Sarcoidosis is a rare disease that causes inflamed lumps of tissue (called granulomas) to develop in the body. Sarcoidosis is not cancer, and it is not contagious.
Sarcoidosis happens most often in the lungs (pulmonary sarcoidosis) and the lymph nodes.
Sarcoidosis can affect multiple organs
Although sarcoidosis happens most often in the lungs (pulmonary sarcoidosis) and the lymph nodes, it can affect other organs. This is known as systemic sarcoidosis. People who have systemic sarcoidosis almost always have pulmonary sarcoidosis as well as other forms.
When granulomas don't heal on their own
In many cases, the granulomas caused by sarcoidosis heal on their own.
When they don’t, however, the tissue of your organs becomes chronically inflamed and may experience permanent damage. When this happens, it is referred to as chronic sarcoidosis. This can change the function of your organs and lead to other complications.
Sarcoidosis causes and risk factors
We do not know exactly what causes sarcoidosis, but we do know that it is related to your immune system. Your immune system fights off foreign invaders, like bacteria, viruses, fungi, and chemicals, by sending white blood cells to the infection site. This causes inflammation, which is itself a defense mechanism. Usually, once the infection has cleared, inflammation dies down.
With sarcoidosis, however, the immune system appears to go into overdrive and starts to attack your healthy tissues. Pockets of tissue remain inflamed, and white blood cells accumulate. This forms the granulomas that characterize sarcoidosis. When your immune system attacks your own cells, it is referred to as an autoimmune disease or disorder. Lupus and rheumatoid arthritis are two other autoimmune disorders that sometimes share symptoms with sarcoidosis.
Some people may have genes that make them more susceptible to this hyperactive immune response. However, there is not enough evidence right now to suggest that sarcoidosis is inherited (passed from one generation to the next). Environmental exposures to certain viruses or chemicals may also play a role in triggering sarcoidosis.
A risk factor is anything that increases your chance of developing a certain condition. However, just because you are at risk for sarcoidosis does not mean you will develop the condition.
The best-known risk factors for sarcoidosis include:
- Age. Sarcoidosis generally develops between ages 20-40. It is rarely seen in children.
- Sex. Females are slightly more likely than males to develop sarcoidosis.
- Race and ethnicity. People of African-American or northern European descent are more likely than other groups to develop sarcoidosis. African-Americans are also more likely to develop systemic sarcoidosis than other groups.
Symptoms of sarcoidosis
You may have sarcoidosis but not experience any symptoms. This is the case for most people, since sarcoidosis tends to go away on its own.
However, if you do experience symptoms, they will probably vary widely from other sarcoidosis patients. Your symptoms will depend on which organs are affected.
Common symptoms of pulmonary sarcoidosis include:
- Chest pain.
- Dry cough that won’t go away.
- Shortness of breath, which often gets worse with activity.
- Wheezing, or a whistling sound when you breathe.
The symptoms of pulmonary sarcoidosis may look like other conditions or health problems. Talk with your healthcare provider for a diagnosis.
Symptoms not directly related to the lungs
Sarcoidosis can also cause symptoms not directly related to the lungs, such as:
- Blurred vision or light sensitivity.
- Eye inflammation, dryness, or pain.
- Fatigue, or tiredness that does not get better with rest.
- Lupus pernio, or skin rashes, lumps, and sores on the face, arms, or shins.
- Night sweats.
- Pain in the joints and bones.
- Papular sarcoidosis, or smooth, painless bumps on the face and near the eyes. This is especially common for people of African-American descent.
- Swollen lymph nodes.
- Weight loss.
If you have symptoms of sarcoidosis, talk to your healthcare provider. They will ask you questions about your health and medical history, your family history, and your symptoms. These can provide clues to determine the underlying cause of your symptoms.
Tests and procedures
Your specialist will perform a physical exam, and may order the following tests:
Blood tests. Your doctor may take a sample of your blood and test its oxygen and carbon dioxide levels to see how well your lungs are functioning. They may also test for liver and kidney function or signs of infection.
Bronchoscopy. A bronchoscopy allows your doctor to look directly into your lungs. A thin, flexible tube with a light and camera at the end (a bronchoscope) will be put into your throat and passed into your lungs. Your doctor can then examine any nodule and take a tissue sample (biopsy) to be examined in a lab. A lung biopsy can be used to diagnose specific infections or lung cancer.
Electrocardiogram (ECG or EKG). This test measures the electrical activity of your heart. It can detect if you have an abnormal heart rhythm or if there is anything off with the electrical activity.
Imaging exams. These tests allow your doctor to see inside your body and look for any abnormalities. Common imaging exams include:
- Chest X-ray. An x-ray uses high-energy beams to create a picture of your chest. It can be used to look for granulomas on the heart and lungs and to rule out other conditions, such as lung cancer.
- CT scan. A CT scanner takes multiple X-ray images to create a cross-sectional view of the body. This can help your doctor see heart and lung damage and granulomas.
Pulmonary function test. This test measures how much air your lungs can hold and how well you can inhale and exhale. Generally, this involves breathing into and out of a machine while following a doctor’s instructions. Based on this information, you and your doctor can better understand your treatment options.
Your doctor may recommend that you get an eye exam or see a dermatologist. You may also need to see a neurologist for special testing if your doctor suspects neurosarcoidosis.
