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Pulmonary sarcoidosis

Sarcoidosis
Synonyms Sarcoïdosis, sarcoid
Chest X-ray of sarcoidosis nodules.png
Chest X-ray showing the typical nodularity of sarcoidosis, predominantly in the bases of the lungs.
Pronunciation
  • sar-koy-DO-sis
Specialty Rheumatology
Symptoms Depends on the organ involved
Lungs: wheezing, cough, shortness of breath, chest pain
Skin: lumps, ulcers, discolored skin
Children: weight loss, bone pain, feeling tired
Usual onset 20–50 year old women
Duration Few years to long term
Causes Unknown
Risk factors Family history
Diagnostic method Based on symptoms and tissue biopsy
Similar conditions Tuberculosis, lymphoma, infectious mononucleosis, pulmonary eosinophilia
Treatment Ibuprofen, prednisone, methotrexate
Prognosis Mortality 1–7%
Frequency 1.9 million with interstitial lung disease (2015)
Deaths 122,000 with interstitial lung disease (2015)
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Classification
External resources

Sarcoidosis is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomas. The disease usually begins in the lungs, skin, or lymph nodes. Less commonly affected are the eyes, liver, heart, and brain. Any organ, however, can be affected. The signs and symptoms depend on the organ involved. Often there are no, or only mild, symptoms. When it affects the lungs there may be wheezing, cough, shortness of breath, or chest pain. Some may have Löfgren syndrome in which there is fever, large lymph nodes, arthritis, and a rash known as erythema nodosum.

The cause of sarcoidosis is unknown. 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. Those with affected family members are at greater risk. Diagnosis is partly based on signs and symptoms, which may be supported by biopsy. 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 (ACE) in the blood. The diagnosis should only be made after excluding other possible causes of similar symptoms such as tuberculosis.

Sarcoidosis may resolve without any treatment within a few years. However, some people may have long term or severe disease. Some symptoms may be improved with the use of anti-inflammatory drugs such as ibuprofen. In cases where the condition causes significant health problems steroids such as prednisone are indicated. Medications such as methotrexate, chloroquine, or azathioprine may occasionally be used in an effort to decrease the side effects of steroids. The risk of death is between one and seven percent. There is a less than five percent chance of the disease returning in someone who has had it previously.


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