Pulmonary Sarcoidosis

What is pulmonary sarcoidosis?

Sarcoidosis is a rare disease. Its cause is unknown. It's linked to an unusual cause of inflammation called granuloma. Granulomas are groups of inflamed tissue that can affect how organs work. Sarcoidosis occurs most often in the lungs and lymph nodes. But it can occur in almost any organ.

Sarcoidosis in the lungs is called pulmonary sarcoidosis. Some people have this disease along with sarcoidosis in other parts of the body. Other people have only lung disease. In rare cases, people have sarcoidosis in other parts of the body, but not the lungs. Pulmonary sarcoidosis changes the structure of the lungs. It can affect your breathing. Sometimes the lung inflammation gets better on its own without treatment. But some people have an aggressive form of the disease. This causes lifelong (permanent) loss of lung function, even with treatment.

What causes pulmonary sarcoidosis?

Experts don't know what causes pulmonary sarcoidosis. The disease is more common in people of African and Scandinavian descent. Some experts think that bacteria, viruses, or chemicals might trigger the disease. It may also be genetic. This is an active area of research.

What are the symptoms of pulmonary sarcoidosis?

Many people with sarcoidosis don't have symptoms. They likely don't know they have the disease. It can affect many organs, causing a variety of symptoms. Pulmonary sarcoidosis can reduce the amount of air the lungs can hold and cause lung stiffness.

Symptoms may be a bit different for each person. Symptoms may include:

• Shortness of breath that often gets worse with activity

• Dry cough that won't go away

• Chest pain

• Wheezing

Sarcoidosis can also cause symptoms not directly related to the lungs, such as:

• Extreme tiredness (fatigue)

• Fever

• Inflammation of the eyes and pain, burning, blurred vision, and light sensitivity

• Night sweats

• Pain in the joints and bones

• Skin rashes, lumps, and color changes on face, arms, or shins

• Swollen lymph nodes

• Weight loss

Many of these symptoms may be caused by other health problems. See your healthcare provider for a diagnosis.

How is pulmonary sarcoidosis diagnosed?

In addition to a complete health history and physical exam, you may have tests such as:

• Chest X-ray. This imaging test is used to assess the lungs, as well as the heart. Chest X-rays may show important information about the size, shape, and location of the lungs, large breathing tubes (bronchi), and the area in the middle of the chest separating the lungs (mediastinum).

• CT scan. This imaging test uses X-rays and computer technology to make horizontal images of the body. A CT scan shows detailed images of any part of the body, including the lungs. CT scans are more detailed than regular X-rays. They can be used to diagnose lung diseases, watch disease progression, and assess response to treatment.

• Pulmonary function tests. These are tests that help to measure the lungs' ability to move air in and out of the lungs. The tests are often done with special machines the person breathes into.

• Blood tests. These can be used to check the amount of carbon dioxide and oxygen in the blood, evaluate liver and kidney function, and look for infection and other diseases.

• Bronchoscopy. A long, thin, flexible tube (bronchoscope) with a light at the end is put down the throat and into the lungs. This lets the healthcare provider view the bronchi, the main airways of the lungs. It helps to evaluate and diagnose lung problems. Lung tissue samples (biopsies) and lung washings (lavage) that remove cells from the lungs can be done through the tube.

• Bronchoalveolar lavage. A sterile saline solution is put into the lungs through a bronchoscope and then suctioned out. The saline carries out cells from the lower respiratory tract. These cells can be checked under a microscope to help find inflammation and infection. The test can help rule out certain causes.

• Lung biopsy. A small piece of tissue, cells, or fluid from the lungs is taken out and checked under a microscope.

How is pulmonary sarcoidosis treated?

Treatment is generally done to control symptoms. It also helps improve the function of organs affected by the disease. Steroid medicine, such as prednisone, helps reduce inflammation. It's often taken by mouth. Other medicines, such as methotrexate, may be used as well. They help prevent steroid-related side effects. Other medicines like infliximab are also used when the disease doesn't respond to the other medicines.

Sometimes no treatment is needed. Different treatments work better for different people. Sometimes more than one treatment is used. Most medicines used to treat sarcoidosis suppress the immune system.

You may also join a rehab program that includes education, exercise, and support. Severe cases of this disease are rare. They may require oxygen therapy or even a lung transplant.

Key points about pulmonary sarcoidosis

• Sarcoidosis is caused by inflammation. Most cases are found in the lungs and lymph nodes. But it can occur in almost any organ.

• Sarcoidosis in the lungs is called pulmonary sarcoidosis. It causes small lumps of inflammatory cells, called granulomas, in the lungs. They can affect how the lungs work.

• Experts don't know what causes this condition.

• Symptoms include shortness of breath, which often gets worse with activity. Other common symptoms are a dry cough that won't go away, chest pain, and wheezing.

• Treatment is generally done to control symptoms and to improve the function of organs affected by the disease. Steroids are often used. Rehab programs that include education, exercise, and support are also used.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.

• Before your visit, write down questions you want answered.

• Bring someone with you to help you ask questions and remember what your healthcare provider tells you.

• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your healthcare provider gives you.

• Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.

• Ask if your condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if you do not take the medicine or have the test or procedure.

• If you have a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your healthcare provider if you have questions. Ask how to contact your healthcare team on evenings, weekends, and holidays if you have urgent concerns.