Download Sjogren syndrome

SJOGREN SYNDROME
Setayesh Nekarae, Ramina Mirzazadeh,
Shaghayegh Ekrami, Rose Bandvar
10A
What is the Sjogren Syndrome?
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Sjögren is a chronic autoimmune disease in which people’s white
blood cells attack their moisture-producing glands. In Sjogren's
syndrome, the mucous membranes and moisture-secreting glands
of your eyes and mouth are usually affected first — resulting in
decreased production of tears and saliva.
More than 4,000,000 of Americans are suffering from
Sjögren’s. It is one of the most prevalent autoimmune disorders.
Does this syndrome occur alone?
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About half of the time Sjögren’s occurs alone,
and the other half it occurs in the presence of
another autoimmune connective tissue disease
such as rheumatoid arthritis, lupus, or
scleroderma also have a higher risk of
developing lymphoma.
Symptoms of Sjogren Syndrome
PRIMARY SYMPTOMS
Dry eye
i. Gritty, sandy feeling
ii. Stinging feeling
Dry mouth
i. Dry, cracked tongue
ii. Sore throat
iii. Difficulty talking
iv. Difficulty swallowing
v. Difficulty chewing dry
food
vi. Change in sense of
taste/smell
vii. Increase in cavities
viii. Mouth sores
ix. Cracked lips
MAY CAUSE
DYSFUNCTION OF
OTHER SYMPTOMS
i.
ii.
iii.
iv.
v.
vi.
vii.
viii.
ix.
x.
xi.
xii.
xiii.
Swollen partoid glands
Nausea
Dry skin
Joint pain
Dry nose
Reflux
Muscle pain
Fatigue
Muscle weakness
Low-grade fever
Vaginal dryness
Neuropathy
Dizziness
i.
ii.
iii.
iv.
v.
vi.
vii.
Kidneys
Gastrointestinal system
Blood vessels
Lungs
Liver
Pancreas
Central Nervous System
Symptoms of Sjogren Syndrome
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Symptoms may remain steady, worsen, or, uncommonly, go into
remission. Since the symptoms of this autoimmune disorder such as
dry eyes and dry mouth are very common among people, and
mostly observed from the age of 40 and above, it is often
mistaken as age-related, thus ignored. However, some
medications can also cause symptoms that are similar to those of
Sjögren's syndrome.
Symptoms of Sjogren Syndrome
Who is more prone to get it?
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Nine out of ten Sjögren's patients are reported to be
women. In addition to prevalence in women, having a firstdegree relative with an autoimmune disease and previous
pregnancies have been identified as epidemiological risk
factors.
Differences in prevalence due to race and ethnicity are
unknown.
Causes of Sjogren Syndrome
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Sjogren's syndrome is an autoimmune disorder. This means that your immune
system mistakenly attacks your body's own cells and tissues.
Although the cause of Sjögren’s syndrome is unknown, it is believed to be under
the influence of a combination of genetic, environmental, and several other
factors, as is the case with many other autoimmune disorders
Scientists aren't certain why some people develop Sjogren's syndrome and
others don't. Certain genes put people at higher risk of the disorder, but it
appears that a triggering mechanism — such as infection with a particular
virus or strain of bacteria — is also necessary.
Hormonal factors:
Sex hormones seem to influence humoral and cell-mediated immune response,
with estrogen being considered one of the biggest factors responsible for
gender immunologic dimorphism. Various animal models have indicated a role
for estrogen deficiency in Sjögren's syndrome; mice that lack aromatase
(estrogen deficiency) develop a lymphoproliferative disease that resembles
Sjögren's.
Diagnosis
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Early diagnosis and proper treatment are important — they
may prevent serious complications and greatly improve a
patient’s quality of life.
Since symptoms of Sjögren’s mimic other conditions and
diseases, Sjögren’s can often be overlooked or
misdiagnosed.
On average, it takes nearly 3.9 years to receive a diagnosis
of Sjögren’s.
Types of Diagnosis
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Sjogren's syndrome can be difficult to diagnose because the signs and
symptoms vary from person to person and can be similar to those
caused by other diseases. Side effects of a number of medications also
mimic some signs and symptoms of Sjogren's syndrome.
A variety of tests can help rule out other conditions and help pinpoint a
diagnosis of Sjogren's syndrome.
