Sarcoidosis is a condition where tiny nodules (lumps), known as granuloma, develop at various sites within your body due to inflammation. It most commonly affects the lungs. However, it can affect almost any part of the body. The exact cause is not known. In many people, sarcoidosis goes away without any treatment. However, in some people, it can become long-standing, serious, and treatment may be needed.
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.
The exact cause of sarcoidosis is not known. However, it does seem to run in some families so it is likely that some 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 definite infection or agent acting as this trigger has been identified.
Sarcoidosis is rare. Every year in the UK, around 3,000 people are diagnosed with sarcoidosis for the first time. 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 can affect almost any part of your body. It can also affect more than one part of your body at the same time:
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 about 4 in 10 people with sarcoidosis, the symptoms come on quite quickly, over a couple of weeks or so. Some doctors call this acute sarcoidosis. 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 (see below) 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.
The symptoms can vary from person to person. This is because sarcoidosis can affect so many different parts of the body. Just one part of your body may be affected by sarcoidosis. Or, more than one part of your body may be affected. Some of the various parts of your body that can be affected and their associated symptoms include the following:
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.
Your doctor may ask you a number of questions if they suspect that you have sarcoidosis. This is to look for any symptoms that you may have and to see which parts of your body sarcoidosis could be affecting. Depending on your symptoms, your doctor may also suggest that they examine you. For example, they may suggest that they listen to your chest, carry out an examination of your lymph glands, examine your skin etc.
Your doctor may then suggest some tests. Some of these tests may depend on which part of your body is affected. Tests may include:
If your doctor suspects sarcoidosis, 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:
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 symptoms - for example, pain from the nodules of erythema nodosum, or joint pains.
If treatment is needed for sarcoidosis, the aim of treatment is to reduce the inflammation and therefore the symptoms that it causes.
The main treatment for sarcoidosis is usually with steroid tablets. The steroids help to reduce inflammation. Prednisolone is usually used and generally needs to be taken daily at first. Treatment with steroid tablets is often needed for at least 6 to 24 months. Over this time, the dose of the steroid may be gradually reduced. 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. Steroid drops or ointments are sometimes used for sarcoidosis affecting the eyes.
Sometimes, steroid tablets may not be effective or may cause side-effects. Side-effects can include:
See separate leaflet called 'Steroid Tablets' for more details.
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 hydroxychloroquine, methotrexate, azathioprine and cyclophosphamide. Infliximab, thalidomide and tetracyclines have also been used in the treatment of sarcoidosis.
Some of these drugs may be used alone (if steroids are not working), or they may be used in combination with steroids to reduce the dose of steroid needed. Each of the drugs has different possible side-effects. Some side-effects can be serious. Serious side-effects are rare but 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 aim is that the tests look for possible side-effects before they become serious. You should ask your specialist to discuss in detail the side-effects of any medication that you are prescribed.
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.
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.
At each follow-up appointment the specialist 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 your symptoms and which part of your body is affected.
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.