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Sarcoidosis

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Sarcoidosis is an inflammatory disease in which clusters of inflamed cells called granulomas can occur in any organ but are mainly seen in the lungs and lymph nodes.

Causes of sarcoidosis

The exact cause or trigger of sarcoidosis is unknown. We do know that when a person has the disease, tiny clusters of inflamed cells (granulomas) form in the organs of the body.

The disease can affect almost any organ. It most commonly affects the lungs.

The disease is more common in African Americans and white people of Scandinavian heritage. Additionally, more women than men have the disease, but anybody can be affected by it. The disease often begins between ages 20 and 40. Sarcoidosis is rare in young children.

A person with a close blood relative who has sarcoidosis is nearly five times as likely to develop the condition.

Sarcoidosis occurs when a genetically predisposed individual is exposed to a trigger in the environment that sets off the immune system. The trigger(s) is yet unknown.

Sarcoidosis symptoms

Symptoms for sarcoidosis vary greatly and can range from no symptoms at all (detected by chance) to having vague symptoms such as fatigue, weight loss, cough, or symptoms related to the organ involved with sarcoidosis.

Almost all people affected by sarcoidosis have lung or chest symptoms:

  • Chest pain, most often felt behind the breast bone
  • Dry cough
  • Shortness of breath
  • Coughing up blood, which is rare, but serious

Symptoms of general discomfort may include:

  • Fatigue
  • Fever
  • Joint ache or pain (arthralgia)
  • Weight loss

Skin symptoms may include:

  • Raised, red, firm skin sores (erythema nodosum), almost always appear on the front part of the lower legs
  • Various rashes
  • Scars that become raised or inflamed

Nervous system symptoms may include:

  • Headache
  • Seizures
  • Weakness and/or numbness on one side of the body or face
  • Mental fog

Eye symptoms may include:

  • Red eyes
  • Blurred vision
  • Loss of vision
  • Dry eyes
  • Pain

Heart symptoms may include:

  • Abnormal heart beats, either too fast or too slow
  • Passing out or nearly passing out
  • Weak heart muscle
  • Trouble breathing at night

Diagnosing and evaluating sarcoidosis

The first step in the assessment of sarcoidosis is confirming the diagnosis by excluding other diseases that may look like sarcoidosis. Then it is important to determine the extent of the disease. This includes:

  • A thorough discussion with a sarcoidosis specialist about your symptoms followed by a physical examination.
  • A biopsy from an affected organ is most likely needed to confirm the diagnosis and rule out other diseases that mimic sarcoidosis.

Assessing extent of the disease with further testing that may include:

  • Chest X-ray to see if the lungs are involved or lymph nodes are enlarged
  • Breathing tests
  • CT scan of the lungs
  • Electrocardiogram to assess the heart
  • Echocardiogram or MRI of the heart if necessary
  • An eye exam by an eye doctor (ophthalmologist)

The following lab tests may be done:

  • Complete blood count
  • Liver and kidney function
  • Calcium levels in urine and blood

Treating sarcoidosis

Not all sarcoidosis patients need to be treated for their disease. The goal of treatment is to prevent further worsening of organs affected with sarcoidosis and to improve their function and with it the patient’s symptoms.

There are several treatment options available:

Steroids (corticosteroids, prednisone)

Steroids reduce the inflammation quickly and can improve symptoms quickly. Long-term use is associated with several side effects so their use is usually limited to short durations. This is accomplished with the help of second line drugs.

Second line drugs

These are also known as steroid-sparing drugs. They are usually used for long-term management of sarcoidosis. Drugs in this category include methotrexate, mycophenolate mofetil, azathioprine, and leflunomide.

Third line drugs

Also known as biological agents (infliximab, adalimumab). They are infusion or injectable drugs that are reserved for patients who do not respond to second line drugs.

Other drugs with specific indications

Hydroxychloroquine

Ancillary therapies

These include oxygen supplementation for those with low oxygen levels and rehabilitation (lung and/or heart).

Outlook for sarcoidosis patients

The overall prognosis or outlook for sarcoidosis patients is promising with proper assessment and management. Many people with sarcoidosis are not seriously ill, and can get better without treatment. Overall death rate from sarcoidosis is less than 5 percent and the two leading causes of death from sarcoidosis are lung fibrosis and heart involvement.

Possible complications from sarcoidosis

Sarcoidosis may lead to these health problems:

  • Fungal lung infections (aspergillosis)
  • Glaucoma and blindness from eye involvement
  • Kidney stones from high calcium levels in blood or urine
  • Osteoporosis and other complications of taking corticosteroids for long periods of time
  • High blood pressure in the arteries of the lungs (pulmonary hypertension)

Contacting a medical professional about sarcoidosis

Every sarcoidosis patient should be assessed in a sarcoidosis specialty center at least once to get a thorough evaluation of their disease. Our sarcoidosis physicians have expertise in the assessment and management of sarcoidosis patients. Our group coordinates care with other sub-specialists and with primary care physicians to ensure comprehensive care for our sarcoidosis patients.