Blood tests
Your doctor may order blood tests to check for:
•Levels of different types of blood cells
•Presence of antibodies common in Sjogren's syndrome
•Evidence of inflammatory conditions
•Indications of problems with your liver and kidneys
Types of Diagnosis
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Eye tests
Your doctor can measure the dryness of your eyes with a test called a
Schirmer tear test. In this test, a small piece of filter paper is placed under
your lower eyelid to measure your tear production.
A doctor specializing in the treatment of eye disorders (ophthalmologist)
may also examine the surface of your eyes with a magnifying device called
a slit lamp. He or she may place drops in your eye that make any damage
to your cornea easier to see.
Types of Diagnosis
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Imaging
Certain imaging tests can check the function of your salivary glands.
• Sialogram
A special X-ray called a sialogram can detect dye that's injected into the
salivary glands located in front of your ears. This procedure shows how
much saliva flows into your mouth.
• Salivary scintigraphy.
This nuclear medicine test involves the intravenous injection of a radioactive
isotope, which is tracked over the course of an hour to see how quickly it
arrives in all your salivary glands.
Types of Diagnosis
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Biopsy
Your doctor may also want to do a lip biopsy to detect the
presence of clusters of inflammatory cells, which can indicate
Sjogren's syndrome. For this test, a small sliver of tissue is removed
from salivary glands located in your lip and examined under a
microscope.
Treatment
Neither a cure for Sjögren's syndrome nor a specific treatment is known to permanently
restore gland secretion. Instead, treatment is generally symptomatic and supportive.
Moisture replacement therapies such as artificial tears may ease the symptoms of dry
eyes. Some patients with more severe problems use goggles to increase local humidity or
have punctal plugs inserted to help retain tears on the ocular surface medications.
Depending on your symptoms, your doctor may suggest medications that:
• Increase production of saliva Drugs such as pilocarpine (Salagen) and cevimeline
(Evoxac) can increase the production of saliva, and sometimes tears.
Side effects may include sweating, abdominal pain, flushing and increased urination.
•Address specific complications If you develop arthritis symptoms, you may benefit from
nonsteroidal anti-inflammatory drugs (NSAIDs) or other arthritis medications.
Prescription eyedrops may be needed if over-the-counter drops aren't helpful. Yeast
infections in the mouth should be treated with antifungal medications.
• Treat systemwide symptoms Hydroxychloroquine (Plaquenil), a drug designed to treat
malaria, is often helpful in treating Sjogren's syndrome.
Drugs that suppress the immune system, such as methotrexate, also may be prescribed.
Surgery
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To relieve dry eyes, you may consider undergoing a minor surgical
procedure to seal the tear ducts that drain tears from your eyes
(punctal occlusion).
Collagen or silicone plugs are inserted into the ducts for a
temporary closure. Collagen plugs eventually dissolve, but silicone
plugs stay in place until they fall out or are removed.
Alternatively, your doctor may use a laser to permanently seal your
tear ducts for a longer time.
Prevention
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There is no prevention mechanism for Sjögren's syndrome due to its
complexity as an autoimmune disorder.
However, lifestyle changes can reduce the risk factors of getting
Sjögren's syndrome or reduce the severity of the condition with
patients who have already been diagnosed.
Diet is strongly associated with inflammation that is mostly seen in
many autoimmune related diseases including Sjögren's syndrome. An
experimental study show that Sjögren's syndrome patients show high
sensitivity to gluten that directly relates to inflammation.
Moderate exercise is also found be helpful in Sjögren's syndrome
patients mainly reducing the effect of lung inflammation
Complications
Complications of the Sjogren syndrome include:
• The most common complications of Sjogren's syndrome involve your eyes and
mouth.
• Dental cavities. Because saliva helps protect the teeth from the bacteria that
cause cavities, you're more prone to developing cavities if your mouth is dry.
• Yeast infections. People with Sjogren's syndrome are much more likely to
develop oral thrush, a yeast infection in the mouth.
• Vision problems. Dry eyes can lead to light sensitivity, blurred vision and
corneal ulcers.
Less Common Complications
Less common complications may affect your:
• Lungs, kidneys or liver. Inflammation may cause pneumonia,
bronchitis or other problems in your lungs; may lead to problems
with kidney function; and may cause hepatitis or cirrhosis in your
liver.
• Lymph nodes. A small percentage of people with Sjogren's
syndrome develop cancer of the lymph nodes (lymphoma).
• Nerves. You may develop numbness, tingling and burning in your
hands and feet (peripheral neuropathy).