Sarcoidosis

Sarcoidosis

Sarcoidosis is a rare condition in which groups of immune cells form lumps, called granulomas, in various organs in the body. Inflammation, which may be triggered by infection or exposure to certain substances, is thought to play a role in the formation of granulomas.

Sarcoidosis can affect any organ. Most often it affects the lungs and lymph nodes in the chest. You may experience fatigue, which is extreme tiredness, or fever, but you may also experience other signs and symptoms depending on the organ that is affected. Your doctor will diagnose sarcoidosis in part by ruling out other diseases that have similar symptoms.

Determining whether treatment is needed and what type depends on your signs and symptoms, which organs are affected, and how well those organs are working. Medicines used to treat sarcoidosis help reduce inflammation or suppress the immune system. Many people recover with few or no long-term problems. Sometimes the disease causes permanent scarring in the affected organs. When scarring happens in the lungs, this is called pulmonary fibrosis.

Causes- Sarcoidosis

Sarcoidosis is a condition in which immune cells form lumps, called granulomas, in your organs. Inflammation is thought to cause granulomas to form and may lead to temporary or permanent scarring at the site of the granulomas. Your inherited genes or certain environmental factors may trigger the inflammation that leads to granulomas.

Sarcoidosis - Crystalline inclusion, polarized

- Genetics

Recent studies suggest that people get sarcoidosis because of genes that make you susceptible to the disease. Some of the genes that are related to sarcoidosis are associated with the immune system.

- Environment

Environmental factors, such as infection or exposure to certain substances, can trigger changes in the immune system and lead to sarcoidosis. Studies suggest that these triggers may cause sarcoidosis only in people with genes that make them susceptible to the disease.

Risk Factors- Sarcoidosis

You may have an increased risk for sarcoidosis because of your age, your family history and genetics, or your race. Some risk factors, such as occupation, can be changed. Most risk factors, such as age, family history and genetics, race and ethnicity, and sex, cannot be changed.

Age - You can be diagnosed with sarcoidosis at any age, but sarcoidosis is most commonly diagnosed in people age 55 and older.

Environment or occupation - Your risk for sarcoidosis may be higher if you have repeated exposure to environmental substances that cause inflammation, such as insecticides or mold, or if you work in health care or as a firefighter. Working in these occupations may expose you to substances that trigger the formation of granulomas.

• Family history and genetics - You have a higher risk of sarcoidosis if you have a close relative with sarcoidosis.
• Other medical conditions - Sarcoidosis sometimes occurs after lymphoma, a type of blood cancer.
• Race or ethnicity - People of any race can get sarcoidosis, but it is more common in people of African or Scandinavian descent.
• Sex - Both men and women can develop sarcoidosis, but it is more common in women.

Sarcoidosis - Honeycombing of lungs

Screening and Prevention- Sarcoidosis

Currently, there are no screening methods to determine who will develop sarcoidosis. If you are at risk for sarcoidosis, your doctor may recommend you try to avoid insecticides, mold, or other environmental sources of substances known to trigger the formation of granulomas.

Signs, Symptoms, and Complications- Sarcoidosis

Many people who have sarcoidosis have no signs or symptoms. Some people experience general signs and symptoms of sarcoidosis such as fever and weight loss. Others will experience signs and symptoms that will depend on which organs are affected. If inflammation continues, some people may develop permanent scarring, which can lead to life-threatening serious heart or lung complications.

Signs and Symptoms - Sarcoidosis

Many people have general signs and symptoms, such as:

• Depression
• Fatigue
• Fever
• Malaise, or a feeling of discomfort or illness
• Pain and swelling in the joints
• Weight loss

Sarcoidosis most often affects the lungs and the lymph nodes in the chest. Some people with sarcoidosis in the lungs may wheeze, cough, feel short of breath, or have chest pain. However, people with sarcoidosis in the lungs do not always have lung-related symptoms.

