Sarcoidosis | |
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Other names | Sarcoïdosis, sarcoid, Besnier–Boeck–Schaumann disease[1] |
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Chest X-ray showing the typical nodularity of sarcoidosis, predominantly in the hila of the lungs. | |
Pronunciation |
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Specialty | Rheumatology, Immunology |
Symptoms | |
Usual onset | 20–50 years old More common in women[4] |
Duration | Few years to long term[2][5] |
Causes | Unknown[2] |
Risk factors | Family history[4] |
Diagnostic method | Based on symptoms and tissue biopsy[6] |
Differential diagnosis | Tuberculosis, lymphoma, infectious mononucleosis, pulmonary eosinophilia[7] |
Treatment | Ibuprofen, prednisone, methotrexate[8][9] |
Prognosis | Mortality 1–7%[5] |
Frequency | 1.9 million with interstitial lung disease (2015)[10] |
Deaths | 122,000 with interstitial lung disease (2015)[11] |
Sarcoidosis (also known as Besnier–Boeck–Schaumann disease) is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomata.[2] The disease usually begins in the lungs, skin, or lymph nodes.[2] Less commonly affected are the eyes, liver, heart, and brain, though any organ can be affected.[2] The signs and symptoms depend on the organ involved.[2] Often, no, or only mild, symptoms are seen.[2] When it affects the lungs, wheezing, coughing, shortness of breath, or chest pain may occur.[3] Some may have Löfgren syndrome with fever, large lymph nodes, arthritis, and a rash known as erythema nodosum.[2]
The cause of sarcoidosis is unknown.[2] Some believe it may be due to an immune reaction to a trigger such as an infection or chemicals in those who are genetically predisposed.[12][13] Those with affected family members are at greater risk.[4] Diagnosis is partly based on signs and symptoms, which may be supported by biopsy.[6] Findings that make it likely include large lymph nodes at the root of the lung on both sides, high blood calcium with a normal parathyroid hormone level, or elevated levels of angiotensin-converting enzyme in the blood.[6] The diagnosis should be made only after excluding other possible causes of similar symptoms such as tuberculosis.[6]
Sarcoidosis may resolve without any treatment within a few years.[2][5] However, some people may have long-term or severe disease.[5] Some symptoms may be improved with the use of anti-inflammatory drugs such as ibuprofen.[8] In cases where the condition causes significant health problems, steroids such as prednisone are indicated.[9] Medications such as methotrexate, chloroquine, or azathioprine may occasionally be used in an effort to decrease the side effects of steroids.[9] The risk of death is 1–7%.[5] The chance of the disease returning in someone who has had it previously is less than 5%.[2]
In 2015, pulmonary sarcoidosis and interstitial lung disease affected 1.9 million people globally and they resulted in 122,000 deaths.[10][11] It is most common in Scandinavians, but occurs in all parts of the world.[14] In the United States, risk is greater among black people as opposed to white people.[14] It usually begins between the ages of 20 and 50.[4] It occurs more often in women than men.[4] Sarcoidosis was first described in 1877 by the English doctor Jonathan Hutchinson as a non-painful skin disease.[15]
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