as renal tubular acidosis.
MEDICAL/SURGICAL/DENTAL
Treating Dry Eyes and Eye Inflammation Caused by
Sjogren’s Syndrome
● Artificial tears during day, gel at night
2. Eye tests
● Blepharitis – hot compress, eyelid cleansers,
eyelid massage
- Schirmer’s test ● Eye drops
● Punctal occlusions

Many people can treat their dry eyes with artificial tears
during the day and a gel at night. Some patients may have
eyelid inflammation (blepharitis) and benefit from the use of
hot compresses and eyelid cleansers, and gentle eyelid
massage to relieve blocked oil glands in the eyelids.
Others may require prescription eye drops.

Some patients find it helpful to wear wraparound


sunglasses or goggles, or to add shields to the sides of
Schimer’s test to see if your tear glands are producing
their glasses. These options help reduce tear evaporation
enough tears to keep your eyes moist.
by protecting your eyes from wind, air and airborne
particles.
- Ocular surface staining
For more significant cases of dry eye, your doctor might
recommend punctal occlusion. In this simple procedure, an
ophthalmologist inserts tiny plugs into your tear ducts to
block them. Tears stay on your eyes longer, which helps
keep them moist.

Treating Dry Mouth and Related Symptoms Caused by


Sjögren’s Syndrome
As with treating dry eye, there are over-the-counter options Using products made for vaginal dryness, such as vaginal
for dry mouth symptoms. They include: moisturizers, estrogen cream, vitamin E oil, hyaluronic acid
suppositories and vaginal lubricants can be useful. Also,
● Sugarless candy, sugar free gum applying adequate artificial lubrication to both partners can
● Lozenges help prevent painful intercourse.
● Pilocarpine
People with Sjögren’s syndrome may experience joint pain.
Sucking on sugarless candy and chewing sugar free gum. Nonsteroidal anti-inflammatory drugs (NSAIDs) such as
If you don’t find that effective, prescription medications are ibuprofen are recommended for the joint pain that may
available that can help increase your saliva production. accompany Sjögren’s syndrome. Low-dose steroids such
Sipping water during the day is the easiest way to ease dry as prednisone can also reduce joint pain. Medications
mouth. An artificial saliva product, such as a spray or called disease-modifying anti-rheumatic drugs,which slow
lozenge, may also help. The medicine pilocarpine can be the effects of lupus and rheumatoid arthritis, have been
used to treat a dry mouth. It comes as tablets that help used successfully to treat Sjögren’s syndrome joint pain. A
your body produce more saliva. medicine called hydroxychloroquine is sometimes
recommended by Sjögren's syndrome specialists as a
People with Sjögren’s syndrome are at increased risk treatment for joint pain or stiffness if other methods have
for cavities and should be sure to: not helped. Exercise regularly – a mixture of aerobic
exercises (such as cycling) and strength and flexibility
exercises may be helpful.
● Brush and floss after meals and snacks.
● See a dentist at least twice each year.
● prescription fluoride toothpaste, fluoride rinse, ● (NSAIDS) Ibuprofen
professional fluoride treatment ● Disease-modifying anti-rheumatic drugs
● Hydroxychloroquine
● Exercise regularly
Brush and floss after meals and snacks. See a dentist at
least twice each year. Use of fluoride treatments that can
prevent cavities The options include daily use of a
prescription fluoride toothpaste, a fluoride rinse, or a
professional fluoride treatment after a dental cleaning.

Increased risk of contracting fungal infections of the


mouth,

● prescription anti-fungal pills or medicated troches

You may also have an increased risk of contracting fungal


infections of the mouth, which can be treated with
prescription anti-fungal pills or medicated troches that
dissolve slowly in your mouth. If you wear dentures, be
sure to disinfect them every night.

Treating Other Symptoms Related to Sjögren’s Syndrome

People with Sjögren’s syndrome may experience dryness


in other areas, including the lips, the nose, the skin and
the vagina.

● Petroleum jelly, lip balms


● Saline nasal spray
● Skin lotion
● vaginal moisturizers, estrogen cream, vitamin E
oil, hyaluronic acid suppositories, vaginal
lubricants
● adequate artificial lubrication

For dry lips, petroleum jelly and lip balms can help.
Blocked nasal passages and a dry nose increase your
need to breathe through the mouth, which can make your
dry mouth worse. Saline nasal sprays can be helpful. Be
sure to treat other causes of congestion, such as allergies
and sinus infections, quickly. Dry skin usually improves
when you use skin lotion regularly and throughout the day,
especially after baths or showers. Some women
experience vaginal dryness, especially after menopause.

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