If sarcoidosis affects other organs or parts of your body, you may have other symptoms related to those organs:

• Abdominal pain
• A larger than normal spleen called splenomegaly
• Anemia
• Burning, itchy, or dry eyes
• Fainting
• Heart palpitations
• Joint pain
• Muscle weakness
• Problems with a liver that is larger than normal, including itching, vomiting, nausea, jaundice, or abdominal pain
• Problems with the nervous system, including headache, dizziness, vision problems, seizures, mood swings, disturbed behavior, hallucinations, delusions, back pain, or pain associated with particular nerves
• Skin changes, including erythema nodosum or lupus pernio, a condition that causes skin sores that usually affect the face, especially the nose, cheeks, lips, and ears. The sores associated with lupus pernio tend to last a long time. Lupus pernio occurs mostly in African Americans and can return after sarcoidosis treatment is over.
• Swelling of the salivary glands
• Swollen or tender lymph nodes in other areas of the body besides the chest, such as in your neck, chin, armpits, or groin

Sarcoidosis - Asteroid body

Lofgren’s syndrome - sign of sarcoidosis

Lofgren’s syndrome occurs in some people when they first have sarcoidosis, especially women between ages 30 and 40. Lofgren’s syndrome usually goes away completely within two years. It may include:

• Erythema nodosum
• Fever
• Joint pain
• Swollen lymph nodes or lymphadenopathy

Complications - Sarcoidosis

If sarcoidosis is untreated or if the treatment does not work, inflammation can continue and scarring may develop. Sarcoidosis can cause serious and life-threatening damage to the organs it affects, including:

• Blindness
• Blood and bone marrow problems, including lower-than-usual numbers of red or white blood cells
• Endocrine conditions, including hypercalcemiaexternal link, diabetes insipidus, and amenorrhea
• Heart complications, including arrhythmia, heart failure, sudden cardiac arrest, cardiomyopathy
• Kidney conditions, such as kidney stones or kidney failure
• Cirrhosis
• Lung diseases, such as pulmonary hypertension and pulmonary fibrosis
• Problems with the nervous system, including brain tumors, meningitis, hydrocephalus, psychiatric problems, and nerve pain

Diagnosis- Sarcoidosis

Your doctor will diagnose sarcoidosis based on your symptoms, a physical exam, imaging tests, or a biopsy of an affected organ. The doctor will also perform tests to rule out other diseases that have similar signs and symptoms.

Diagnostic tests and procedures - Sarcoidosis

To diagnose sarcoidosis and determine which organs are affected, your doctor may have you undergo some of the following tests and procedures:

• Biopsy of the lungs, liver, skin, or other affected organs to check for granulomas
• Blood tests, including complete blood counts, to check hormone levels and to test for other conditions that may cause sarcoidosis
• Bronchoscopy, which may include rinsing an area of the lung to get cells or using a needle to take cells from the lymph nodes in the chest
• Chest X-ray to look for granulomas in the lungs and heart and determine the stage of the disease. Often, sarcoidosis is found because a chest X-ray is performed for another reason.
• Neurological tests, such as electromyography, evoked potentials, spinal taps, or nerve conduction tests, to detect 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
• Gallium scanexternal link, which uses a radioactive material called gallium to look for inflammation, usually in the eyes or lymph nodes.
• High-resolution computed tomography (CT) scan to look for granulomas
• Magnetic resonance imaging (MRI) to help find granulomas. Learn more in our Chest MRI Health Topic.
• Positron electron tomography (PET) scan, a type of imaging that can help find granulomas
• Pulmonary function tests to check whether you have breathing problems
• Ultrasound to look for granulomas

Tests for other medical conditions - Sarcoidosis

To help diagnose sarcoidosis, your doctor may need to perform tests or ask questions to rule out other medical conditions that have similar signs and symptoms as sarcoidosis.

