Sarcoidosis is a rare inflammatory disease that can develop in any part of the body. When the disease occurs in the lungs, it is called pulmonary sarcoidosis.
What is pulmonary sarcoidosis?
Inflammation, caused by specialized cells, is a targeted response the immune system uses to defend the body against infections. If the immune system cannot immediately destroy a threat, it can form granulomas, or clumps of inflammatory cells, to surround a threat in the body and isolate it so it can be destroyed later. Once the threat is removed, the granulomas naturally dissipate.
Sarcoidosis is characterized by the abnormal formation of granulomas that build up in organs and tissues. In about 90 percent of cases, these granulomas form in the lungs. When this happens, it can lead to inflammation and scarring of the lung tissue. Lung scarring, also called pulmonary fibrosis, causes structural changes and results in the lung tissue becoming thicker and stiffer, which can make it difficult to breathe and reduces the amount of air the lung can hold.
The exact cause of sarcoidosis is still unknown, but there is evidence suggesting that certain inherited factors could increase the risk of developing the disease. For example, an overreaction of the immune system in response to an infection or an environmental factor, such as dust or chemicals, can trigger sarcoidosis.
Symptoms of pulmonary sarcoidosis
Symptoms of pulmonary sarcoidosis can vary based on how advanced the condition is and may differ from one individual to the other. Some people may not have any symptoms at all.
Common symptoms of pulmonary sarcoidosis include:
- Shortness of breath
- Persistent dry cough
- Chest pain
- Wheezing
In rare cases, pulmonary sarcoidosis can cause bronchiectasis, inflammation and scarring in the airways, leading to mucus being trapped and increasing the risk of infections.
Other general symptoms of sarcoidosis, which can also occur in pulmonary sarcoidosis, include:
- Tiredness
- Fevers
- Red or sore eyes
- Night sweats
- Joint, muscle, and bone pain
- Skin rashes or lumps
- Swollen lymph nodes in the face, neck, armpits, or groin
- Abnormal heart rhythm
- Weight loss
Diagnosis of pulmonary sarcoidosis
There is no single test to identify pulmonary sarcoidosis so several tests are usually needed to exclude other lung disorders before reaching a diagnosis.
These include imaging tests to examine the condition of the lungs, such as a chest X-ray, CT scan, or bronchoscopy.
A lung biopsy, in which a small piece of lung tissue is removed to be examined under a microscope, may also be done to check for abnormalities associated with pulmonary sarcoidosis.
As pulmonary sarcoidosis generally affects how well the lungs work, several pulmonary function tests may be carried out. These can assess how easily air can move in and out of the lungs, and the volume of air being exchanged.
Blood tests may reveal whether there are abnormal oxygen or carbon dioxide levels in the blood, indicating a problem with breathing. Blood and urine tests may also reveal whether there are issues with other organs, such as the kidneys or liver, which may also be affected by sarcoidosis.
Pulmonary sarcoidosis may be diagnosed as a particular stage, which is used as an indication of where the granulomas are found. The stages do not indicate a progression of the condition, and the patient may move between stages. The stages are:
- Stage 1: granulomas present in the lymph nodes only
- Stage 2: granulomas present in both the lymph nodes and lungs
- Stage 3: granulomas present in the lungs only
- Stage 4: scarring of the lung tissue and permanent damage
Treatment of pulmonary sarcoidosis
Treatment is generally focused on managing symptoms of the disease or suppressing the immune system. For example, to manage joint and muscle pain, painkillers such as ibuprofen may be recommended. Antibiotics may be prescribed for an infection.
In some cases, the granulomas can dissipate on their own, and as long as no permanent damage is done to the lungs, the patient may not need treatment.
If the condition persists, the most commonly prescribed therapy are steroids, such as prednisone. These can help control and reduce inflammation. They can be taken orally or inhaled directly into the lungs.
If steroid therapy does not work, patients may be prescribed a therapy to suppress the immune system such as methotrexate.
Rarely, for very severe cases, oxygen therapy or a lung transplant may be required if there is extensive damage to the lungs.
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