Transcript Sarcoidosis

What Is Sarcoidosis ?
**Sarcoidosis (sar-koy-DO-sis) is a disease of
unknown cause that leads to inflammation. It
can affect various organs in the body .
**Normally, your immune system defends your body against
foreign or harmful substances. For example, it sends special cells
to protect organs that are in danger
**These cells release chemicals that recruit other cells to
isolate and destroy the harmful substance. Inflammation
occurs during this process. Once the harmful substance is
destroyed, the cells and the inflammation go away .
**In people who have sarcoidosis, the inflammation
doesn't go away. Instead, some of the immune system cells
cluster to form lumps called granulomas (gran-yu-LO-mas)
in various organs in your body .
**Sarcoidosis can affect any organ in your body. However,
it's more likely to occur in some organs than in others. The
disease usually starts in the lungs, skin, and/or lymph nodes
(especially the lymph nodes in your chest).
**If many granulomas form in an organ, they can affect
how the organ works , These granulomas might alter the
normal structure and possibly the function of the affected
organs.
Pahtophsiology :
1- T helper 1 lymphocytes play a central
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2- The net result is an amplification loop

role in the immune response , the initial
response to some infectious, genetic, or
environmental antigen is a release of
interleukin-2 and tumor necrosis factor
gamma (TNF-gamma) from Th cells which
in turn recruits more immune cells to the
area of inflammation .
involving antigen-recognition,
proinflammatory cytokine release, cell
activation, and recruitment
3- These cells accumulate within the lungs and
other affected tissues, eventually leading to the
formation of noncaseating granulomas .
4- This local immune response and granuloma
formation leads to the tissue and organ
destruction characteristic of sarcoidosis .
5- In the lungs, about 75% of the granulomas are located
close to or within the connective tissue sheath of
bronchioles and subpleural spaces. The pulmonary
vasculature is also involved in more than 50% of patients.
6-Sarcoid granulomas either resolve or leave
behind fibrotic changes .
What can happen as the
disease progresses ?
1-In many people with sarcoidosis, 
the disease appears briefly and then
disappears without the person even
knowing they have the disease.
2- Twenty percent to 30 percent of
people have some permanent lung
damage.

3- For 10 percent to 15 percent,
sarcoidosis is a chronic condition.
4- In some people, the disease
might result in the deterioration of
the affected organ. Sarcoidosis can
be fatal in up to 5 percent of
patients
Clinical Presentation
What Are the Signs and
Symptoms of Sarcoidosis?
1- Many people who have sarcoidosis have no
symptoms or mild symptoms. Often, the
disease is found during achest x- ray done for
other reason .
2- Lofgren's
Syndrome : 
Lofgren's syndrome is a classic set of signs
and symptoms that appear in some people
when they first develop sarcoidosis. Signs
and symptoms may include:


A- Fever. This symptom only occurs in
.some people
B- Enlarged lymph nodes (which can be
seen on a chest x ray ) .
C- Arthritis, usually in the ankles. This
.symptom is more common in men
D- Erythema nodosum. This is a rash of red
or reddish-purple bumps on your ankles
and shins. The rash may be warm and
tender to the touch. This symptom is more
.common in women
. Cough
.Shortness of breath
.Wheezing
.Chest pain
.Rash (may or may not be raised).
.Fever
.Pain or irritation of eyes
.Fatigue, especially with exertion
.Muscle weakness
.Night sweats
.Loss of appetite
.Weight loss
.Swollen lymph nodes
.Seizures
.Tremors
.Difficulty hearing
.Blurred vision or blindness
.Poor coordination
.Trouble walking
.Irregular heart rate
.Pain, swelling, and stiffness in the joints
.Facial paralysis ( Bells palsy).
Notes :
1- The clinical symptoms of sarcoidosis may
present in many different ways depending on the
patient's ethnicity and the chronicity, site, extent
of involvement of the organ, and the activity of
the granulomas
2- Nonspecific symptoms such as fever, fatigue,
malaise, and weight loss may occur in up to one
third of sarcoidosis patients
3- Patients may have a combination of any of
these forms of the disease
What Causes Sarcoidosis?
The cause of sarcoidosis isn't known. 
More than one factor may play a role in
.causing the disease
1- Some researchers think that 
sarcoidosis develops when your
immune system responds to a trigger,
such as bacteria, viruses, dust, or
.chemicals
2- Genetics also may play a role in
sarcoidosis. Researchers believe that
:sarcoidosis occurs if
A- You have a certain gene (or certain genes) that raise
your risk for the disease
—And—
B- You're exposed to something that triggers your
immune system .
**Triggers may vary depending on your
genetic makeup. Certain genes may
influence which organs are affected and how
.severe your symptoms are
Diagram of the stages of Sarcoidosis
Who Is At Risk for
Sarcoidosis ?
1- Sarcoidosis affects people of all ages and 
races. However, it's more common among
African Americans and Northern Europeans.
In the United States, the disease affects
African Americans somewhat more often and
.more severely than Whites
2- Studies have shown that sarcoidosis
tends to vary in different ethnic
groups. For example, eye problems
due to the disease are more common
.in Japanese people

