Pulmonary Sarcoidosis
Pulmonary sarcoidosis is a condition where the immune system overreacts and forms tiny lumps of inflamed cells called granulomas in the lungs, affecting how well you can breathe and causing symptoms that range from mild to severe.
Table of contents
- What is Pulmonary Sarcoidosis?
- Who Gets Pulmonary Sarcoidosis?
- What Causes Pulmonary Sarcoidosis?
- Signs and Symptoms
- Stages of Pulmonary Sarcoidosis
- How is Pulmonary Sarcoidosis Diagnosed?
- Treatment Options
- Living With Pulmonary Sarcoidosis
- Outlook and Prognosis
What is Pulmonary Sarcoidosis?
Pulmonary sarcoidosis is a form of sarcoidosis—a disease that causes inflammation throughout the body—that specifically affects the lungs. The condition causes tiny, grain-like lumps of immune cells to form in the lungs. These lumps are called granulomas.[1][2]
A granuloma is an area of inflammation created by your immune system. It’s made up of a cluster of white blood cells that have been “walled off” from the rest of your body. Your immune system does this to try to protect you from something it mistakenly thought was harmful. Granulomas are surrounded by dense tissue, which makes them feel hard and lumpy.[2]
Sarcoidosis most often affects the lungs and the lymph nodes in the chest. In fact, about 90 percent of patients with sarcoidosis have lung involvement.[4][5] While sarcoidosis can affect almost any organ in the body, including the eyes, skin, heart, and brain, the lungs remain the most commonly affected area.[1]
Many cases of pulmonary sarcoidosis go away on their own. However, the inflammation can sometimes cause permanent scarring in the lungs, a condition known as pulmonary fibrosis, which makes breathing difficult.[6]
- Lungs
- Lymph nodes
Who Gets Pulmonary Sarcoidosis?
Pulmonary sarcoidosis can affect anyone, but certain groups of people are more likely to develop the condition. It is more common in Black people than in white people. Women are slightly more likely to be affected than men. Most cases are diagnosed between the ages of 25 and 40.[2][4]
People of northern European descent, especially those from Scandinavian countries like Denmark, Norway, and Sweden, also have a higher risk.[4][6] Additionally, if you have a first-degree biological relative—a parent, child, or sibling—who has sarcoidosis, you are at a higher risk of developing it yourself, though the disease itself cannot be inherited directly.[2]
In the United States, between 150,000 and 200,000 people live with sarcoidosis. It affects about 11 out of every 100,000 white people and 34 out of every 100,000 African Americans. Overall, a person has about a 2.4 percent chance of developing the condition during their lifetime.[6]
What Causes Pulmonary Sarcoidosis?
Scientists are not sure exactly what causes pulmonary sarcoidosis. The disease is likely caused by a combination of genetic and environmental factors. Some people appear to have gene changes that make them more likely to develop sarcoidosis.[1]
The condition may be triggered by bacteria, viruses, dust, chemicals, or other substances that can cause inflammation. When exposed to these triggers, the immune system may overreact, causing inflammation that forms granulomas.[1][6] Some cases are linked to exposure to insecticides, mold, or certain medications, such as those used to treat HIV, cancer, or an overactive immune system.[6]
It is important to know that sarcoidosis is not contagious, and it is not a type of cancer. However, some studies have shown that people with sarcoidosis may have a slightly increased risk of developing cancer.[5][6]
Signs and Symptoms
Many people with pulmonary sarcoidosis do not have any symptoms at all. The condition is often discovered only when a chest X-ray is done for another reason.[1][5] When symptoms do appear, they can vary widely depending on how badly the lungs are affected.
Sarcoidosis can begin with general symptoms that affect the whole body, including:[1][3][4]
- Extreme tiredness or fatigue
- Slight fever
- Swollen lymph nodes, such as in the chest, neck, armpits, or groin
- Unexplained weight loss
- Pain and swelling in joints, such as the ankles
- Night sweats
- General feelings of being unwell
When sarcoidosis affects the lungs specifically, patients may experience:[2][3][6]
- Dry cough that does not go away
- Shortness of breath, especially during physical activity
- Chest pain
- Wheezing
Many people with pulmonary sarcoidosis also experience depression. This mental health condition is common among those who have diseases that interfere with their daily lives or social activities.[6]
Stages of Pulmonary Sarcoidosis
Pulmonary sarcoidosis is sometimes described in stages called the Siltzbach classification system. These stages are based on how the lungs appear on a chest X-ray and describe where the granulomas are located.[2][6]
It is important to understand that these stages are not necessarily an indicator of how severe the disease is. You are not expected to move through each stage in order. Someone with sarcoidosis can move between stages or have granulomas disappear completely. The only stage that is irreversible is stage four, which represents permanent lung damage.[2]
- 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 both 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
How is Pulmonary Sarcoidosis Diagnosed?
