What Is Sarcoidosis?
Sarcoidosis is an inflammatory disease that can affect multiple organs in the body, but most often involves the lungs and lymph glands in the chest. If you have sarcoidosis, red, swollen lumps called granulomas form in your affected organs. Symptoms, which can range from non-existent to severe, depend partly on where the lumps form.
Is sarcoidosis an autoimmune disease?
Sarcoidosis does involve immune system activity, but it's not generally considered an autoimmune disease. Autoimmunity means your immune system attacks parts of your body. However, researchers are continuing to study the idea that at least some cases of sarcoidosis involve autoimmunity.
Is sarcoidosis cancer?
Sarcoidosis is not cancer. In rare cases, doctors might mistake cancer for sarcoidosis, delaying proper diagnosis of the cancer.
The symptoms of sarcoidosis can vary greatly, depending in part on which organs are involved. In some people, symptoms may begin suddenly and and then stop. Others may have no outward symptoms. Still others may have symptoms that appear slowly and subtly, but which last or come back repeatedly over a long time span.
General symptoms include:
- Swollen, painful joints
- Achy or weak muscles
- Swollen lymph nodes
- Unexplained weight loss
- Kidney stones
- Night sweats
Lung sarcoidosis symptoms
The lungs are usually the first area to be affected by sarcoidosis: 9 of 10 people with sarcoidosis have some type of lung (pulmonary) involvement. Pulmonary sarcoidosis can be serious, leading to scar tissue (fibrosis) in the lungs. This complication can interfere with breathing.
If you have lung sarcoidosis, your symptoms might include:
- Shortness of breath
- Chest pain
Skin sarcoidosis symptoms
Sarcoidosis can cause changes on your skin, nails, and hair. Common symptoms include:
- Small bumps called papules on your neck or face, especially around your eyes. These are especially common in Black women and may be reddish brown, violet, tan, brown, or the same color as your skin.
- Raised patches that can be smooth or scaly. These may be reddish brown, purple, or brown. When patches form and heal on your scalp, they can cause hair loss.
- Firm bumps and patches. On darker skin, these patches and bumps may look darker or lighter than your skin tone. On lighter skin, they might look pink or red.
- Painless or slightly tender lumps under your skin, usually on your arms.
- A rash, often on your shins, sometimes along with a fever
- Changes around a tattoo, scar, or body piercing. You might see tiny bumps and the skin might feel lumpy, sore, or itchy.
- Discolored or deformed nails
Cardiac sarcoidosis symptoms
When granulomas grow in your heart tissue, they can affect how your heart works. This could lead to:
- Chest pain
- Shortness of breath
- Fluttering or irregular heartbeats
- Swelling in your legs from excess fluid
- Heart failure
Eye sarcoidosis symptoms
You might have signs of sarcoidosis in your eyes that will only show up on an eye exam. If you do have symptoms, they might include:
- Blurred vision
- Eye pain
- Dry eyes
- Burning or itching eyes
- Severe redness
- Light sensitivity
If sarcoidosis affects your nervous system, it can cause a wide range of symptoms including loss of hearing, taste, and smell, dizziness, seizures, and mental health changes.
Sarcoidosis can also affect the liver, your bones, or any other organ.
Sarcoidosis Risk Factors
Sarcoidosis is a rare disease, affecting fewer than 200,000 people in the United States. It can occur in people of any age, race, or gender, but is more common in some groups than others. Risk factors include:
Age: Sarcoidosis most often shows up between ages 25 and 40.
Race: In the United States, sarcoidosis is most common among people of African descent. People of Scandinavian descent also have an increased risk.
Gender: Sarcoidosis is slightly more common among those assigned female at birth than those assigned male.
Environment: If you live or work around insecticides, molds, dust, or other substances that might trigger inflammation, you may face a higher risk. Farmers, firefighters, and autoworkers are in this group. Military veterans exposed to burn pits are too.
Family history: If a close relative has sarcoidosis, your risk is higher.
