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Sarcoidosis

sarcoidosis

Sarcoidosis is a condition where tiny nodules (lumps), known as granuloma, develop at various sites within your body due to inflammation. It generally affects the lungs. However, it can affect almost any part of the body. In some people, it can become long-standing, serious, and treatment may be needed.

What do you understand by sarcoidosis?

Sarcoidosis is a condition where tiny nodules (lumps), known as granuloma, develop at various sites within your body, due to inflammation. These granulomas are made up of cells involved in inflammation.

Sarcoidosis most commonly affects the lungs and lymph glands in the chest area (pulmonary sarcoidosis, however, it can affect almost any part of your body, including your eyes, skin, heart, liver, kidneys, salivary glands, muscles, nose, sinuses, brain and nerves. If lots of granulomas form in a part (or organ) of your body, this can start to affect how that part of your body works and functions. So, it is the presence of the granulomas formed in response to inflammation that causes the symptoms of sarcoidosis in the various parts of your body.

How is sarcoidosis caused?

The exact cause of sarcoidosis is not known. However, people have a genetic susceptibility (tendency) to develop sarcoidosis. It has been suggested that something such as an infection or another 'agent' found in the environment may trigger sarcoidosis in someone who is genetically susceptible to it. So far, no trigger has been identified.

Who are more prone to get sarcoidosis?

Sarcoidosis is rare. 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. However, sarcoidosis can affect younger or older people. Sarcoidosis seems to be more common in Scandinavian people as well as African-Americans and African-Caribbeans.

Sarcoidosis affects which part of the body?

It can affect almost any part of your body. It can also affect more than one part of your body at the same time:

  • The lungs and the lymph glands in the chest area are most commonly involved in sarcoidosis. They are affected in about 9 in 10 people with sarcoidosis.
  • The skin is affected in about 1 in 4 people with sarcoidosis.
  • The lymph glands elsewhere in the body are affected in about 1 in 6 people with sarcoidosis.
  • The eyes are affected in about 1 in 8 people with sarcoidosis.
  • The nerves and nervous system are affected in about 1 in 20 people with sarcoidosis.
  • The heart is affected in about 1 in 50 people with sarcoidosis.
  • Sarcoidosis can also affect other parts of the body, including bones, joints, muscles, the liver, the spleen, the kidneys, and the gut.
What is the progress of the disease?

You may not know that you have sarcoidosis. It may be diagnosed when they you have a chest X-ray for another reason. Up to half of people with sarcoidosis do not know that they have it. They have no symptoms.

In acute sarcoidosis, the symptoms come on quite quickly, over a couple of weeks or so. Generally, acute sarcoidosis has a good outlook and tends to go away of its own accord without treatment. Löfgren's syndrome is a type of acute sarcoidosis. It is a combination of erythema nodosum with swollen or enlarged lymph glands seen on your chest X-ray, uveitis (inflammation in your eye) and joint pains.

In other people with sarcoidosis, symptoms come on more slowly over a few months or so. Some doctors call this chronic sarcoidosis. Symptoms can get worse over time and often treatment is needed.

How does sarcoidosis present?

The symptoms can vary from person to person. This is because sarcoidosis can affect so many different parts of the body. Some of the various parts of your body that can be affected and their associated symptoms include the following:

  • Lungs - you may notice shortness of breath (particularly on exertion), cough, wheezing, chest pain or, rarely, you may cough up blood (haemoptysis).
  • Skin - erythema nodosum is a condition that causes red nodules (rounded lumps), most commonly on your shins. This may develop at the start of sarcoidosis and is the most common skin rash in sarcoidosis. Other skin problems that may occur include a purplish, raised rash on your nose, cheeks, chin and ears. This rash is called lupus pernio. Some people with sarcoidosis may develop small nodules (lumps) just under the surface of their skin.
  • Lymph glands - these may be swollen and you may notice lumps under the 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.
  • Eyes - sarcoidosis can cause a type of inflammation within your eyes called uveitis. In this condition the eyes become red and painful. It can also affect vision.
  • Heart - sarcoidosis can affect your heartbeat by making it slower or irregular. Damage to your 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. Cardiomyopathy can also lead to heart failure.
  • Nervous system - your nervous system may be affected in a number of ways in sarcoidosis. For example, the nerves of your face, or your head, or your arms and legs may become affected. This may lead to problems with swallowing, drooping of your face or eyesight or hearing problems. Or you may notice numbness and pins and needles in your face, arms or legs. Sarcoidosis can also cause a type of meningitis. Rarely, it can lead to convulsions (fits) or stroke.
  • Kidneys - sarcoidosis may affect your kidneys, causing kidney stones because of high levels of calcium in your 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.
  • Bones, joints and muscles - you may experience joint pains if you have sarcoidosis. Rarely, cysts can develop in your bones. Inflammation of your muscles is also possible.
  • General symptoms may also develop which include fever, loss of appetite and weight loss. Fatigue can be a big problem for people with sarcoidosis. Some people may also become quite depressed.
How is sarcoidosis diagnosed?

