Sarcoidosis is a condition causing inflammation within the body. Tiny
lumps (granuloma) form as a response to the inflammation at various
sites within the body. Sarcoidosis most commonly affects the lungs and
lymph glands in the chest area. However, it can affect almost any part
of the body. The exact cause is not known. In many people, sarcoidosis
can go away without any specific treatment. However, in some people, it
can become longstanding and treatment may be needed.
What is sarcoidosis?
Sarcoidosis is a
condition causing inflammation within the body. This inflammation
causes tiny lumps to form, known as granuloma, at various sites within
the body. Sarcoidosis most commonly affects the lungs and lymph glands
in the chest area (pulmonary sarcoidosis). However, it can affect
almost any part of the body including the eyes, skin, heart, liver,
kidneys, salivary glands, muscles, nose, sinuses, brain and nerves. It
is the presence of the granulomas formed in response to the
inflammation that causes the symptoms in the various parts of the body.
What causes sarcoidosis?
The
exact cause of sarcoidosis is not known. It seems likely that there is
some genetic susceptibility to sarcoidosis as it can run in families.
It has been suggested that an infection, or some environmental agent,
may trigger sarcoidosis in someone who is genetically prone to it. So
far, no specific infection or agent acting as this trigger has
definitely been identified.
Who gets sarcoidosis?
Sarcoidosis is
relatively rare and affects about 10 to 20 people per 100,0000 in the
UK. Women are a little more likely to be diagnosed with sarcoidosis
than men. It is most commonly first diagnosed between the ages of 20 to
40 years. However, sarcoidosis can affect younger or older people.
Sarcoidosis seems to be more of a problem in some countries rather than
others. It is most common in Sweden and Denmark. It also seems to be
more common in Afro-Caribbeans than other ethnic groups.
Which parts of the body are affected in sarcoidosis?
Sarcoidosis can affect almost any part of the body. It can also affect more than one part of the body at one time.
- The lungs are most commonly involved in sarcoidosis. 9 in 10 people with sarcoidosis have lung involvement.
- The lymph glands, either in the chest or elsewhere in the body, are affected in 3 in 4 people with sarcoidosis.
- The skin is affected in 1 in 4 people with sarcoidosis.
- The eyes are affected in 1 in 4 people with sarcoidosis.
- The heart is affected in 1 in 20 people with sarcoidosis.
- The nerves and nervous system is affected in 1 in 20 people with sarcoidosis.
- Sarcoidosis can also affect other parts of the body including the
bone marrow, the liver, the spleen, the kidneys, the heart, the nervous
system and the gut.
How does sarcoidosis develop and progress?
Up
to half of people with sarcoidosis do not know that they have it. In
these people it is diagnosed when they have a chest X-ray for another
reason. They have no symptoms.
In about 4 in 10 people with
sarcoidosis, the symptoms come on quite quickly over a couple of weeks.
This may be referred to as acute sarcoidosis. Generally, acute
sarcoidosis has a good outlook and tends to go away without treatment.
Löfgren syndrome is one type of acute sarcoidosis which is a
combination of erythema nodosum (see below) with swollen or enlarged
lymph glands seen on your chest X-ray, uveitis (inflammation in the
eye) and joint pains. It tends to come on quickly and often goes away
without specific treatment.
In others, symptoms come on more
slowly over a few months. This may be referred to as chronic
sarcoidosis. Symptoms can get worse over time and often treatment is
needed.
What are the symptoms of sarcoidosis?
The
symptoms in sarcoidosis can vary between people. This is because
sarcoidosis can affect so many different organs and parts of the body.
General symptoms can include fever, loss of appetite, feeling tired,
weight loss and joint pains. Other symptoms depend on the area of the
body affected:
-
Lungs - You may notice shortness of
breath (particularly on exertion), cough, chest pain or, rarely, you
may cough up blood (haemoptysis).
-
Lymph glands - These may be swollen and you may notice lumps
under your arms, in your neck or in your groins. Doctors may also
notice swollen glands in your chest area when they look at your chest
X-ray.
-
Skin - You may develop small nodules (lumps) just under the
surface of your skin. You may also develop a purplish, raised rash on
your nose, cheeks, chin and ears. This rash is called lupus pernio.
Erythema nodosum is a condition that causes red nodules (rounded
lumps), most commonly on the shins. This may develop at the start of
sarcoidosis and is the most common skin rash in sarcoidosis. (See is a
separate leaflet called 'Erythema Nodosum').
-
Eyes - Sarcoidosis can cause inflammation within your eyes,
including uveitis. This can affect your vision. Your eyes can become
red and painful. (See separate leaflet on 'Uveitis and Iritis'.) You should see a doctor straight away if you have sarcoidosis and notice any eye symptoms.
