Sarcoidosis

What is sarcoidosis?

Sarcoidosis is a condition where lumps called granulomas develop at different sites within the body. Granulomas are made up of clusters of cells involved in inflammation. If many granulomas form in an organ, they can prevent that organ from working properly.

What causes sarcoidosis?

The exact cause of sarcoidosis is not known. It probably involves a precise combination of genetic and environmental factors. The condition does run in some families. So far, a single factor causing sarcoidosis has not been identified.

What parts of the body can be affected?

Sarcoidosis can affect many different parts of the body.
The lungs and lymph glands in the chest are most commonly involved, affecting 9 in 10 patients with sarcoidosis.
Other parts of the body that may be commonly involved are the skin, eyes and lymph glands elsewhere in the body.
The joints, muscles and bones are involved in 1 in 5 patients.
The nerves and nervous system are involved in about 1 in 20
patients.
The heart is involved in about 1 in 50 patients.

What are the symptoms of sarcoidosis?

The symptoms of sarcoidosis depend on which part of the body is affected. They can include:

cough
feeling breathless
red or painful eyes
swollen glands
skin rashes
pain in joints, muscles or bones
numbness or weakness of the face, arms, or legs

Patients with sarcoidosis may feel tired and lethargic (fatigued), lose weight or suffer with fevers and night sweats.

Sometimes, the symptoms of sarcoidosis start suddenly and don’t last long. In other patients, the symptoms may develop gradually and last for many years. Some people don’t have any symptoms at all and are told they have sarcoidosis after having a routine chest X-ray or other investigations.

How is sarcoidosis diagnosed?

There is no single or specific test to diagnose sarcoidosis. Your specialists will take a detailed medical history and perform a thorough physical examination. You may be asked to have the following investigations:

Blood tests: Your doctor may arrange blood tests to check your kidney and liver function, and your calcium levels. They may also check a marker in your blood called angiotensin-converting enzyme (ACE), which is sometimes raised in patients with sarcoidosis.
ECG: a tracing of the electrical activity of your heart.
Chest X-ray
Lung function tests: These are breathing tests which see how well your lungs are working. These can be used to monitor your lung disease and also to see whether it is responding to treatment.
CT scan of your chest: This shows a detailed picture of your lungs. There are characteristic patterns on these pictures that can help your specialist to identify either scarring or inflammation of lung tissue.
PET CT: This is a scan of your body to look for areas that may be affected by sarcoidosis but might not be causing you any symptoms.
Biopsy: Your specialist might wish to remove a small piece of tissue to confirm the diagnosis. The site of the biopsy and specific procedure performed will be discussed with you, as it depends on which parts of the body are affected.

As sarcoidosis can affect many different parts of the body, your doctor may ask other specialists (who specialise in the part of your body affected by sarcoidosis) to look after you as well.

The outlook

Sarcoidosis gets better without treatment in most patients (around 60%). In others, the condition persists and may require some treatment. In the minority of patients that develop a more serious form of the disease, more aggressive and prolonged treatment is sometimes required.

Sometimes symptoms may suddenly get worse - this is known as a ‘flare-up’. This may be triggered by stress, infections, a change in environment or, often, nothing recognisable.

A much smaller proportion of patients develop permanent scarring of their lungs (called pulmonary fibrosis).

How is sarcoidosis treated?

Treatment may be required for patients whose sarcoidosis is causing severe symptoms or is preventing the affected organ(s) from functioning normally.

Medications

Steroids are produced naturally in the body by the adrenal gland. Additional steroid in the form of prednisolone can be given to attempt to reduce inflammation in some patients.

They are usually given in tablet form but may be given by injection into a vein. If you are prescribed steroid tablets on a long-term basis, you should not stop them abruptly.

You will be given a ‘steroid emergency card’ which you should always carry with you.
The specialist may also assess the need for bone protection medication and anti-reflux treatment to protect against some side effects whilst on steroids.

Sometimes corticosteroids may not be completely effective, or cause side effects. Other medications may be used, either alone or in combination, to help reduce the steroid dose. These are often called ‘immunosuppressive’ or ‘steroid-sparing’ medications. Methotrexate, Mycophenolate mofetil and
Azathioprine are commonly used.

Whilst you are taking immunosuppressant medication you will require regular blood tests to monitor your response to
treatment.

Clinical trials

You should also discuss with your team if there are any clinical trials in which you can participate. Clinical trials are voluntary research studies, which are designed to answer specific questions about your care or the safety and/or effectiveness of medications.

How can I help myself?

Have your annual respiratory vaccinations (COVID-19 and Flu) and the pneumonia vaccination (you only have this once).

You may be eligible for a variety of benefits such as Attendance Allowance or Personal Independence Payment if you need help with personal care or getting about.

Our specialist nurses run a regular Pulmonary Fibrosis Support Group which is a space for discussion with other patients with similar lung problems. Here we also aim to provide several presentations from a variety of guest speakers and charities.

Keep active and do what you enjoy!