This article was last reviewed on
This article waslast modified on
21 September 2017.
What is sarcoidosis?

Sarcoidosis is a condition in which inflammatory cells produce nodules called granulomas in multiple organs. Granulomas can develop anywhere in the body, but they commonly affect the lungs, skin, lymph nodes, and eyes. Granulomas change the structure of the tissues around them and, in sufficient numbers they can cause damage, inflammation and symptoms and may interfere with normal functions.

The cause of sarcoidosis is not known. The condition is thought to be associated with both a genetic predisposition and the immune system's reaction to an environmental trigger – such as exposure to a virus, bacteria, allergen, dust, or chemical. Anyone can develop sarcoidosis, but it most frequently occurs in women of African or Northern European descent who are 20 to 50 years of age. It effects about 19 people in every 1,000 in the UK.

Sarcoidosis varies in severity. A person may have the disease without knowing it as mild cases cause no, few, or nonspecific symptoms. It can cause acute illness, resolve on its own within a few years (remission), recur, or continue as a chronic condition. More than half of those affected will go into remission within 3 years of diagnosis, and two-thirds will be in remission within 10 years.

Most people with sarcoidosis will not experience long-term health effects, but about one-third will have some degree of organ damage. Sarcoidosis can cause blindness in rare cases and can sometimes be fatal, primarily in those with severe lung or heart involvement.

 

Accordion Title
About Sarcoidosis
  • Signs and Symptoms

    The severity of sarcoidosis and the symptoms a person experiences depend upon the tissues and organs affected. A person may have no symptoms or may have nonspecific findings such as:

    • Fever
    • Fatigue
    • Weight loss
    • Night sweats
    • Loss of appetite
    • Swollen lymph nodes
    • Joint pain

    Signs and symptoms associated with specific organs are listed below.

    Lungs

    The American Lung Association estimates that as many as 90% of those with sarcoidosis will have lung involvement. Lung tissues may stiffen and scar tissue may develop. Those affected may have:

    • Dry cough
    • Wheezing
    • Shortness of breath
    • Chest pain

    Skin

    Up to 25% of those with sarcoidosis have skin problems, including:

    • Sores on or near the cheeks, ears, nose, and eyelids
    • A raised, reddish, bumpy rash on the ankles or shins that may itch or be tender and warm
    • Discoloured skin
    • Inflammation and nodules around scars

    Eyes

    • Blurred vision
    • Sensitivity to light
    • Eye pain and itching
    • Burning and redness
    • Tears
    • Inflammation

    Heart

    Brain and Nervous System

    • Headaches
    • Weakness
    • Seizures
    • Decreased coordination and tremors

    Muscle and Bone

    • Pain
    • Joint stiffness

    Sarcoidosis can also cause kidney and liver dysfunction, an enlarged liver or spleen, and swollen salivary glands. It can alter the way the body processes calcium, leading to increased calcium in the blood and urine and the formation of kidney stones. In rare cases, it may cause kidney failure.

     

  • Tests

    The goals with testing are to diagnose sarcoidosis, evaluate its severity, and to monitor its course over time. Testing is also used to distinguish sarcoidosis from conditions with similar symptoms and from conditions that are also associated with the development of granulomas – such as tuberculosis and some fungal infections.

    Laboratory Tests

    Tissue biopsy is the primary test used to confirm a diagnosis of sarcoidosis; characteristic changes in the structure of the tissue can be seen under the microscope. Other laboratory tests are not specific for sarcoidosis, but they are helpful in assessing disease activity, looking for damage to individual organs, and ruling out other diseases that may cause similar problems Tests that may be requested include:

    • ACE (Angiotensin converting enzyme) - often elevated in patients with sarcoidosis but may be increased with other conditions; this test is used to help diagnose sarcoidosis, monitor disease activity, and monitor response to treatment.
    • Calcium - to detect increases in the blood or urine. Hypercalciuria (increased calcium in urine) occurs in about 50%, while hypercalcaemia (increase blood calcium) can be found in about 5% of patients with Sarcoidosis
    • ESR (Erythrocyte Sedimentation Rate) - sometimes requested to detect inflammation
    • Liver Function Tests and/or Renal Profile - a group of tests used to evaluate liver and/or kidney function, to determine if those organs are affected
    • FBC (Full Blood Count) - may be requested to evaluate red and white blood cell changes
    • CSF analysis - an evaluation of the cerebrospinal fluid when brain or nervous system involvement is suspected
    • AFB cultures, sputum cultures, and fungal tests - to help distinguish between sarcoidosis and other conditions associated with the lungs and with granuloma development

    Non-Laboratory Tests

    • X-ray - one of the primary tests used to detect lung involvement; granulomas may be initially detected in people without symptoms who have an X-ray for another reason.
    • Lung function tests are performed to evaluate lung involvement, lung capacity, and condition severity. (For more on these, visit the Royal Brompton and Harefield NHS Foundation Trust website.)
    • CT (Computed Tomography), MRI (Magnetic Resonance Imaging), gallium scan, or other imaging scans are sometimes requested to help diagnose and evaluate sarcoidosis. ECG (electrocardiogram) - sometimes ordered when heart involvement is suspected.

     

  • Treatment

    There is no way to prevent or cure sarcoidosis, but in many cases it will resolve on its own over time. The goals of treatment are to relieve symptoms, decrease inflammation, and to minimise tissue and organ damage. People who have few or mild symptoms may not need treatment, but their condition should be monitored.

    Those with moderate to severe symptoms and those at risk for tissue or organ damage are usually treated with corticosteroids such as prednisone. These anti-inflammatory medications may be given orally, topically, or through an inhaler. Long-term use of corticosteroids can cause significant side-effects.

    Other medications may include:

    • Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen for pain and inflammation
    • Methotrexate and other drugs that suppress the immune system for lung, skin, or eye involvement
    • Hydroxychloroquine (an anti-malarial drug) may be useful for skin and nervous system involvement, especially in people who have increased calcium levels 

    Most people can be successfully treated, but they may need to take medications for an extended period of time. Rarely, a person may need an organ transplant if the lungs or liver have become severely damaged.