Sarcoidosis

What is sarcoidosis?

Sarcoidosis is an inflammatory disease of the lung, which can also involve the entire body. Inflammation is usually a healthy response to injury and infection, but in this case, it has been magnified and exaggerated. Consider it a type of “arthritis” of the lung. However, unlike arthritis, which is predictable, sarcoidosis is fairly unpredictable. It can appear and disappear at will. It can be completely benign and never require therapy, or it can be devastating and lead to pulmonary hypertension (severe high blood pressure in the lungs), fibrosis (scarring), and lung destruction requiring lung transplantation. Think of sarcoidosis as a sneaky inflammatory disease, which requires constant vigilance, and immediate action whenever it “rears its ugly head.” Since it is a systemic disease, it behooves you to have an initial “survey of the damage,” with careful evaluation of the eyes, heart, lungs, liver, joints, skin, and kidneys. Serial evaluations are then needed and are focused on sites of activity. Most people with sarcoidosis are seen at least once, by a Pulmonologist (lung), Rheumatologist (joint), Ophthalmologist (eye), Nephrologist (kidney) and Cardiologist (heart). It can also spread to the pituitary/brain, but commonly, it is limited to the lung.

How is active sarcoidosis treated?

One might think that being an inflammatory lung disease it would be treated with inhaled steroids. You would not be far from the truth. Unfortunately, inhaled steroids cannot reach the potency usually needed for active sarcoidosis and high dose oral and/or intravenous steroids are usually necessary, often for a prolonged time with a slow taper. It is this high dose which gives steroids a bad name, and causes so many side effects, which usually require additional medications to treat the side effects so that patients can be adequately treated. Some of these side effects include insomnia, heartburn, bone loss, cataracts, swelling and weight gain, muscle weakness, increased risk of certain infections, and accelerated heart disease. Steroids are used because initially they are the safest medication. However, with chronic use they have so many side effects, that they are usually undesirable. If someone does require chronic therapy, immunosuppressant medications are usually better tolerated. They do, however, require more close monitoring to use.

How do I know if I need to stay on steroids?

The usual treatment for the first flare of sarcoidosis is with a prolonged course of high dose steroids. It is common to be on tapering doses of steroids over a six-month period. If your symptoms return after stopping or while tapering the steroids, your doctor may discuss other options for treatment with you. Some people respond so well to steroids, that they may go many years without any medications and without any signs of disease.

What tests might be done to evaluate sarcoidosis?

The usually testing includes: CAT scan of the chest, pulmonary function testing, blood work (including evaluation of the liver and kidney), ACE level, gallium scan, urine testing, ophthalmologic screening, and an EKG. Tests for tuberculosis exposure and immune function may also be performed. Sometimes, tests are done to exclude other joint disorders. Frequently, to make the diagnosis of sarcoidosis a biopsy is required. The biopsy is usually taken from a site from which the disease is suspected to be active. This may include a lung biopsy performed by bronchoscopy (camera in the airways) or mediastinoscopy (camera through the chest wall). Biopsies can also be taken, however, from the skin, the liver, or the kidney.

Could I have developed sarcoidosis from exposure to a toxin or pollutant?

There is not one clear cause of sarcoidosis currently. However, there are many studies underway investigating this question and there is some evidence that increased airway irritants or infections may play a role in the cause of this disease.

Will my sarcoidosis get worse or go away?

Most patients who are treated will go into remission and will not have active disease. However, some patients will have the disease flare or recur either immediately or in the next 5-10 years. It can continue to wax and wane like this throughout their lives. Some patients will have disease that will spread and get worse despite treatment and may require aggressive interventions, including dialysis and lung transplantation.

Self-Check for Management of Sarcoidosis
  • I understand the different organ systems that can be affected by sarcoidosis and what screening tests I need to do yearly.
  • I understand the side effects of high dose steroids, and how to best protect myself and deal with insomnia, bone loss, heartburn, and infection risk.
  • I am up to date with my vaccinations to prevent lung infections.
Managing Doctors
  • Primary Care
  • Pulmonologist
  • Rheumatologist
  • Ophthalmologist
  • Nephrologist
  • Cardiologist
  • Neurologist
  • Endocrinologist
  • Dermatologist
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