Your doctor may then suggest some tests depending on the symptoms and his examination. Some of these tests may depend on which part of your body is affected. Tests may include:

  • Blood tests - your doctor may 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 your kidney and liver function and check for anaemia. Some doctors also suggest a blood test to check the levels of an enzyme (a protein) in your blood, known as angiotensin-converting enzyme (ACE). The levels of this enzyme are raised in some people with sarcoidosis.
  • Chest X-ray - if your doctor suspects that your lungs may be affected by sarcoidosis, they will usually arrange a chest X-ray.
  • Spirometry - your doctor may suggest a special test of your lungs, called spirometry. A spirometer is a device, which measures the amount of air that you can blow out.
  • Electrocardiogram (ECG) - your doctor may arrange an ECG. This records the electrical activity of your heart and shows if there are any problems with this due to sarcoidosis that has affected your heart.
  • Dipstick test of your urine - a special testing strip is dipped into your urine sample to look for any signs of blood vessel or kidney problems due to sarcoidosis.
  • Referral to a specialist would be advised after some initial tests (above), they will usually refer you to a specialist to confirm the diagnosis and for advice about how best to treat you. The specialist may suggest some other tests to help confirm the diagnosis and to see which parts of your body sarcoidosis is affecting. For example:
    • A biopsy - to make a definite diagnosis of sarcoidosis. The inflammation of sarcoidosis has a typical appearance when it is examined under a microscope. A biopsy may also be taken from other areas if sarcoidosis is suspected there. For example, your skin, lymph glands, etc. Note: not everyone with sarcoidosis needs a biopsy. For example, if you have the typical symptoms of Löfgren's syndrome, you may not need a biopsy to confirm the diagnosis.
    • CT or MRI scans - if your lungs are thought to be affected, a specialist may suggest a CT scan to allow a more detailed look at your lungs. An MRI scan of your heart or your brain may be suggested if sarcoidosis is thought to be affecting your heart or your nervous system.
    • Echocardiogram - you may be referred for an echocardiogram (an ultrasound scan of your heart). This can show if your heart is enlarged or affected by sarcoidosis.
    • More detailed lung function tests - other tests to look at how your lungs are working may be suggested. Lung function tests may also be used to look to see if your sarcoidosis is worsening or if it is responding to treatment.
    • Tests to look for eye involvement - you may be referred for a detailed examination of your eyes by an eye specialist.
How do you treat sarcoidosis?

More than three-quarters of people with sarcoidosis do not need any treatment because their symptoms are not severe. However, sometimes simple painkillers such as paracetamol or non-steroidal anti-inflammatory drugs, such as ibuprofen, may help to ease.

If treatment is needed for sarcoidosis, the aim of treatment is to reduce the inflammation and therefore the symptoms that it causes.

  • Steroid medication
  • The main treatment for sarcoidosis is usually with steroid tablets. The steroids help to reduce inflammation. 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.
  • Other drug treatments, broadly known as cytotoxic or immunosuppressive drugs can be taken. They include drugs such as hydroxychloroquine, methotrexate, azathioprine and cyclophosphamide. Infliximab, thalidomide and tetracyclines have also been used in the treatment of sarcoidosis.
  • Surgery and nondrug treatment for sarcoidosis in some settings can be used. 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.
Is regular follow-up required?

Generally, if you have sarcoidosis, you will be seen regularly by a specialist in an outpatient clinic. The specialist that you see will depend on which area of the body the sarcoidosis affects. For example, it is likely that you will see a respiratory (lung) specialist if the sarcoidosis affects your lungs and an ophthalmology (eye) specialist if it affects your eyes. You may need to see more than one specialist.

What is the outlook for sarcoidosis?

As many as 2 in 3 people with sarcoidosis do not need any specific treatment. Their sarcoidosis will get better of its own accord over the following two to five years. In about 1 in 3 people with sarcoidosis, it becomes chronic, or long-term, and may require treatment. Rarely, someone with sarcoidosis can be severely affected and may die. This is usually due to severe involvement of the lungs, leading to respiratory (lung) failure.

The outlook tends to be not so good if sarcoidosis affects areas of your body outside your lungs, particularly if it affects your heart or your 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.


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