Sarcoidosis: Your GoodRx Guide

Definition

Sarcoidosis is a rare inflammatory disease where small clumps of immune cells form in different organs in the body. These clumps are called granulomas. They can be found anywhere in the body, but the lungs tend to be affected in most people with sarcoidosis. Other commonly affected areas include lymph nodes, skin, joints, and eyes. Over time, sarcoidosis can cause permanent scarring — also known as  fibrosis — in the tissues and organs it affects.

Sarcoidosis affects people differently. That means the signs, symptoms, and severity are not the same from person to person. Some people will need lifelong treatment, and, in some cases, sarcoidosis goes away on its own. 

Some people have what’s called Lofgren syndrome when they first get sarcoidosis. This is a sudden but short-lived subset of sarcoidosis that lasts for a year or two. You may hear about other “types” of sarcoidosis, such as cardiac (heart) sarcoidosis or pulmonary (lung) sarcoidosis. These simply refer to the location of the disease in the body and are not a separate disease. 

Sarcoidosis affects the lungs in 90% of people with it. 50% to 80% of people with sarcoidosis will need long-term treatment.

Causes

Despite much research, the exact cause of sarcoidosis is unknown. Formation of granulomas is thought to be due to a complex immune process that leads to inflammation in certain areas. This inflammation likely starts with an environmental trigger – such as infection or chemicals – in people who are genetically predisposed. This means some people are more likely to get it because of their genes, and, when they are exposed to a trigger, the disease develops.

Sarcoidosis is not typically considered an autoimmune disease. However, there are similar characteristics and some evidence to support an autoimmune process in sarcoidosis. More research is needed to determine the exact cause.

Sarcoidosis can affect people of any age, sex, or ethnicity. But a few factors can increase the risk of sarcoidosis:

• Genetics: People with sarcoidosis are five times more likely to have a relative with it.

• Obesity: The incidence is higher in women with a past or current high BMI; research is lacking in men. 

• Race: Sarcoidosis is two to seven times more common in Black people vs non-Hispanic white people. The lowest incidence is reported in people of Hispanic and Asian descent. 

It was previously thought that sarcoidosis most commonly affects women between the ages of 20 to 40 years. But recent research suggests it’s just as common between ages 46 to 65. In fact, one study found that 55% of people with a new diagnosis of sarcoidosis were over age 55. Some studies report that sarcoidosis is more common in people with female anatomy, but others do not confirm this finding. 

Symptoms

Symptoms of sarcoidosis tend to come on slowly. Because any organ(s) can be affected, there is a lot of variation in the symptoms. It’s even possible to have no symptoms at all. Common, generalized symptoms include:

• Fatigue

• Fever

• Weight loss

• Feeling unwell

• Sleep changes

• Joint pain

• Muscle pain

• Weakness 

• Numbness, tingling, and/or pain in the hands and feet

Sarcoidosis can also cause symptoms specific to the organ(s) affected. For example, sarcoidosis in the lungs — which occurs in up to 90% of people with sarcoidosis — can lead to: 

• Cough

• Wheezing 

• Chest pain

• Shortness of breath

Sarcoidosis in other organs can cause:

• Painful, itchy, teary eyes

• Blurred vision

• Irregular heart beat

• Abdominal pain

• Skin rash

• Skin inflammation with tender lumps (called erythema nodosum)

Lofgren syndrome tends to come on suddenly. The signs and symptoms are: 

• Fever

• Pain in more than one joint

• Enlarged lymph nodes in the chest

• Erythema nodosum

In addition to these physical symptoms, sarcoidosis can have effects on mental health. Depressive symptoms, anxiety, and stress are possible, as well as problems with thinking, concentration, and memory.

Diagnosis

There is no specific test for sarcoidosis. The diagnosis is usually based on a combination of your symptoms, physical examination, and other testing (see below). Because there is no test, and symptoms of sarcoidosis can be similar to those of other conditions, diagnosis can be a bit tricky. It’s important to rule out other conditions in the process.

To make a diagnosis, the following are required:

• Symptoms of sarcoidosis

• No other obvious explanation for having granulomas

• Confirmation of granuloma with biopsy (more below)

Common tests performed to confirm sarcoidosis and rule out other conditions include: 

• Imaging tests such as chest X-ray, CT, or MRI to get a good look at your lungs (and any other organ(s) your provider thinks is involved).

• Pulmonary function testing (PFT), or lung-function testing, to assess how well your lungs are working.

• Biopsy, or tissue sample study, to look at the cells of a granuloma under a microscope. This can be done with tissue from your lungs, skin, lymph nodes, etc., depending on your symptoms.

• Blood tests, which can check your blood counts, kidney and liver function, and markers of inflammation.

• Cardiac (heart) tests, such as an electrocardiogram or echocardiogram, to see if the disease affects your heart.

• Eye examination and testing by a specialist if sarcoidosis affects your eyes.

• Neurologic testing, such as a nerve conduction study or a spinal tap, to determine if the nervous system is involved.

Living with sarcoidosis

It’s difficult to predict the course of sarcoidosis. In many cases, medication isn’t needed. However, some people will need aggressive treatment. It’s important to discuss your symptoms, other medical problems (if any), and healthcare goals with your provider. These can help determine the best treatment course for you. 

Regardless of whether medication is recommended right away, regular checkups with your healthcare provider will be. It’s important to stay on top of signs and symptoms to be sure the disease is not progressing. For example, if sarcoidosis affects your lungs, you may need repeat imaging or lung-function tests to help guide decisions regarding treatment.

The good news is, sarcoidosis is usually not a fatal disease. But it can affect many aspects of life, including sleep, mental health, and day-to-day functioning. As such, other therapies can help with symptoms and quality of life. Examples include:

• Cognitive therapy

• Stress reduction

• Physical therapy

• Counseling 

These options can help reduce fatigue, increase physical stamina, and improve psychological well-being.

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Medications

Medication is usually recommended when the disease becomes life-threatening or symptoms become disabling and/or affect quality of life. Research suggests 50% to 80% of people with sarcoidosis will need long-term treatment.

Glucocorticoids, or “steroids,” are the first-line treatment. Steroids, most commonly prednisone, reduce inflammation and granuloma formation. In turn, they can help with symptoms and lung function. A high dose may be needed at first. But weaning down to the lowest possible dose over time (if possible) can help avoid long-term effects of steroids. 

Second-line therapy usually involves medications called disease-modifying antirheumatic drugs (DMARDs), such as methotrexate, azathioprine, and leflunomide. These medications modify the immune response. They are called DMARDs because they slow the progression of autoimmune conditions like rheumatoid arthritis. However, they do not modify or slow the disease process in sarcoidosis. These medications are not FDA approved for the treatment of sarcoidosis. But they are often recommended by experts when steroids don’t work well enough.

Biologic agents and biosimilars — specifically the tumor necrosis factor (TNF) inhibitors infliximab and adalimumab — may be prescribed when steroids and DMARDs don’t work or cause too many side effects. Small studies have shown some efficacy, but they are also not FDA approved for the treatment of sarcoidosis. 

You can also speak with your healthcare provider about medication options for depressive symptoms and fatigue, if you’re interested.

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