Medications: Risks are higher if you take certain drugs, including some used for HIV and cancer.
The exact cause of sarcoidosis is not known. Studies suggest that in some people, the inflammation that leads to symptoms is triggered by an infection or by exposure to something else in the environment, such as chemicals or dust. Sarcoidosis might also involve autoimmune responses, in which your immune system attacks your body's own proteins.
Is sarcoidosis hereditary?
Since your risk is higher if you have a close family member with the disease, scientists suspect that genes play a role. Genetic differences might explain why infections and other exposures trigger sarcoidosis in some people but not others. But scientists haven't discovered the gene or genes involved.
Sarcoidosis can be hard to diagnose, partly because you might have no clear symptoms or your symptoms and test results may mimic those seen in other conditions. To start the process, your doctor will carefully review your medical history and examine you. The main tools your doctor will use to diagnose sarcoidosis include:
Chest X-rays to look for granulomas and scarring in your lungs and heart. (Some people are diagnosed with sarcoidosis after a chest X-ray is done for some other reason). The X-ray can also help the doctor see how advanced any lung involvement is -- a process called staging.
Other imaging tests, such as an MRI or ultrasound. These can look for granulomas elsewhere in your body.
Biopsies, which are tests in which small tissues samples are taken to check for signs of disease. You might get biopsies of your skin, lymph nodes, lungs, or other organs. If you need a lung or lymph node biopsy, your doctor may do a bronchoscopy, in which a lighted tube is passed down your throat to reach the target areas.
Additional tests might be done to confirm the diagnosis or see how sarcoidosis is affecting your body. These could include:
- Lab tests of your blood and urine to show how your kidneys, liver, and other organs are working
- Lung function tests to see if you have any breathing problems
- Eye exams to look for signs of damage, even if you aren't having any eye symptoms
- Heart tests, including an electrocardiogram (EKG or ECG) to check the electrical activity in your heart
- Neurological tests to see if your nervous system is affected
- Imaging tests, such as a PET scan, that can show inflammation in your body
- Tuberculosis skin test to rule out TB, which is sometimes mistaken for sarcoidosis
There is no cure for sarcoidosis, but the disease often gets better on its own over time. Many people with sarcoidosis have mild symptoms and don't need treatment. Treatment, when it is needed, can reduce symptoms and prevent organ damage.
Your doctor may prescribe medications including:
Corticosteroids. These drugs, like prednisone and cortisone, reduce inflammation. You might get them as pills, inhaled sprays, or creams, depending on which parts of your body are involved. These are the most common treatments for sarcoidosis. Side effects of steroid pills can include mood changes, weight gain, increased blood pressure, and increased blood sugar.
Immunosuppressants. These drugs tamp down your immune system, reducing inflammation and organ damage. Methotrexate is an immunosuppressant drug often used for sarcoidosis. You take these drugs as pills or shots. Side effects can include liver damage or blood problems.
Anti-tumor necrosis factor-alpha antibodies (anti-TNF-alpha antibodies). These drugs target a specific immune system chemical. You get them through an intravenous (IV) treatment, meaning the drugs go through a needle into a vein. Side effects can include an increased risk of serious infection.
Anti-malarial medications. Drugs commonly used to treat malaria, such as hydroxychloroquine and chloroquine, also can reduce inflammation and are sometimes prescribed for sarcoidosis. You take them in pill form. Side effects can include serious skin rashes and vision changes.
In some cases, your doctor might recommend you take nonsteroidal anti-inflammatory drugs (NSAIDS) that you can buy without a prescription. These are drugs like aspirin, ibuprofen, and naproxen that can temporarily relieve symptoms like joint pain and fever. Long-term use can lead to heartburn, vomiting, and stomach bleeding, so you should always talk to your doctor before you start any regular NSAID use.
Important to know: Some drugs prescribed for sarcoidosis aren't approved by the FDA for that use, so patients sometimes have trouble getting insurers to pay for them. Studies to expand the number of drugs rigorously tested and approved for sarcoidosis are underway. You can ask your doctor if participation in a clinical trial might be right for you.
