Sarcoidosis

Your immune system creates inflammation to help defend you against germs and sickness. But in sarcoidosis, inflammation goes off track and the cells in your immune system form lumps, called granulomas, in your body. Over time, inflammation may lead to permanent scarring of organs.

Studies suggest that triggers to your immune system, including infections without symptoms or coming into contact with substances in the environment, can lead to sarcoidosis in certain people. Some of these people have genes that put them at risk for the disease.

There are many risk factors for sarcoidosis. Some risk factors, such as your job, can be changed. But your age, family history and many other risk factors cannot be changed.

• Age. You can get sarcoidosis at any age but the risk goes up with age, especially after age 55.
• Living or working near insecticides, mold, or other substances that may cause inflammation. You may also be around these substances as a healthcare, farming, automotive industry worker, or firefighter
• Family history and genetics, such as having a close relative with sarcoidosis
• Medicines, such as certain types of HIV medicines and monoclonal antibodies used to treat cancer or overactive immune system
• Other medical conditions, such as lymphoma, a type of blood cancer
• Race or ethnicity. Your risk is higher if you are of African or Scandinavian descent.
• Sex. Women are more likely to have sarcoidosis, although men can also have it.

Talk to your doctor about your risk factors.

There are no screening methods to determine who will develop sarcoidosis. If you are at risk for sarcoidosis, your doctor may talk to you about trying to avoid insecticides, mold, or other substances that can trigger granulomas.

Many people who have sarcoidosis have no symptoms or generally feel unwell. Symptoms will also depend on what organ is affected. Sarcoidosis most often affects the lungs and the lymph nodes in the chest. If inflammation continues, some people may have permanent scarring (fibrosis) that can cause life-threatening heart or lung problems.

Signs and symptoms

Many people have general symptoms, such as feeling depressed, very tired, and a general feeling of discomfort. You may also faint or have unexplained weight loss.

Lofgren’s syndrome is a classic set of signs and symptoms of sarcoidosis:

• Swollen lymph nodes in your chest, neck, chin, armpits, or groin
• A rash of small, itchy or painful bumps called erythema nodosum. They most commonly appear on your head, neck, or legs.
• Blurred vision, eye pain or redness, light sensitivity, or watery eyes
• Joint pain, stiffness, or swelling
• Fever

Some people have Lofgren’s syndrome when they first develop sarcoidosis. This is most common in women between ages 30 and 40. It usually goes away completely within 2 years.

Sarcoidosis in the lungs may cause you to:

• Wheeze
• Cough
• Feel short of breath
• Have chest pain

However, you can have sarcoidosis in the lungs without these symptoms. Other symptoms based on the affected organ include:

• Skin rashes or sores, such as bumps called erythema nodosum (explained above) or lupus pernio. Lupus pernio causes skin sores that usually affect the face, especially the nose, cheeks, lips, and ears. These sores usually last a long time. Lupus pernio affects African Americans more often than other groups.
• Enlarged liver or spleen or jaundice, which can make your eyes or skin yellow
• Nervous system problems, such as headache, dizziness, vision problems, seizures, mood swings, hallucinations, delusions, or nerve pain
• Heart palpitations or an irregular heartbeat
• Abdominal pain, nausea, or vomiting
• Muscle pain or soreness
• Swollen salivary glands

Complications

If untreated, or if the treatment does not work, sarcoidosis can cause serious complications, such as:

• Lung problems, including pulmonary fibrosis (permanent lung scarring) or pulmonary hypertension
• Blindness
• Blood problems, such as anemia or low white blood cell counts
• Hormone problems, such as too much calcium in the blood or urine, diabetes insipidus, or not having a menstrual period
• Heart problems, including an irregular heartbeat, cardiomyopathy, heart failure, or sudden cardiac arrest
• Kidney problems, such as kidney stones or kidney failure
• Liver disease (cirrhosis)
• Problems with the nervous system, including brain inflammation or tumors, buildup of pressure in the brain, psychiatric problems, or nerve pain

Sarcoidosis is diagnosed based on your symptoms, a physical exam and imaging tests or a biopsy. Before diagnosing you with sarcoidosis, your doctor will rule out other possible conditions.

Stages of sarcoidosis

Doctors use stages to describe sarcoidosis of the lung or lymph nodes of the chest. The stages are based on where the granulomas are located and whether there is scarring on imaging tests. Stage IV is the most severe and means you have permanent scarring in the lungs.