-
Heart - Sarcoidosis can affect your heartbeat by making it
slower or irregular. Damage to the lungs caused by sarcoidosis may also
cause changes in the right side of your heart and it can become
enlarged. This is known as cor pulmonale and can lead to heart failure
if left untreated. Your heart can also become generally enlarged in
sarcoidosis, known as cardiomyopathy. This means that your heart cannot
beat as strongly or effectively and you can become breathless. It can
also lead to heart failure.
-
Nerves - Your nervous system can be affected in a number of
ways in sarcoidosis. It can lead to headaches, problems swallowing,
drooping of your face, a type of meningitis, eyesight or hearing
problems. It can also cause numbness and pins and needles in your face,
arms or legs. Rarely, it can lead to convulsions or stroke.
-
Kidneys - Sarcoidosis mainly affects the kidneys by causing kidney stones because of high levels of calcium in the blood.
-
Liver and spleen - These organs may become enlarged in
sarcoidosis. Rarely this can cause problems with your blood clotting or
can lead to anaemia.
How is sarcoidosis diagnosed?
The
investigations that your doctor will carry out if they suspect that you
have sarcoidosis will depend on which part of your body is affected by
the inflammation.
Tests to look for lung or chest involvement
-
Chest X-ray and CT scan
- If your doctor suspects that your lungs may be affected by
sarcoidosis, they will usually arrange a chest X-ray. They may also
suggest a CT scan of your lungs and chest area.
-
Lung function tests - You may be referred for lung function
tests, or spirometry. A spirometer is a device which measures the
amount of air that you can blow out. (See separate leaflet on 'Spirometry'.)
-
Lung biopsy - To make a definite diagnosis of sarcoidosis,
in most cases, a biopsy needs to be taken from one of the areas of
inflammation (granulomas). In the lungs, this is usually carried out by
passing a small telescope via the nose, down the trachea (windpipe) and
into the lungs. The procedure is known as a bronchoscopy. A sample of
tissue is taken from the lungs and is sent to the laboratory. The
inflammation of sarcoidosis has a characteristic appearance when it is
examined under a microscope.
Tests to look for heart involvement
-
ECG
- If your doctor suspects that your heart may be affected by
sarcoidosis, they may arrange an electrocardiogram or 'ECG'. An ECG
records the electrical activity of the heart and shows if there are any
problems with this. Your doctor may also suggest an exercise ECG which
records the electrical activity of your heart whilst you exercise. (See
separate leaflets on 'Electrocardiogram (ECG)' and 'Electrocardiogram (ECG) - Exercise'.)
-
Echocardiogram - You may be referred for an echocardiogram
(an ultrasound scan of your heart). This can show if the heart is
enlarged or affected by sarcoidosis. Sometimes, your doctor may suggest
more detailed scans to look at your heart. (See separate leaflet on 'Echocardiogram'.)
Tests to look for eye involvement
Your
doctor may refer you for a detailed examination of your eyes by an eye
specialist, even if you do not have any specific eye symptoms. This is
because sarcoidosis involving the eyes can seriously affect the vision
if it is not treated quickly. The examination will usually involve the
specialist using a slit-lamp (a special microscope) to examine your
eyes.
Other tests
-
Blood tests - Your
doctor may also suggest some blood tests to look for signs of
inflammation. They may check the level of calcium in your blood as this
can be raised in sarcoidosis. They may also check the levels of an
enzyme (a protein) in your blood known as angiotensin-converting enzyme
(ACE). In about three quarters of people with sarcoidosis, the levels
of this enzyme are raised. Sometimes, doctors use repeated measurements
of ACE levels to monitor the response to treatment in sarcoidosis.
-
Biopsy - Your doctor may also suggest that they take a
biopsy (a sample) from any other area of your body affected by the
sarcoidosis, for example your skin or a lymph gland. Like with the
biopsy from the lung, the sample of tissue is sent to the laboratory.
The inflammation of sarcoidosis has a characteristic appearance when it
is examined under a microscope.
-
Other investigations - Your doctor may refer you for other
investigations depending on your symptoms. For example, if you have
pins and needles, headaches or numbness, suggesting that sarcoidosis
may be affecting your nervous system, they may refer you for a CT or
MRI scan of the brain.
What is the treatment for sarcoidosis?
More
than three-quarters of people with sarcoidosis do not need any specific
treatment because their symptoms are not severe. They may just need to
take nonsteroidal anti-inflammatory drugs, such as ibuprofen, to ease
general symptoms such as joint pains.
If treatment is needed for
sarcoidosis, the aim of treatment is to reduce the inflammation and
therefore the symptoms that it causes.