3- Sarcoidosis is somewhat more common in
women than in men. The disease usually develops
.between the ages of 20 and 50
4- People who have certain jobs also may be at
higher risk for sarcoidosis. Examples include :
1- Health care workers
2- People whose jobs expose them to agricultural
dust, insecticides, pesticides, or mold
3- Suppliers of building materials, hardware, or
gardening materials
4- Firefighters
People who have a family history of sarcoidosis
.also are at higher risk for the disease
Diagnosis :
1- Physical Exam :

red bumps on your skin; swollen lymph
nodes; an enlarged liver, spleen, or
salivary glands; or redness in your
eyes. Abnormal breathing and
heartbeat sounds may be a sign that
sarcoidosis is affecting your lungs or
heart .

2- Chest X Ray :
The test may show granulomas or enlarged lymph
nodes in your chest. About 95 percent of people
who have sarcoidosis have an abnormal chest x
.ray
Chest X-ray changes are divided into
four stages :
**Stage 1 bihilar lymphadenopathy
**Stage 2 bihilar lymphadenopathy
and reticulonodular infiltrates
**Stage 3 bilateral pulmonary
infiltrates
**Stage 4 fibrocystic sarcoidosis
typically with upward hilar retraction,
cystic & bullous changes
3- Biopsy
confirm a diagnosis or rule out other causes 
of your symptoms. A biopsy involves taking a
small sample of tissue from one of your
.affected organs
Sarcoidosis. Lung biopsy specimen showing noncaseating
.granulomas consistent with the diagnosis of sarcoidosis
4-Blood tests :
To check for a variety of factors :
A- A complete blood count to check for
anaemia .
B- Liver function tests to check for liver
abnormalities
C- Increased calcium levels, which can
occur with sarcoidosis
D- Elevated levels of angiotensinconverting enzyme (ACE), which is often
released by the granulomas.
5- Urine test—24-hour urine test to check for
elevated levels of calcium in the urine
6- biopsy :removal of a sample of the affected
tissue for testing
7- ECG: test that records the electrical
activity of the heart.
8- Pulmonary function tests (PFTs) :tests to
determine how well you can breathe
9 – Bronchoscopy :the insertion of a lighted tube
into the lungs to look at the tissue and obtain a
sample for biopsy. bronchoalveolar lavage. This
involves flushing the lungs with fluid and
collecting the fluid for analysis.
10- Gallium scan—the injection of radioactive
material into the body. The material collects in
areas with sarcoidosis. A machine later scans the
body and identifies areas with higher
concentrations of the injected material.
11- Eye exam—done with a special lighted
instrument that allows the doctor to look
inside the eye.
12- CT scan : a type of x-ray that uses a
computer to make pictures of the inside of the
body.
13- Thyroid function tests :
Sarcoidosis may cause
Hypo or hyperthyrodism .
How Is Sarcoidosis Treated?
The goals of treatment are to:
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**Relieve symptoms 
**Improve organ function 
**Control inflammation and reduce the
)size of granulomas (inflamed lumps).
**Prevent pulmonary fibrosis (lung 
scarring) if your lungs are affected

Self-care :
To help minimize your symptoms, 
follow these guidelines :
1- .Don't smoke. If you smoke, quit 
2- Avoid exposure to dust and
chemicals

3-Notify your doctor at once if any
.symptoms develop or worsen

Prevention
.Don't smoke. If you smoke, quit

.Avoid exposure to chemicals and toxins
To prevent infection, wash your hands
before eating or preparing food and
.after using the bathroom
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ETANRCEPT :
DEFINITION : INTERFER TNF BY ACTING AS
TNF INHIBITOR .

MECHANISM OF ACTION : 
Etanercept mimics the inhibitory effects of 
naturally occurring soluble TNF receptors, the
difference being that etanercept, because it is a
fusion protein rather than a simple TNF
receptor, has a greatly extended half-life in the
bloodstream, and therefore a more profound
and long-lasting biologic effect than a naturally
occurring soluble TNF receptor
)Remicade( Infliximab
DEFINITION : it is called a chimeric
monoclonal antibody.
Infliximab blocks the action of TNFα
by preventing it from binding to its
.in RECEPTOR IN THE CELL .
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HUMIRA