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Eosinophilia myalgia syndrome(EMS)

MedGen UID:
38987
Concept ID:
C0085179
Disease or Syndrome
Synonyms: EMS; L-tryptophan induced EMS; Severe muscle pain and abnormally high eosinophils; Syndrome with inflammatory and autoimmune components that affect the skin, fascia, muscle, nerve, blood vessels, lung, and heart
SNOMED CT: EMS - Eosinophilia myalgia syndrome (95416007); Eosinophilia myalgia syndrome (95416007)
 
Monarch Initiative: MONDO:0004941

Definition

A complex systemic syndrome with inflammatory and autoimmune components that affect the skin, fascia, muscle, nerve, blood vessels, lung, and heart. Diagnostic features generally include eosinophilia, myalgia severe enough to limit usual activities of daily living, and the absence of coexisting infectious, autoimmune or other conditions that may induce eosinophilia. Biopsy of affected tissue reveals a microangiopathy associated with diffuse inflammation involving connective tissue. (From Spitzer et al., J Rheumatol Suppl 1996 Oct;46:73-9; Blackburn wd, Semin Arthritis Rheum 1997 Jun;26(6):788-93) [from MONDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEosinophilia myalgia syndrome

Professional guidelines

PubMed

Hertzman PA
J Rheumatol Suppl 1996 Oct;46:7-12. PMID: 8895176
Hertzman PA, Clauw DJ, Kaufman LD, Varga J, Silver RM, Thacker HL, Mease P, Espinoza LR, Pincus T
Ann Intern Med 1995 Jun 1;122(11):851-5. doi: 10.7326/0003-4819-122-11-199506010-00008. PMID: 7741371
N Engl J Med 1990 Aug 9;323(6):417-8. doi: 10.1056/NEJM199008093230613. PMID: 2370894

Recent clinical studies

Etiology

Rangan C, Barceloux DG
Dis Mon 2009 May;55(5):263-91. doi: 10.1016/j.disamonth.2009.01.003. PMID: 19362176
Foti R, Leonardi R, Rondinone R, Di Gangi M, Leonetti C, Canova M, Doria A
Autoimmun Rev 2008 Feb;7(4):331-9. Epub 2008 Jan 11 doi: 10.1016/j.autrev.2007.12.004. PMID: 18295739
Kuncl RW, George EB
Curr Opin Neurol 1993 Oct;6(5):695-704. doi: 10.1097/00019052-199310000-00004. PMID: 8293140
Belongia EA, Mayeno AN, Osterholm MT
Annu Rev Nutr 1992;12:235-56. doi: 10.1146/annurev.nu.12.070192.001315. PMID: 1503805
Le Quintrec JS, Le Quintrec JL
Baillieres Clin Rheumatol 1991 Apr;5(1):21-38. doi: 10.1016/s0950-3579(05)80294-8. PMID: 2070426

Diagnosis

Tamaki H, Chatterjee S, Langford CA
Immunol Allergy Clin North Am 2015 Aug;35(3):453-76. Epub 2015 Jun 17 doi: 10.1016/j.iac.2015.05.001. PMID: 26209895
Ter Poorten MC, Thiers BH
Dermatol Clin 2002 Jul;20(3):421-33, vi. doi: 10.1016/s0733-8635(02)00008-6. PMID: 12170876
Blackburn WD Jr
Semin Arthritis Rheum 1997 Jun;26(6):788-93. doi: 10.1016/s0049-0172(97)80022-4. PMID: 9213377
DeSpain JD, Swinfard RW
Dermatol Clin 1992 Jan;10(1):1-18. PMID: 1730164
Kazura JW
Cleve Clin J Med 1991 May-Jun;58(3):267-70. doi: 10.3949/ccjm.58.3.267. PMID: 1893558

Therapy

Turner EH, Loftis JM, Blackwell AD
Pharmacol Ther 2006 Mar;109(3):325-38. Epub 2005 Jul 14 doi: 10.1016/j.pharmthera.2005.06.004. PMID: 16023217
Blackburn WD Jr
Semin Arthritis Rheum 1997 Jun;26(6):788-93. doi: 10.1016/s0049-0172(97)80022-4. PMID: 9213377
Kazura JW
Cleve Clin J Med 1991 May-Jun;58(3):267-70. doi: 10.3949/ccjm.58.3.267. PMID: 1893558
Silver RM
Arch Dermatol 1991 Aug;127(8):1214-6. PMID: 1863082
Hess EV
Curr Opin Rheumatol 1991 Oct;3(5):809-14. doi: 10.1097/00002281-199110000-00010. PMID: 1751313

Prognosis

Foti R, Leonardi R, Rondinone R, Di Gangi M, Leonetti C, Canova M, Doria A
Autoimmun Rev 2008 Feb;7(4):331-9. Epub 2008 Jan 11 doi: 10.1016/j.autrev.2007.12.004. PMID: 18295739
Philen RM, Posada M
Semin Arthritis Rheum 1993 Oct;23(2):104-24. doi: 10.1016/s0049-0172(05)80017-4. PMID: 8266108
Sack KE, Criswell LA
South Med J 1992 Sep;85(9):878-82. doi: 10.1097/00007611-199209000-00005. PMID: 1523446
Jimenez SA, Varga J
Curr Opin Rheumatol 1991 Dec;3(6):986-94. doi: 10.1097/00002281-199112000-00015. PMID: 1772754
Auerbach SB, Falk H
Cleve Clin J Med 1991 May-Jun;58(3):215-7. doi: 10.3949/ccjm.58.3.215. PMID: 1654229

Clinical prediction guides

Zhang H, Yu P, Xiang ML, Li XB, Kong WB, Ma JY, Wang JL, Zhang JP, Zhang J
Med Biol Eng Comput 2016 Mar;54(2-3):361-9. Epub 2015 Jun 5 doi: 10.1007/s11517-015-1321-8. PMID: 26044554
Blackburn WD Jr
Semin Arthritis Rheum 1997 Jun;26(6):788-93. doi: 10.1016/s0049-0172(97)80022-4. PMID: 9213377
Varga J, Jimenez SA, Uitto J
J Invest Dermatol 1993 Jan;100(1):97S-105S. doi: 10.1111/1523-1747.ep12356368. PMID: 8423409
Kamb ML, Murphy JJ, Jones JL, Caston JC, Nederlof K, Horney LF, Swygert LA, Falk H, Kilbourne EM
JAMA 1992 Jan 1;267(1):77-82. doi: 10.1001/jama.267.1.77. PMID: 1727200
Milburn DS, Myers CW
DICP 1991 Nov;25(11):1259-62. doi: 10.1177/106002809102501116. PMID: 1763543

Recent systematic reviews

Shaw K, Turner J, Del Mar C
Cochrane Database Syst Rev 2002;(1):CD003198. doi: 10.1002/14651858.CD003198. PMID: 11869656
Shaw K, Turner J, Del Mar C
Cochrane Database Syst Rev 2001;2002(3):CD003198. doi: 10.1002/14651858.CD003198. PMID: 11687048Free PMC Article

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