Other sarcoidosis treatments
In addition to medication, some people will benefit from treatments including:
- Physical therapy to reduce fatigue and improve strength
- Pulmonary rehabilitation to improve breathing
- Implantation of a cardiac pacemaker or defibrillator for heart rhythm problems
- Organ transplant if sarcoidosis has severely damaged your heart, lungs, or liver
Your doctor may tell you that your disease is at a certain stage, between 0 and 4. These stages are based on how your lungs look on a chest X-ray.
You can have serious symptoms at any stage, but doctors generally expect people at lower stages to have a higher chance of recovering completely, even without treatment. You can move between stages or not.
These are the stages:
Stage 0: No signs of sarcoidosis in your lungs or the lymph nodes in the center of your chest (though you may have signs elsewhere).
Stage I: Granulomas in the lymph nodes only. You may have no symptoms or may have fevers, joint pains, and night sweats that developed suddenly. This combination of symptoms and lymph node involvement is called Lofgren syndrome and is the type of sarcoidosis most likely to go away on its own, usually within 6 months to 2 years.
Stage II: Granulomas in the lungs and the lymph nodes.
Stage III: Granulomas in the lung tissue only.
Stage IV: Permanent scarring of lung tissue. This scarring can't be reversed.
In addition to looking at the stage of any lung involvement, your doctor will consider which other organs might be involved to help you plan your treatment.
For most people, sarcoidosis is a temporary condition that goes away, with or without treatment, and without permanent damage. About two-thirds will recover within 2 to 3 years; those with Lofgren syndrome tend to get better even sooner.
If you're still having symptoms after 3 years, you are more likely to keep having symptoms. About 10% to 20% of people with long-lasting sarcoidosis develop permanent organ damage, most often in the lungs.
Long-term complications can develop in several part of the body:
Lungs: If you have permanent scarring in your lungs, it can get harder to breathe. You might develop a condition called pulmonary hypertension, a type of high blood pressure affecting the blood vessels in your lungs. It can make your heart work harder than normal to get blood into your lungs.
Eyes: Inflammation may damage your retinas, which might eventually lead to blindness. In rare cases, sarcoidosis leads to glaucoma or cataracts.
Kidneys: Sarcoidosis can change the way your body handles calcium, potentially leading to kidney stones and poor kidney function.
Heart: If you have granulomas in your heart, you can have problems with heart rhythm, blood flow, and normal heart functioning.
Nervous system: When sarcoidosis affects the brain and nerves, it can lead to symptoms that mimic multiple sclerosis, such as weakness and numbness. It can also cause drooping facial muscles and symptoms ranging from seizures to hearing problems.
Can sarcoidosis be fatal?
Most people with sarcoidosis will live as long as people without the condition. Death, from heart, lung, or other problems, happens in 1% to 5% of cases.
Living With Sarcoidosis
When you have sarcoidosis, it's important to follow recommendations from your doctor and to let the doctor know if you have any new or changing symptoms.
Even if you're not having bothersome or new symptoms, keep follow-up appointments so your doctor can monitor your health, see if medications are working, and spot any complications.
You also can feel better and limit complications and treatment side effects by making some lifestyle changes in these areas:
Healthy eating. Aim for a nutritious diet, with lean meats and other proteins, fruits, vegetables, whole grains, and not too much sugar. Try to maintain a healthy weight.
Avoiding alcohol. Some medications used for sarcoidosis can be hard on your liver. Alcohol can increase the risk of liver damage.
Not smoking. Smoking can make your lung symptoms worse. Reach out to a smoking cessation program if you want to quit and need help.
Being physically active. Staying active can improve your mood, strengthen your muscles, and reduce fatigue.
Managing stress. Stress-reduction techniques like meditation can help some people with chronic illness.
Getting enough sleep. Improving your sleep can lower inflammation and improve well-being. If you are struggling to sleep, talk to your doctor.
Seeking support. If you're struggling to cope, consider meeting with a counselor or joining a support group.