Sarcoidosis can be challenging to diagnose because there is no single test that can confirm it. Symptoms often do not appear in the early stages, and when they do, they can look like symptoms of many other diseases.[4][10]
Your healthcare provider will start with a physical exam and will ask you about your symptoms and possible risk factors. The provider will also listen to your heart and lungs, check your lymph nodes for swelling, and look at any skin problems.[10]
Several tests may be ordered to help rule out other conditions and show what body systems may be affected by sarcoidosis:[3][6][10]
- Blood tests to check your overall health and how well your kidneys and liver are working
- Chest X-ray to look at your lungs and heart
- Computed tomography (CT) scan of the chest to get detailed images of your lungs
- Pulmonary function tests to measure how much air you can breathe in and out and how much oxygen your lungs send to your blood
- Electrocardiogram (ECG or EKG) to check for heart problems
- Eye exam to check for vision problems that may be caused by sarcoidosis
- Positron emission tomography (PET) scan or magnetic resonance imaging (MRI) if sarcoidosis may be affecting your heart or brain
Your healthcare provider may also take a small sample of tissue called a biopsy. The sample can be taken from any part of your body that may be affected by sarcoidosis—most commonly the lungs, skin, or lymph nodes. The sample is examined under a microscope to look for the granulomas that are typical of sarcoidosis.[3][10]
The most commonly used procedure for lung biopsy is called bronchoscopy. During this procedure, your doctor passes a small tube with a camera through your nose or mouth into your lungs. Using special ultrasound guidance, your doctor can perform biopsies of the lymph nodes in your chest.[3]
Treatment Options
Not everyone with pulmonary sarcoidosis needs treatment. The condition often goes away on its own. In fact, roughly 30 percent of cases may subside by themselves without ever being treated.[11][12] If the disease is not worsening, your provider may simply watch you closely to see whether the disease goes away on its own.[15]
If you do need treatment, your healthcare provider will likely recommend medications to reduce inflammation. The most common treatment is corticosteroids, such as prednisone. These medicines help reduce the immune system’s overreaction and decrease inflammation.[6][9][12]
When corticosteroids are started, there is a greater than 50 percent chance of needing long-term treatment. Prolonged use of prednisone at doses greater than 10 milligrams per day can be associated with serious side effects. In these cases, or when the disease does not respond to corticosteroids, your doctor may recommend other medications called steroid-sparing drugs. These include medicines like methotrexate.[11][12]
Some people may also benefit from other treatments, such as:[9]
- Oxygen therapy to help you breathe more easily
- Pulmonary rehabilitation, a program that includes exercise training, education, and support to help improve your breathing and quality of life
Even if you do not have symptoms, you should see your healthcare provider for ongoing care. Your provider will monitor you for side effects from medications and check whether the disease is getting worse.[15]
Living With Pulmonary Sarcoidosis
While there is no cure for sarcoidosis, making healthy lifestyle changes can help you manage symptoms and improve your overall health and quality of life.
Diet and Nutrition
Eating a balanced diet is important for managing sarcoidosis symptoms. Foods rich in antioxidants, such as fruits and vegetables, can help reduce inflammation. Omega-3 fatty acids, found in fish like salmon and in flaxseeds, are also known to fight inflammation. Try to avoid processed foods and those high in sugar, as they can increase inflammation and make symptoms worse.[17]
Because sarcoidosis disrupts your body’s ability to regulate calcium, you may want to avoid too much sun exposure, a diet high in dairy products, or taking vitamin D or calcium supplements. All of these can worsen problems with calcium levels in your body.[16][20]
Staying Active
Regular physical activity can greatly improve your overall health and help manage sarcoidosis symptoms. Extreme tiredness can make it hard to exercise, but physical activity can actually improve your energy levels and help with other symptoms, such as shortness of breath and muscle weakness.[15]
Gentle activities like walking, swimming, or yoga can boost your immune system, increase lung capacity, and improve your mood. It is important to start slowly and gradually increase the intensity of your workouts. Always listen to your body and avoid pushing yourself too hard.[17]
Managing Stress
Stress can worsen sarcoidosis symptoms, so finding ways to manage stress is important. Practices such as meditation, deep breathing exercises, and mindfulness can help calm your mind. Even simple activities like reading a book, listening to music, or spending time with loved ones can help reduce stress.[17]
Getting Enough Sleep
Getting adequate sleep is essential for managing sarcoidosis. Sleep helps your body heal and regenerate. Try to get seven to nine hours of sleep each night. Create a relaxing bedtime routine, avoid screens before bed, and make sure your sleeping environment is comfortable.[17]
Avoiding Triggers
Certain environmental factors can trigger sarcoidosis symptoms. Try to avoid exposure to dust, chemicals, and pollutants. If you smoke, seek help to quit, as smoking can significantly worsen your condition. Also, limit alcohol intake, as it can interfere with medications and negatively impact your immune system.[17]
Staying Hydrated
Drinking plenty of water is important for everyone, but especially for those managing sarcoidosis. Water helps keep your body hydrated and can help flush out toxins. Try to drink at least eight glasses of water a day.[17]
Support and Community
Having a strong support system can make a big difference in managing sarcoidosis. Surround yourself with family and friends who understand your condition and can offer emotional support. Joining a support group, either in person or online, can also provide comfort and practical advice from others who are experiencing the same challenges.[17][20]
Outlook and Prognosis
The outlook for people with pulmonary sarcoidosis varies widely. Many people with the condition have a good prognosis. Up to one half of people diagnosed with sarcoidosis improve without treatment, and the disease may go into remission, meaning symptoms disappear.[11][15]
If your sarcoidosis goes into remission, your doctor may carefully stop your medicines. However, you will still need to watch for a flare—a return of symptoms. If you do have a flare, you may need another round of treatment. Flares can be hard to predict, but most often they happen within six months of stopping treatment. The longer you go without symptoms, the less likely you are to have a flare.[15]
Some people have sarcoidosis that persists or comes back for many years after diagnosis. This may be called chronic, severe, advanced, or progressive sarcoidosis. Your provider will order tests to keep track of your condition and check for complications.[15]
It is important to watch for warning signs of complications that may require emergency medical treatment, such as sudden shortness of breath, muscle weakness, changes in vision, or signs of heart problems like irregular heartbeat or chest pain.[15]
Regular check-ups with your healthcare provider are essential. Your provider can monitor your condition, adjust treatments, and offer advice on lifestyle changes. Make sure to discuss any new symptoms or concerns with your doctor.[17]
