Sarcoidosis is a disease that leads to inflammation, usually in your lungs, skin, or lymph nodes. It starts as tiny, grain-like lumps, called granulomas. Sarcoidosis can affect any organ in your body.

No one is sure what causes sarcoidosis. It affects men and women of all ages and races. It occurs mostly in people ages 20 to 50, African Americans, especially women, and people of Northern European origin.

Many people have no symptoms. If you have symptoms, they may include

• Cough
• Shortness of breath
• Weight loss
• Night sweats
• Fatigue

Tests to diagnose sarcoidosis include chest x-rays, lung function tests, and a biopsy. Not everyone who has the disease needs treatment. If you do, prednisone, a type of steroid, is the main treatment.

Sarcoidosis is a condition that develops when groups of cells in your immune system form red and swollen (inflamed) lumps, called granulomas, in various organs in the body. The inflammation that leads to these granulomas can be caused either by infections or by certain things you come into contact with in your environment.

Sarcoidosis can affect any organ. Most often it affects the lungs and lymph nodes in the chest. You may feel extremely tired or have a fever. You may also have other symptoms depending on what organ is affected. Your doctor will diagnose sarcoidosis in part by ruling out other diseases that have similar symptoms.

Not everyone needs treatment. Treatment will depend on your symptoms and which organs are affected. Medicines can help treat the inflammation or lower your body’s immune response. Many people recover with few or no long-term problems. Sometimes the disease causes permanent scarring (fibrosis) in the lungs or other organs and can lead to life-threatening heart or lung problems.

Neurosarcoidosis is the occurrence of sarcoidosis in the nervous system. Sarcoidosis is a chronic inflammatory disorder that primarily affects the lungs, but can also impact almost every other organ and system in the body. It typically occurs in adults between 20 and 40 years of age.

Neurosarcoidosis is characterized by inflammation and abnormal cell deposits in any part of the nervous system—the brain, spinal cord, or peripheral nerves. It most commonly occurs in the cranial and facial nerves, the hypothalamus (a specific area of the brain), and the pituitary gland. It is estimated to develop in five to 15 percent of those individuals who have sarcoidosis.

Weakness of the facial muscles on one side of the face (Bell's palsy) is a common symptom of neurosarcoidosis. The optic and auditory nerves can also become involved, causing vision and hearing impairments. Neurosarcoidosis can cause:

• Headache
• Seizures
• Memory loss
• Hallucinations
• Irritability
• Agitation
• Changes in mood and behavior

Neurosarcoidosis can appear in an acute, explosive fashion or start as a slow, chronic illness. Because neurosarcoidosis manifests in many different ways, a diagnosis may be difficult and delayed.

There is no standard of treatment for neurosarcoidosis. Doctors generally recommend corticosteroid therapy as first-line therapy for individuals with the condition.  Additional treatment with immunomodulatory drugs has benefited some individuals. While the use of corticosteroids and immunosuppressive drugs is effective, these medications also have undesirable side effects.

The prognosis for people with neurosarcoidosis varies. Many with the condition will recover completely; others will have a chronically progressing or on-and-off course of illness.

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 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, can also lead to 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 some immune system triggers can lead to sarcoidosis in certain people. Triggers can include infections without symptoms or coming into contact with substances in the environment. Your gene can affect how your immune system reacts to a trigger.

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 that includes:

• Swollen lymph nodes in your chest, neck, chin, armpits, or groin
• A rash of small, itchy, or painful bumps called erythema nodosum that 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

If you have sarcoidosis in the lungs, you may experience these symptoms:

• Wheezing
• Coughing
• Shortness of breath
• Chest pain

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

Other symptoms

You may have other symptoms based on which organs are affected:

• 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 salivary glands

Sarcoidosis is diagnosed based on your symptoms, a physical exam, and imaging tests or a biopsy. Before diagnosing you with sarcoidosis, your healthcare provider 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 lumps, or granulomas, are found and whether there is scarring on imaging tests. Stage IV is the most serious 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 provider if you live or work near insecticides or mold or have other risk factors for sarcoidosis. 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 scalp and lower legs.

