Sarcoidosis

Overview

Sarcoidosis is a complex and rare inflammatory disease. In those who have it, tiny clumps of inflammatory cells called granulomas may form in potentially any organ, including the lungs and heart. Despite increasing advancements in research, sarcoidosis remains difficult to diagnose and treat, and it has no known cure.

Usually, your immune system creates inflammation to help fend off illness. With sarcoidosis, inflammation (that is not caused by an infection) causes clusters of cells, or granulomas. This typically occurs in the lungs. There, permanent scarring can develop, and as time goes on, this scarring can make it harder to breathe. However, sarcoidosis can affect any organ. Fibrosis in the lungs or other organs can lead to life-threatening heart or lung problems. Many people recover with few or no long-term problems.

Related terms: Boeck’s Sarcoid, cardiac sarcoidosis, systemic sarcoidosis

Signs and Symptoms

Many people who have sarcoidosis have no symptoms, or they may feel unwell but without any obvious symptoms. Others may be depressed, feel very tired, or have a general feeling of discomfort. You may also faint or have unexplained weight loss.

Lofgren’s Syndrome

Lofgren’s syndrome is a classic set of symptoms of sarcoidosis. It includes:

• Swollen lymph nodes in your chest, neck, chin, armpits, or groin
• A rash of small painful bumps called erythema nodosum that most commonly appear on your 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 two years.

Lungs

Symptoms of sarcoidosis in the lungs may include:

• Wheezing
• Coughing
• Shortness of breath
• Chest pain

However, you can have sarcoidosis in the lungs without these symptoms. Skin rashes or sores can include erythema nodosum 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 American people more often than other groups.

Heart

Potential symptoms of cardiac sarcoidosis include:

• Irregular heartbeats, which can include palpitations or skipped beats
• Lightheadedness or fainting
• Shortness of breath, coughing, or chest tightness

Other Symptoms

Other symptoms depend on which organs are affected, and may include:

• Larger than normal 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 parotid or salivary glands

Related Health Effects

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

• Heavy bleeding after delivery
• Preterm delivery
• Preeclampsia, which is high blood pressure and too much protein in the urine
• Venous thromboembolism which is blood clots 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. Also, tell your doctor about any medicines you take, as some sarcoidosis medicines are not safe to take during pregnancy.

Causes and Risk Factors

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 clusters, called granulomas, which may lead to permanent scarring of organs.

Immune system triggers can lead to sarcoidosis in certain people. Triggers can include infections or substances in the environment. Genes can affect how the immune system reacts to a trigger.

There are many risk factors for sarcoidosis. Some risk factors, such as where you work, 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 as you get older, especially after age 55.
• Environment: Living or working near insecticides, mold, or other substances that may cause inflammation raises your risk. You may be around these substances if you are in health care or the automotive industry or are a farmer or firefighter.
• Family history and genetics: Having a close relative with sarcoidosis raises your risk.
• Medicines: Certain types of HIV medicines and monoclonal antibodies used to treat cancer, or an overactive immune system can raise your risk.
• 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.

Other medical conditions, such as lymphoma, a type of blood cancer, are also associated with sarcoidosis.

Prevention Guidance

If you are at risk for sarcoidosis, your provider may talk to you about trying to avoid certain substances in your environment that can trigger granulomas. Some risk factors, such as where you work, can be changed.

A doctor may recommend lifestyle changes, such as eating healthier, exercise, or quitting smoking. It is important to have a healthcare provider, who can keep track of the condition and adjust treatment as needed.

Diagnosis

Sarcoidosis is diagnosed based on your symptoms, a physical exam, imaging tests, and sometimes a biopsy. Before diagnosing you with sarcoidosis, your healthcare provider will rule out other possible conditions. Sarcoidosis can be hard to diagnose because symptoms can vary from person to person, and doctors must run many tests to rule out other conditions.

Doctors use stages to describe sarcoidosis of the lung or lymph nodes of the chest. The stages are based on where the granulomas are found and whether there is scarring. Stage IV is the most serious and indicates permanent scarring in the lungs.

During the physical exam, your provider may:

• Check your temperature.
• Check 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 on the scalp and lower legs.

There are no screening tests to determine who will develop sarcoidosis. If you are at risk for sarcoidosis, your provider may talk to you about trying to avoid certain substances in your environment that can trigger granulomas.