Medical history and physical exam

Bring a list of symptoms with you to your appointment. Tell your doctor if you live or work near insecticides or mold or have other risk factors for sarcoidosis. During the physical exam, your doctor may:

• Check your temperature
• Feel different parts of your body to see if your lymph nodes, spleen, or liver are swollen
• Listen to your chest with a stethoscope as you breathe in and out
• Look for rashes or sores on your body, such as scalp and lower legs

Diagnostic tests and procedures

To diagnose sarcoidosis, your doctor may have you undergo some of the following tests and procedures:

• Chest X-rays to look for granulomas or scarring in the lungs and heart. This will also help determine the stage of the disease. Often, sarcoidosis is found because a chest X-ray is performed for another reason.
• biopsy of the skin, lymph nodes, lungs, or other affected organs to diagnose granulomas. This is often needed to confirm your sarcoidosis diagnosis. A bronchoscopy is the procedure used to get the biopsy sample from your lungs or lymph nodes in your chest.
• Blood tests to check your blood counts, hormone levels, and kidney function
• Other imaging tests to look for granulomas or inflammation in the heart, eyes, lymph nodes, or other areas. This may include magnetic resonance imaging (MRI), an ultrasound, a high-resolution computed tomography (CT) scan, a positron electron tomography (PET) scan, or a gallium scan. Many of these tests may use a radioactive material, such as gallium.

Tests to look at how sarcoidosis is affecting the body

• Neurological tests, such as electromyography, evoked potentials, spinal taps, or nerve conduction tests, to find problems with the nervous system caused by sarcoidosis
• Eye exam to look for eye damage, which can occur without symptoms in a person with sarcoidosis
• Pulmonary function tests to check whether you have breathing problems
• Heart tests to monitor how well your heart is working. Sarcoidosis only rarely affects the heart, but cardiac sarcoidosis may be life threatening. Tests may include electrocardiography (ECG or EKG), echocardiography, or cardiac MRI

The goal of treatment is remission, which means the condition is not causing problems. Not everyone who is diagnosed with sarcoidosis needs treatment. Sometimes the condition goes away on its own. Your treatment will depend on your signs and symptoms, which organs are affected, and whether those organs are working well. Some people do not respond to treatment.

Medicine

Your doctor may prescribe medicines to lower inflammation or treat an overactive immune response. Immune-lowering medicines raise the risk of infections. Talk with your doctor about the benefits and risks of these medicines.

Corticosteroids (steroid hormone medicine) to treat inflammation. The corticosteroid prednisone is the most common treatment for sarcoidosis. Corticosteroids can be taken as pills or be injected, inhaled, or taken as eye drops or other topical medicines.

Corticosteroids can have serious side effects with long-term use, especially if taken in high doses. Side effects of the corticosteroid pill may include high blood sugar or blood pressure, mood changes, weight gain, and increased appetite. The pill also raises the risk of cataracts (clouding of the eye), glaucoma (damage to a nerve in the eye from high pressure), or osteoporosis (bone thinning). Common side effects from inhaled corticosteroids include a hoarse voice or a mouth infection called thrush.

Other medicines to lower your immune system response

• Medicines used to treat severe rheumatoid arthritis, such as methotrexate, azathioprine, and leflunomide. They are typically taken as a pill but may be injected. Side effects may include liver damage or blood problems.
• Monoclonal antibodies (also called immunotherapy) used to treat cancer or overactive immune system. These include rituximab, infliximab, golimumab, and adalimumab. They may be injected under the skin or into the blood through an IV. Although rare, they may cause a life-threatening immune reaction. Other possible side effects include heart problems, low blood counts, or a higher risk of certain cancers. But these depend on the medicine.
• Medicines used to treat malaria, such as hydroxychloroquine or chloroquine. Side effects may include eye damage, heart problems, and low blood sugar.
• Corticotropin (a hormone medicine) injection in the muscle or under the skin. This may be used when prednisone does not work or has severe side effects. Side effects may include high blood pressure, problems controlling blood sugar, increased appetite, or mood changes.
• Pentoxifylline, which you take as a pill. It is normally prescribed to improve blood flow. Side effects may include nausea.

Other medicines

• Antibiotics to treat sarcoidosis of the skin. Examples include minocycline, tetracycline, and doxycycline. Side effects may include dizziness and gastrointestinal (GI) problems.
• Colchicine to treat joint pain from sarcoidosis. This medicine is taken as a pill and is usually prescribed for gout. Side effects include nausea, vomiting, diarrhea, and stomach cramps or pain.

If you have been diagnosed with sarcoidosis, it is important that you continue your medicine, follow up with your doctor when directed, and make healthy lifestyle changes. Also get support and take care of your mental health.

Some people may have life-long remission (where you do not have symptoms). Others may go into remission for a while and then have a flare, or return, of the disease. Read on below if you are pregnant or planning to become pregnant.