• Blood tests to help the doctor distinguish between sarcoidosis and cancer or infections
• A bronchoscopy to find signs that may suggest an infection or cancer
• Questions about environmental exposure to help determine whether the granulomas are related to sarcoidosis or another condition. Exposure to beryllium, for example, can cause granulomas similar to sarcoidosis even though they are actually associated with chronic beryllium disease
• A urinalysis to help the doctor rule out other conditions that resemble sarcoidosis

An illustration depicting sarcoidosis

Stages of Sarcoidosis

Doctors use stages to describe the various imaging findings of sarcoidosis of the lung or lymph nodes of the chest. There are four stages of sarcoidosis, and they indicate where the granulomas are located. In each of the first three stages, sarcoidosis can range from mild to severe. Stage IV is the most severe and indicates permanent scarring in the lungs.

• Stage I: Granulomas are located only in the lymph nodes.
• Stage II: Granulomas are located in the lungs and lymph nodes.
• Stage III: Granulomas are located in the lungs only.
• Stage IV: Pulmonary fibrosis.

Treatment- Sarcoidosis

The goal of treatment is remission, a state in which the condition is not causing problems. Not everyone who is diagnosed with sarcoidosis needs treatment. Sometimes the condition goes away on its own. Whether you need treatment—and what type you need—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.

Medicines - Sarcoidosis

Because inflammation is thought to be involved in sarcoidosis, your doctor may prescribe medicines to reduce inflammation or treat an overactive immune system that may be causing too much inflammation in the body. Some of the medicines include:

• Corticosteroids to reduce inflammation. The corticosteroid prednisone is the most commonly used 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.
• Disease-modifying antirheumatic drugs (DMARDs) to treat an overactive immune system. Examples of DMARDs include methotrexate, azathioprine, and leflunomide. Potential side effects include liver damage.
• Monoclonal antibodies to treat an overactive immune system. Examples include infliximab, adalimumab, rituximab, and golimumab.
• Antibiotics to treat sarcoidosis of the skin. Side effects of common antibiotics, such as minocycline, tetracycline, and doxycycline, include dizziness and gastrointestinal problems.
• Antimalarials to treat sarcoidosis of the skin, lungs, or nervous system. These medicines are typically used to fight malaria. Side effects include eye damage.
• Colchicine to treat joint pain from sarcoidosis. This medicine is usually prescribed for gout. Side effects include nausea, vomiting, diarrhea, and stomach cramps or pain.
• Corticotropin to treat an overactive immune system. This is a type of hormone therapy. Side effects include changes to appetite or mood.
• Pentoxifylline to block the release of TNF-a, a substance in white blood cells that can cause granulomas. This medicine is normally prescribed to improve blood flow. Side effects include nausea.

Living With

If you have been diagnosed with sarcoidosis, it is important that you continue your medicine, follow up with your doctor when directed, make healthy lifestyle changes, and prevent complications. Some people may achieve total, or life-long, remission. Other people may achieve temporary remission and go on to experience a relapse, or return of the disease. If you are pregnant, talk to your doctor about the medicines you take to avoid problems for you and your baby.

Sarcoidosis - Hamazaki-Wesenberg (H-W) bodies- Lung

Receive Follow Up Care - Sarcoidosis

How often you need to see your doctor will depend on the severity of your symptoms, which organs are affected, which treatments you are using, and whether you have any side effects from treatment. Even if you do not have symptoms, you should see your doctor for ongoing care.

Monitor Your Condition - Sarcoidosis

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.

Some people have sarcoidosis that persists or recurs for many years after diagnosis. This may be called chronic, severe, advanced, refractory, or progressive sarcoidosis. To monitor your condition, your doctor may recommend the following tests at regular intervals.

• A chest X-ray to monitor your lungs for permanent scarring, also known as pulmonary fibrosis.
• An eye exam to detect eye damage. See an eye doctor every year. Sarcoidosis can cause permanent eye damage without symptoms.
• 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.
• Liver function tests to determine if your liver is working the way that it should. These tests are particularly important if you have liver sarcoidosis. You may need treatment for cholestasis, which is a type of liver disease that causes severe itching without a rash.
• Pulmonary function tests to monitor how sarcoidosis is affecting your lungs over time. If your lung function worsens, you may need different treatment.

Your doctor may perform other tests based on which organs are involved and what medicine is prescribed. For example, your doctor will monitor you for side effects of corticosteroids, such as osteoporosis, if used long term.