Your healthcare provider may have you undergo certain tests and procedures to diagnose sarcoidosis:

• Chest X-rays look for granulomas or scarring in the lungs and heart and help determine the stage of the disease. Often, sarcoidosis is found because a chest X-ray is done for another reason.
• A biopsy of the skin, lymph nodes, lungs, or other affected organs may help confirm your sarcoidosis diagnosis. Your doctor may do a bronchoscopy to get a sample from your lungs or lymph nodes in your chest.
• Blood tests check your blood counts, hormone levels, and how well your kidneys are working.
• Other imaging tests look for granulomas or inflammation in the heart, eyes, lymph nodes, or other areas and may include magnetic resonance imaging (MRI) or an ultrasound.
• Neurological tests, such as electromyography, evoked potentials, spinal taps, or nerve conduction tests, look for problems with the nervous system caused by sarcoidosis.
• Eye exams look for eye damage, which can occur without symptoms in a person with sarcoidosis.
• Lung function tests check on the breathing.
• Heart tests monitor how well your heart is working. Sarcoidosis rarely affects the heart, but cardiac sarcoidosis may be life threatening. Tests may include electrocardiography (ECG or EKG), echocardiography, or cardiac MRI.

People often spend years in search of a cardiac sarcoidosis diagnosis. Inaccurate diagnoses can mean lost time and lost organ function, which cannot be regained.

Treatment and Management

The goal of treatment for sarcoidosis is remission, which means the condition still exists but does not cause problems. Not everyone who is diagnosed with sarcoidosis needs treatment. Sometimes the condition goes away on its own. Your treatment will depend on your symptoms, which organs are affected, and whether those organs are working well.

There is no cure for sarcoidosis. For some people, the condition will go away without treatment. For others, treatment can help decrease the severity of symptoms.

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 corticosteroids may include high blood sugar, high blood pressure, mood changes, weight gain, and increased appetite. The pill also raises the risk of cataracts, glaucoma, or osteoporosis. Common side effects from inhaled corticosteroids include a hoarse voice or a mouth infection called thrush.

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

Living With Sarcoidosis

If you have sarcoidosis, you should get ongoing care regardless of having symptoms. Your provider will monitor you for side effects from long-term use of corticosteroids or other medicines.

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

If your sarcoidosis goes into remission, your doctor may carefully stop your medicines. However, you will still need to watch for a flare. If you do have a flare, 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.

Some people have sarcoidosis that persists or comes back for many years after diagnosis, which may be called chronic, severe, advanced, refractory, or progressive sarcoidosis. Your provider may order tests to keep track of your condition and check for complications.

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

• Get regular physical activity. Extreme tiredness can make it hard to exercise if you have sarcoidosis. However, physical activity can 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.
• Quit smoking. If you smoke, quit. Also, try to avoid other lung irritants, such as dust, chemicals, vaping, and secondhand smoke.
• Try to get enough good quality sleep. Experts recommend that adults get 7 to 9 hours of sleep per day.

Sarcoidosis and Mental Health

Sarcoidosis may make you feel lonely, anxious, or depressed. You may continue to feel very tired even after your treatment has ended. Certain activities or treatments may help improve your mental health and quality of life:

• Counseling, particularly cognitive therapy
• Joining a patient support group may help you adjust to living with sarcoidosis
• Talking to your doctor about medicines or other treatments such as antidepressants or other treatments
• Support from family and friends

Find Clinical Trials

Clinical trials uncover better ways to prevent, diagnose, treat, and understand diseases and conditions. To ensure results apply to everyone, volunteers of all ages, sexes, and backgrounds, including both healthy individuals and those with specific medical conditions, are needed. Find clinical trials on sarcoidosis.

Find Treatment

People often spend years in search of a cardiac sarcoidosis diagnosis. The Foundation for Sarcoidosis Research is dedicated to working with patients, clinicians, researchers, and the community to help patients connect with sarcoidosis specialists to improve their chance for effective treatment and better patient outcomes.

Research Information

The National Institutes of Health (NIH) is the largest public funder of biomedical research in the world. NIH invests most of its budget in medical research seeking to enhance life and to reduce illness and disability. NIH-funded research has led to breakthroughs and new treatments helping people live longer, healthier lives, and building the research foundation that drives discovery.

NIH supports research on the causes and treatment of inflammatory diseases.

Find NIH-funded research projects using NIH RePORTER, a searchable database of current and past research projects supported by NIH and other federal agencies.