Pulmonary Sarcoidosis

What is pulmonary sarcoidosis?

Sarcoidosis is a disease that causes inflammation (swelling). It causes tiny, grain-like lumps of immune cells to form, called granulomas.

Pulmonary sarcoidosis affects the lungs. Sarcoidosis can also affect the skin, lymph nodes or any other organ in the body.

Many cases of pulmonary sarcoidosis go away. But the inflammation can cause permanent scarring in the lungs.

Is pulmonary sarcoidosis a cancer?

Sarcoidosis isn’t a type of cancer. But some studies have shown that people with sarcoidosis have an increased risk of cancer.

What are the symptoms of pulmonary sarcoidosis?

Not all people with this condition have signs of disease. But pulmonary sarcoidosis symptoms may include:

• Chest pain.
• Cough that doesn’t go away.
• Fatigue, tiredness, malaise or generally not feeling well.
• Shortness of breath (dyspnea).
• Wheezing.

Many people with pulmonary sarcoidosis also experience depression. This disorder is common among those who have conditions that interfere with their daily lives or social activities.

What causes pulmonary sarcoidosis?

Scientists aren’t sure what causes pulmonary sarcoidosis. Some cases run in families. Others are related to exposure to medications, chemicals, bacteria or viruses. Researchers are continuing to study possible causes.

How common is pulmonary sarcoidosis?

In the United States, 150,000 to 200,000 people live with sarcoidosis. It affects 11 out of 100,000 white people and 34 of every 100,000 African Americans. Overall, a person has about a 2.4% chance of developing the condition.

Most cases of sarcoidosis affect the lungs.

Who might get pulmonary sarcoidosis?

Pulmonary sarcoidosis affects all genders, ages and races. But it’s more common among:

• Individuals whose ancestors came from Africa or northern Europe, such as Denmark, Norway and Sweden.
• People exposed to insecticides, mold or other substances that can cause inflammation.
• People who have taken certain medications, such as those to treat HIV, cancer or an overactive immune system.
• Those with a family history of pulmonary sarcoidosis.
• Women or people assigned female at birth (AFAB).

How is pulmonary sarcoidosis diagnosed?

If a healthcare provider suspects pulmonary sarcoidosis, they may order the following tests:

• Biopsy, which takes a small sample of tissue or fluid from the lungs to be examined under a microscope.
• Blood tests to assess whether other organs are affected.
• Chest X-rays.
• Pulmonary function testing, such as gas diffusion test, to see how well the lungs are working.
• Other tests that provide images of the lungs, such as CT scans, MRI or positron emission tomography (PET) scans.

What are the stages of pulmonary sarcoidosis?

Based on your test results and symptoms, your healthcare provider may assign a stage. The stages of pulmonary sarcoidosis range from no disease to the most severe disease:

• Stage 0: Chest X-ray is normal, with no signs of granulomas.
• Stage I: Granulomas are present only in the lymph nodes, not the lungs.
• Stage II: Granulomas are present in the lymph nodes and lungs.
• Stage III: Granulomas are present only in the lungs.
• Stage IV: Pulmonary sarcoidosis has caused permanent, irreversible scarring in the lung tissue.

What are my options for pulmonary sarcoidosis treatment?

Not everyone with pulmonary sarcoidosis needs treatment. The condition often goes away on its own.

But if you need treatment, your healthcare provider will probably recommend prednisone. This corticosteroid calms the immune system. But the medication can cause side effects if used for a long time, so it’s only appropriate for short-term treatment.

For people who need treatment for pulmonary sarcoidosis but can’t tolerate prednisone, healthcare providers may recommend:

• Corticotropin, which helps your body produce natural steroid hormones.
• Methotrexate, which can slow the immune system.
• Tumor necrosis factor (TNF) inhibitors, which are often used against rheumatoid arthritis (for example, infliximab or adalimumab).

Rare, severe cases of pulmonary sarcoidosis cause permanent lung damage, which may require:

• Pulmonary rehabilitation.
• Supplemental oxygen.
• Surgery to replace a lung with a donor lung (lung transplant surgery).

How can I prevent pulmonary sarcoidosis?

Scientists don’t understand what causes pulmonary sarcoidosis, so there are no proven strategies to prevent it. However, some cases have been linked to exposure to certain chemicals, such as pesticides. Avoiding chemicals may keep your lungs healthier.

Is pulmonary sarcoidosis life-threatening?

Many cases of pulmonary sarcoidosis get better on their own, which is called spontaneous remission. Other cases go away with short-term treatment. Those cases aren’t life-threatening.

However, over time, pulmonary sarcoidosis can create permanent scarring in the lungs, which can shorten someone’s life span.

Can pulmonary sarcoidosis come back after treatment?

Some people may achieve permanent remission. This means the condition goes away and doesn’t come back.

But pulmonary sarcoidosis can relapse (come back after remission or successful treatment). This is sometimes called a flare.

What are the complications of pulmonary sarcoidosis?

If granulomas don’t heal, they can cause complications, including:

• Bronchiectasis: Bronchiectasis is a long-term condition that affects the bronchi, large tubes that carry air from your windpipe to your lungs. Bronchi can become thick from inflammation and infection. This makes breathing difficult and causes a person to cough up mucus. It also makes the lungs prone to infections.
• Pulmonary fibrosis: Pulmonary fibrosis occurs when lung tissue becomes inflamed, scarred and stiff. It’s a serious condition that causes shortness of breath and permanent lung damage.
• Pulmonary hypertension: Pulmonary hypertension is high blood pressure in the lung arteries. It occurs when blood vessels in the lungs become too narrow.
• End-stage lung disease: This final stage of lung disease means your lungs can’t remove carbon dioxide from the body or provide enough oxygen to the body.

How do I take care of myself with pulmonary sarcoidosis?

If you’ve been diagnosed with pulmonary sarcoidosis, certain strategies can help you manage the condition:

• Avoid things that irritate the lungs, such as dust, chemicals, smoking and secondhand smoke.
• Choose nutritious foods, exercise regularly and maintain a weight that’s healthy for you, which can help reduce symptoms.
• Make and attend all recommended follow-up appointments, including blood tests and lung function tests.
• Take all medications exactly as prescribed.
• Talk to your healthcare providers and loved ones about your mental health so you can prevent or manage depression.
• Report any changes in the way you feel, especially the way you breathe.

When should I seek medical attention for pulmonary sarcoidosis?

Your healthcare provider will talk to you about signs and symptoms to report. These vary depending on the locations of sarcoidosis. With pulmonary sarcoidosis, seek medical attention for any sudden changes in your ability to breathe.

A note from Cleveland Clinic

Pulmonary sarcoidosis causes inflammation and tiny lumps called granulomas in the lungs. Many people with this condition get well on their own or feel better with short-term treatment. If you have trouble breathing, talk to a healthcare provider for diagnosis and treatment options.