Adopt a healthy lifestyle - Sarcoidosis

Your doctor may recommend that you adopt lifelong lifestyle changes to help prevent sarcoidosis from worsening if you do not need medicine. These may include:

• Heart-healthy eating
• Aiming for a healthy weight
• Being physically active. Even though fatigue can make it hard to exercise if you have sarcoidosis, physical activity can actually improve energy and help with other symptoms, such as shortness of breath and muscle weakness. Try to stay as active as you can, but talk to your doctor first about an exercise program that is appropriate for you.
• Managing stress
• Quitting smoking. If you smoke, quit. Also, try to avoid other lung irritants, such as dust, chemicals, and secondhand smoke.

Gallium scan showing panda (A) and lambda (B) patterns

Prevent a Relapse of Sarcoidosis

If your sarcoidosis goes into remission, your doctor may carefully stop your medicines. However, he or she will still need to monitor you for a relapse, also called a flare, of the disease.

Relapses can be hard to predict. Most people who relapse do so in the first six months after stopping treatment. The longer you go without symptoms, the less likely you are to have a relapse.

See your doctor if you have a relapse. You may need a second round of treatment.

Prevent Complications of Sarcoidosis

To help prevent some of the complications of sarcoidosis, your doctor may recommend the following:

• An implantable cardioverter defibrillator that may help prevent sudden cardiac arrest.
• Surgery if sarcoidosis has damaged organs or you are at risk for serious or life-threatening health problems. Surgery can include a heart, lung, or liver transplant, or treatment for complications in the brain.
• Lifestyle changes if you have hypercalcemia, which means your body has problems absorbing calcium. This can lead to high levels of calcium in your blood and urine. Your doctor may recommend avoiding sunlight, drinking plenty of fluids, and eating fewer foods with calcium.

Other treatments may be used to treat organs that are less commonly affected.

• Anti-epileptic medicines if sarcoidosis affects your brain and causes seizures
• Eye drops
• Hormone replacement therapy if sarcoidosis causes endocrine problems
• Medicines for nerve pain
• Nonsteroidal anti-inflammatory drugs (NSAIDs)
• Physical therapy
• Radiation
• Vasodilator therapy to relax your blood vessels. This may be necessary if your sarcoidosis causes pulmonary hypertension.

Learn Warning Signs of Sarcoidosis

Watch for the warning signs of complications that may require emergency medical treatment. These include signs of blindness or brain tumors, such as changes in your vision or if you cannot see clearly or see color. Other complications that require immediate medical attention include kidney failure, sudden cardiac arrest, and sudden shortness of breath or muscle weakness.

Emotional Issues of Sarcoidosis

Sarcoidosis may make you feel socially isolated, anxious, or depressed, and you may continue to feel fatigued even after your treatment has ended. But certain activities or treatments may help improve your emotional health.

• Counseling, particularly cognitive therapy, can be helpful.
• Joining a patient support group, which may help you adjust to living with sarcoidosis. You can see how other people who have the same symptoms have coped with them. Talk with your doctor about local support groups or check with an area medical center.
• Medicines or other treatments. Your doctor may recommend 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.

Chest X-ray of sarcoidosis nodules

Pregnancy - Sarcoidosis

If you have sarcoidosis and are pregnant or planning to become pregnant, talk with your doctor about the risks involved. Also, if you become pregnant, it is important to get good prenatal care and regular sarcoidosis checkups during and after pregnancy.

Most women who have sarcoidosis give birth to healthy babies. Women with sarcoidosis are at risk for some complications related to pregnancy, including:

• Hemorrhaging after giving birth
• Preterm delivery
• Preeclampsia
• Venous thromboembolism, such as pulmonary embolism and deep vein thrombosis

Talk to your doctor about any medicines you take. Some medicines prescribed to adults with sarcoidosis are not safe to take during pregnancy.

External links

• WASOG (World Association for Sarcoidosis and Other Granulomatous Disorders)

Sarcoidosis Resources
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