Sarcoidosis

What Is Sarcoidosis?

Sarcoidosis

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.

What causes 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 genes can affect how your immune system reacts to a trigger.

What raises your risk of sarcoidosis?

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.
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  • 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.

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 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
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.
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 antibodies, also called immunotherapy, are used to treat cancer or an overactive immune systemThese 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 tract problems.
  • 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.

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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 blood 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.

Source: https://www.nhlbi.nih.gov/health/sarcoidosis

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