Steroids tablets
The main treatment for
sarcoidosis is usually with steroid tablets taken by mouth. The
steroids help to reduce the inflammation. Prednisolone is usually used
and generally needs to be taken daily initially. Treatment may be
needed for up to six months. Sometimes longer courses of steroid
treatment are needed. You are more likely to need treatment with
steroid tablets if sarcoidosis is affecting your nervous system, heart
or eyes, or if you have high calcium levels in your blood or severe
breathing/lung symptoms.
Sometimes, steroid tablets may not be effective or may cause side effects. Side effects can include:
- An increased risk of infection.
- Thinning of the skin.
- Thinning of the bones (osteoporosis).
- Difficulty sleeping and mood changes.
- Easy bruising.
- Weight gain.
- High blood pressure.
- An increased risk of developing diabetes.
Steroid drops or ointments
Steroid drops or ointments are sometimes used for sarcoidosis affecting the eyes.
Other drug treatments
If steroid tablets are
causing side effects, or are ineffective, there are some other drugs
that can be used as alternatives. These are broadly known as cytotoxic
or immunosuppressive drugs. They include drugs such as methotrexate,
azathioprine and cyclosporin. Infliximab, thalidomide and tetracyclines
have also been used in the treatment of sarcoidosis as well as the
anti-malarial agent chloroquine.
These drugs may be used alone
(if steroids are not working) or may be used in combination with
steroids to reduce the dose of steroid drugs needed. Each of the drugs
has different possible side-effects. Some side-effects can be serious.
These are rare and include damage to the liver and blood producing
cells. Therefore, it is usual to have regular tests - usually blood
tests - whilst you take some of these drugs. The tests look for
possible side-effects before they become serious. You should ask your
specialist to discuss the side effects of any medication that you are
prescribed in detail.
Surgery and non-drug treatment
Very rarely,
sarcoidosis can cause severe scarring to the lungs. One of the options
for treatment if this occurs is lung transplantation. Heart
transplantation has also been used in rare cases where sarcoidosis
severely affects the heart. Occasionally, people with sarcoidosis that
is affecting the heart may need a pacemaker to help control the rhythm
of the heart.
Will I need any follow-up or monitoring?
Generally,
if you have sarcoidosis, you are seen regularly by a specialist in an
outpatient clinic. The specialist that you see will depend on which
area of the body the sarcoidosis is affecting. For example, it is
likely that you will see a respiratory (lung) specialist if the
sarcoidosis is affecting your lungs and an ophthalmology (eye)
specialist if it is affecting your eyes. You may need to see more than
one specialist.
At each follow-up appointment your doctor may
ask you questions about any symptoms that you have, they may arrange a
chest X-ray and some lung function tests. They may arrange examination
of your eyes. They may also suggest other tests depending on which part
of your body is affected by the sarcoidosis.
What is the outlook (prognosis) for sarcoidosis?
Up
to two thirds of people with sarcoidosis do not need any specific
treatment and the sarcoidosis will spontaneously improve over the
following two to five years. In about one third of people, sarcoidosis
becomes chronic, or long-term and does require treatment. A minority of
people with sarcoidosis can be severely affected and may die. This is
usually due to severe involvement of the lungs and respiratory (lung)
failure.
The outlook is worse if sarcoidosis affects areas of
the body outside the lungs, particularly if it affects the heart or the
nerves. People who develop sarcoidosis quickly, over a few weeks, tend
to have a better outlook. As do those who develop erythema nodosum, or
those who just have swollen lymph glands seen on their chest X-ray and
no real breathing or chest symptoms.
Further help and advice
Sarcoidosis and Interstitial Lung Association (SILA)
Chest Clinic Office, 2nd Floor Admin Block, King's College Hospital, Denmark Hill, London, SE5 9RS.
Contact by post or via their website: www.sila.org.uk
Information and support activities for sufferers.
British Lung Foundation
73-75 Goswell Road, London EC1V 7ER
Telephone: 08458 50 50 20 Web: www.lunguk.org
Offers support for everyone affected by lung disease. Provides information about sarcoidosis and other lung diseases.
British Association of Dermatologists
Willan House, 4 Fitzroy Square, London, W1T 5HQ.
Telephone: 0207 383 0266 Web: www.bad.org.uk
See their patient information section on their website which includes information about sarcoidosis.
References
Comprehensive patient resources are available at www.patient.co.uk
Disclaimer: This article is for information only and should not
be used for the diagnosis or treatment of medical conditions. EMIS and
PiP have used all reasonable care in compiling the information but make
no warranty as to its accuracy. Consult a doctor or other health care
professional for diagnosis and treatment of medical conditions.
© EMIS and PiP 2008 Updated: 23 May 2008
