Sarcoidosis

Sarcoidosis
Other namesSarcoïdosis, sarcoid, Besnier–Boeck–Schaumann disease[1]
Chest X-ray showing the typical nodularity of sarcoidosis, predominantly in the hila of the lungs.
Pronunciation
  • sar-koy-DOH-sis
SpecialtyRheumatology, immunology
Symptoms
Usual onset20–50 years old
More common in women[4]
DurationFew years to long term[2][5]
CausesUnknown[2]
Risk factorsFamily history[4]
Diagnostic methodBased on symptoms and tissue biopsy[6]
Differential diagnosisTuberculosis, lymphoma, infectious mononucleosis, pulmonary eosinophilia[7]
TreatmentIbuprofen, prednisone, methotrexate[8][9]
PrognosisMortality 1–7%[5]
Frequency1.9 million with interstitial lung disease (2015)[10]
Deaths122,000 with interstitial lung disease (2015)[11]

Sarcoidosis (/ˌsɑːrkɔɪˈdoʊsɪs/; 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 symptoms 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]

  1. ^ Konstantinidis G (2005). Elsevier's Dictionary of Medicine and Biology: in English, Greek, German, Italian and Latin. Elsevier. p. 1454. ISBN 978-0-08-046012-3.
  2. ^ a b c d e f g h i j k l "What Is Sarcoidosis?". NHLBI. June 14, 2013. Archived from the original on 6 April 2016. Retrieved 28 March 2016.
  3. ^ a b c d "What Are the Signs and Symptoms of Sarcoidosis?". NHLBI. June 14, 2013. Archived from the original on 7 April 2016. Retrieved 29 March 2016.
  4. ^ a b c d e "Who Is at Risk for Sarcoidosis?". NHLBI. June 14, 2013. Archived from the original on 7 April 2016. Retrieved 28 March 2016.
  5. ^ a b c d e Wijsenbeek MS, Culver DA (December 2015). "Treatment of Sarcoidosis". Clinics in Chest Medicine. 36 (4): 751–67. doi:10.1016/j.ccm.2015.08.015. PMID 26593147.
  6. ^ a b c d Govender P, Berman JS (December 2015). "The Diagnosis of Sarcoidosis". Clinics in Chest Medicine. 36 (4): 585–602. doi:10.1016/j.ccm.2015.08.003. PMID 26593135.
  7. ^ Ferri FF (2010). Ferri's differential diagnosis: a practical guide to the differential diagnosis of symptoms, signs, and clinical disorders (2nd ed.). Philadelphia, PA: Elsevier/Mosby. p. Chapter S. ISBN 978-0-323-07699-9.
  8. ^ a b Drent M, Cremers JP, Jansen TL (May 2014). "Pulmonology meets rheumatology in sarcoidosis: a review on the therapeutic approach". Current Opinion in Rheumatology. 26 (3): 276–84. doi:10.1097/bor.0000000000000052. PMID 24614277. S2CID 24353355.
  9. ^ a b c Judson MA (February 2016). "Corticosteroids in Sarcoidosis". Rheumatic Disease Clinics of North America. 42 (1): 119–35, ix. doi:10.1016/j.rdc.2015.08.012. PMID 26611555.
  10. ^ a b "Global, regional, and national incidence, prevalence, and years lived with disability for 310 diseases and injuries, 1990–2015: a systematic analysis for the Global Burden of Disease Study 2015". Lancet. 388 (10053): 1545–1602. October 2016. doi:10.1016/S0140-6736(16)31678-6. PMC 5055577. PMID 27733282.
  11. ^ a b "Global, regional, and national life expectancy, all-cause mortality, and cause-specific mortality for 249 causes of death, 1980–2015: a systematic analysis for the Global Burden of Disease Study 2015". Lancet. 388 (10053): 1459–1544. October 2016. doi:10.1016/S0140-6736(16)31012-1. PMC 5388903. PMID 27733281.
  12. ^ Baughman RP, Culver DA, Judson MA (March 2011). "A concise review of pulmonary sarcoidosis". American Journal of Respiratory and Critical Care Medicine. 183 (5): 573–81. doi:10.1164/rccm.201006-0865CI. PMC 3081278. PMID 21037016.
  13. ^ "What Causes Sarcoidosis?". NHLBI. June 14, 2013. Archived from the original on 6 April 2016. Retrieved 28 March 2016.
  14. ^ a b Kobak S (October 2015). "Sarcoidosis: a rheumatologist's perspective". Therapeutic Advances in Musculoskeletal Disease. 7 (5): 196–205. doi:10.1177/1759720x15591310. PMC 4572362. PMID 26425148.
  15. ^ James DG, Sharma OP (September 2002). "From Hutchinson to now: a historical glimpse" (PDF). Current Opinion in Pulmonary Medicine. 8 (5): 416–23. doi:10.1097/00063198-200209000-00013. PMID 12172446. Archived (PDF) from the original on 2016-03-04.