Manage your condition

Even if you do not have symptoms, you should see your doctor for ongoing care. For example, your doctor will monitor you for side effects from long-term use of corticosteroids or other medicines. 

If the disease is not worsening, your doctor may watch you closely to see whether the disease goes away on its own. If the disease does start to get worse, your doctor can prescribe treatment.

Remission and flares

If your sarcoidosis goes into remission, your doctor may carefully stop your medicines. However, you will still need to watch for a flare. See your doctor if this happens. You may need another round of treatment.

Flares can be hard to predict. 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.

Tests for complications

Some people have sarcoidosis that persists or recurs for many years after diagnosis. This may be called chronic, severe, advanced, refractory, or progressive sarcoidosis. Your doctor may order tests to keep track of your condition and check for complications. Visit the Diagnosis section to learn about these tests.

Know when to seek medical care

Watch for the warning signs of complications that may require emergency medical treatment. These include signs of changes in vision that may be a sign of or brain tumors. Other complications that require immediate medical attention include kidney failure, sudden cardiac arrest, and sudden shortness of breath or muscle weakness.

Make healthy lifestyle changes

A healthy lifestyle may help you feel better and prevent sarcoidosis from getting worse.

• Choose healthy foods. Eating more fruits and vegetables can provide important health benefits.
• Aim for a healthy weight
• Get regular physically activity. Fatigue can make it hard to exercise if you have sarcoidosis. However, physical activity can actually improve energy and help with other symptoms, such as shortness of breath and muscle weakness. Try to stay active but talk with your doctor about what level of physical activity is right for you.
• Manage stress
• Quit smoking. If you smoke, quit. Also, try to avoid other lung irritants, such as dust, chemicals, and secondhand smoke. Visit Smoking and Your Heart and the National Heart, Lung, and Blood Institute's Your Guide to a Healthy Heart. For free help quitting smoking, you may call the National Cancer Institute's Smoking Quitline at 1-877-44U-QUIT (1-877-448-7848).
• Try to get enough good quality sleep. Experts recommend that adults get 7 to 9 hours of sleep per day.

Learn more about these healthy lifestyle changes in our Heart-Healthy Living Health Topic.

Take care of your mental health

Sarcoidosis may make you feel lonely, anxious, or depressed. You may continue to feel fatigued even after your treatment has ended. But certain activities or treatments may help improve your mental health.

• Counseling, particularly cognitive therapy, can be helpful.
• Joining a patient support group may help you adjust to living with sarcoidosis. You can see how other people manage similar symptoms and their condition. Talk with your doctor about local support groups or check with an area medical center.
• Medicines or other treatments. Your doctor may talk to you about medicines, such as antidepressants, or other treatments that can improve your quality of life.
• Support from family and friends can help relieve stress and anxiety. Let your loved ones know how you feel and what they can do to help you.

Treating complications

If you have complications, your doctor may recommend the following medicines or procedures. Ask your doctor about the benefits and risks of any treatment.

Lung problems:

• Inhaled corticosteroids or oxygen therapy to help with breathing problems
• Vasodilator treatment to relax your blood vessels, if you have pulmonary hypertension
• Pulmonary rehabilitation to help you breathe easier and be more active

Heart problems:

• A device called an implantable cardioverter defibrillator that can help prevent sudden cardiac arrest
• High blood pressure medicines, such as ACE inhibitors and beta blockers

Hormone problems:

• If you have too much calcium in the blood or urine, you may need to avoid sunlight, drink plenty of fluids, and eat fewer foods with calcium.
• Hormone replacement treatment for some types

Brain, nerve, or muscle problems:

• Anti-seizure medicines if sarcoidosis affects your brain and causes seizures
• Medicines for nerve or muscle pain
• Physical therapy to improve muscle strength
• Surgery to remove brain tumors

Severe organ damage. Transplant surgery may be needed if your sarcoidosis causes life-threatening lung, heart, or liver damage.

Pregnancy and sarcoidosis

Most women who have sarcoidosis give birth to healthy babies. However, there is a higher risk of certain complications, including:

• Heavy bleeding after giving birth
• Preterm delivery
• Preeclampsia, which is high blood pressure and too much protein in the urine
• Venous thromboembolism, which is a blood clot in the lungs or deep veins, usually in the legs

If you have sarcoidosis and are pregnant or planning to become pregnant, talk with your doctor. It is important to get good prenatal care and regular sarcoidosis checkups before, during, and after pregnancy. Talk to your doctor about any medicines you take. Some sarcoidosis medicines are not safe to take during pregnancy.