Diagnostic tests and procedures

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. This will also help figure out the stage of the disease. Often, sarcoidosis is found because a chest X-ray is done for another reason.
• biopsy of the skin, lymph nodes, lungs, or other affected organs may help confirm your sarcoidosis diagnosis. Your doctor will do a bronchoscopy to get the biopsy 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. These may include magnetic resonance imaging (MRI) or an ultrasound.

The tests below look at how sarcoidosis is affecting the body.

• 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 whether you have breathing problems.
• Heart tests 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 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.

Medicine

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.

Corticosteroids to treat inflammation

The corticosteroid (steroid hormone medicine) 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.

Medicines to lower your immune system response

• Medicines used to treat severe rheumatoid arthritis include methotrexate, azathioprine, and leflunomide. You usually take this as a pill or an injection (shot). Side effects may include liver damage or blood problems.
• Monoclonal antibodiesexternal link, also called immunotherapy, are used to treat cancer or an overactive immune system. These include rituximab, infliximab, golimumab, and adalimumab. Your doctor will give you the medicine as a shot or through an IV. Side effects are rare but can include a life-threatening immune reaction, heart problems, low blood counts, or a higher risk of certain cancers.
• Medicines used to treat malaria include hydroxychloroquine or chloroquine. Side effects may include eye damage, heart problems, and low blood sugar.
• Corticotropin (a hormone medicine) is given as a shot. It may be used when prednisone does not work or has serious side effects. Side effects of this medicine may include high blood pressure, problems controlling blood sugar, increased appetite, or mood changes.
• Pentoxifylline is taken as a pill. It is normally prescribed to improve blood flow. Side effects may include nausea.

Medicines to treat other symptoms

• Antibiotics treat sarcoidosis of the skin. Examples include minocycline, tetracycline, and doxycycline. Side effects may include dizziness and Gastrointestinal tractproblems.
• Colchicine treats joint pain from sarcoidosis. You take this medicine as a pill. It is usually prescribed for gout. Side effects include nausea, vomiting, diarrhea, and stomach cramps or pain.

Treating complications

If untreated, or if the treatment does not work, sarcoidosis can cause serious health problems. 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 help with breathing problems.
• Vasodilator treatment relaxes your blood vessels if you have pulmonary hypertension.
• Pulmonary rehabilitation helps you breathe easier and be more active.

Heart problems

• Implantable cardioverter defibrillator (ICD) helps prevent cardiac arrest.
• High blood pressure medicines, such as ACE inhibitors and beta blockers, lowers blood pressure.

Hormone problems

• Lifestyle changes, such as avoiding too much sunlight, drinking plenty of fluids, and eating fewer foods with calcium, may be needed if you have too much calcium in the bloodexternal link or urine.
• Hormone replacement treatment is used to treat some types of sarcoidosis.

Brain, nerve, or muscle problems

• Anti-seizure medicines are used if sarcoidosis affects your brain and causes seizures.
• Medicines can be given for nerve or muscle pain.
• Physical therapy improves muscle strength.
• Surgery removes brain tumors.

Severe organ damage

• Transplant surgery can be performed if sarcoidosis causes life-threatening lung, heart, or liver damage.

Manage your condition

Even if you do not have symptoms of sarcoidosis, you should see your healthcare provider for ongoing care. For example, 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.

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. 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.

Tests for complications

Some people have sarcoidosis that persists or comes back for many years after diagnosis. This 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.

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 brain tumors. Other complications that require immediate medical attention include kidney failure, 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.

• 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, and secondhand smoke.
• Try to get enough good quality sleep. Experts recommend that adults get 7 to 9 hours of sleep per day.

Take care of your mental health

Sarcoidosis may make you feel lonely, anxious, or depressed. You may continue to feel very tired 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 provider about local support groups or check with an area medical center.
• Talk to your doctor about medicines or other treatments. Antidepressants or other treatments may 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.

Pregnancy

Most women who have sarcoidosis give birth to healthy babies. However, they have 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. Also, tell your doctor about any medicines you take. Some sarcoidosis medicines are not safe to take during pregnancy.