Treatments for sarcoidosis
Your sarcoidosis treatment will depend on what organs are affected and the severity of your symptoms. There is no cure for sarcoidosis and no way to reverse tissue damage. Instead, treatment focuses on managing your symptoms and preventing further damage.
Most cases of sarcoidosis resolve on their own, in a matter of months. If you have few or no symptoms, your doctor might recommend a “wait and see” approach, or “watchful waiting.” During this period you can use over-the-counter medications to manage pain or discomfort, and your doctor will take regular blood samples or X-rays to make sure the disease is not getting worse.
For severe symptoms
If you have severe sarcoidosis symptoms or the function of your organs is impaired, your doctor may recommend medications. Commonly prescribed medications for sarcoidosis include:
- Corticosteroids. These powerful anti-inflammatory drugs can be taken as pills, eye drops, or a cream. Your doctor may prescribe them in high doses at first to stabilize your condition, then lower the dose over time. They are usually very effective, even if sarcoidosis comes back (relapse). However, taking corticosteroids long-term can have negative side effects like osteoporosis, a condition where your bones become brittle. Your doctor may recommend you take a vitamin D supplement or extra calcium to combat these side effects.
- Hydroxychloroquine. This drug is often used to treat sarcoidosis of the skin and lungs. It can also treat hypercalcemia, or having too much calcium in your blood, which is common with sarcoidosis. However, hydroxychloroquine can have negative side effects, especially for your eyes.
- Immunosuppressants. Sarcoidosis is an autoimmune condition where your immune system goes into overdrive. Taking drugs that reduce or suppress your immune system can therefore help alleviate inflammation and prevent further damage. Immunosuppressants might be a good choice for you if you are experiencing negative side effects from corticosteroids.
- Tumor Necrosis Factor-Alpha (TNF-Alpha) Inhibitors. These medications are used to treat some cancers and rheumatoid arthritis. TNF-alpha inhibitors are an option for severe cases of sarcoidosis that have not responded to other treatments. However, they come with serious side effects, so you and your doctor will need to weigh the risks and benefits.
Additional treatments and lifestyle changes
Your doctor might recommend that you seek additional treatments, like physical therapy, as part of your overall care. In physical therapy, you will learn breathing exercises and movements that allow you to increase your strength and flexibility, reduce feelings of fatigue, and improve your overall health.
In addition, you may want to consider the following lifestyle changes to ease sarcoidosis symptoms:
- Eat a balanced diet of whole grains, fruits and vegetables. Try to aim to have half your plate dedicated to fruits and vegetables.
- Lead an active lifestyle. Aim for 30 minutes of exercise at least 3 times a week. Even something as simple as a brisk walk can be good for your health.
- Quit smoking. Smoking can irritate the lung tissue and make sarcoidosis symptoms worse. If you smoke, consider quitting. If you don’t smoke, don’t start.
- Always wear protective gear at work. If you work near fumes, chemicals, or dust, be sure to wear the right protective gear, like eye goggles and a mask or respirator.
Surgery is rarely used to treat sarcoidosis. In cases with significant lung, heart, or liver damage, an organ transplant may be needed.
When sarcoidosis affects multiple organs, it is referred to as systemic sarcoidosis. The organs that can be affected include:
- The brain (neurosarcoidosis). Neurosarcoidosis affects the brain, the spinal cord, or both. It can interfere with your nerve cells’ ability to transmit messages.
- The eyes (ocular sarcoidosis). Ocular sarcoidosis can affect any part of the eye, including the eyelid. It can lead to uveitis, or inflammation of the middle layer of the eye’s wall.
- The heart (cardiac sarcoidosis). Cardiac sarcoidosis is a serious but rare condition where granulomas develop on the heart muscle. This can interfere with the electrical activity that regulates your heartbeat.
- The liver (hepatic sarcoidosis). Hepatic sarcoidosis can reduce the function of your liver and lead to abnormal liver tests.
- The skin. Sarcoidosis of the skin often leads to bumps or skin lesions. They may or may not be painful.
Complications of sarcoidosis
While sarcoidosis usually goes away on its own, chronic sarcoidosis can lead to long-term tissue damage and prevent your organs from working well. Complications of untreated sarcoidosis include:
Cirrhosis, or scarring of the liver. Chronic inflammation of the liver can lead to scarring, which prevents the liver from functioning properly. This can become life-threatening if left untreated.
Heart failure. Granulomas on the heart can disrupt your heart’s electrical activity, which regulates your heartbeat. This can lead to disruption in blood flow and normal heart function that get worse over time.
Nerve damage. Neurosarcoidosis breaks down the myelin sheaths, a protective layer that surrounds your nerve cells and acts like insulation on a wire. Without myelin, the electrical messages that nerve cells send are disrupted, and you may lose control of certain body parts, especially the muscles in the face.
Pulmonary fibrosis, or scarring of the lungs. Chronic pulmonary sarcoidosis can cause permanent damage and scarring to the lungs. When the lungs scar, the tissue becomes less elastic, meaning you cannot inhale effectively and get enough oxygen. Pulmonary fibrosis also increases your risk for pulmonary hypertension, or high blood pressure in the blood vessels leading to your lungs. Pulmonary hypertension can strain your heart muscle over time.
Uveitis and vision loss. Uveitis is a common complication of ocular sarcoidosis. It is an inflammation of the middle layer of the eye’s wall. This can contribute to cataracts, or clouding of your eye’s lens; glaucoma, excessive eye pressure; and damage to the retina. All of these conditions can cause you to lose your eyesight.