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Macrophage activation syndrome

MedGen UID:
242753
Concept ID:
C1096155
Disease or Syndrome
Synonyms: Macrophage Activation Syndrome; Syndrome, Macrophage Activation
SNOMED CT: Macrophage activation syndrome (430478003)
 
Orphanet: ORPHA158061

Definition

A complication of rheumatic disease that is caused by excessive activation and uncontrolled proliferation of T lymphocytes and well-differentiated macrophages. It is characteized by fever, pancytopenia, liver insuffficiency, coagulopathy and neurologic symptoms.(NICHD) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMacrophage activation syndrome
Follow this link to review classifications for Macrophage activation syndrome in Orphanet.

Recent clinical studies

Etiology

Ravelli A, Minoia F, Davì S, Horne A, Bovis F, Pistorio A, Aricò M, Avcin T, Behrens EM, De Benedetti F, Filipovic L, Grom AA, Henter JI, Ilowite NT, Jordan MB, Khubchandani R, Kitoh T, Lehmberg K, Lovell DJ, Miettunen P, Nichols KE, Ozen S, Pachlopnik Schmid J, Ramanan AV, Russo R, Schneider R, Sterba G, Uziel Y, Wallace C, Wouters C, Wulffraat N, Demirkaya E, Brunner HI, Martini A, Ruperto N, Cron RQ; Paediatric Rheumatology International Trials Organisation; Childhood Arthritis and Rheumatology Research Alliance; Pediatric Rheumatology Collaborative Study Group; Histiocyte Society
Ann Rheum Dis 2016 Mar;75(3):481-9. doi: 10.1136/annrheumdis-2015-208982. PMID: 26865703
Shakoory B, Carcillo JA, Chatham WW, Amdur RL, Zhao H, Dinarello CA, Cron RQ, Opal SM
Crit Care Med 2016 Feb;44(2):275-81. doi: 10.1097/CCM.0000000000001402. PMID: 26584195
Shimizu M, Nakagishi Y, Inoue N, Mizuta M, Ko G, Saikawa Y, Kubota T, Yamasaki Y, Takei S, Yachie A
Clin Immunol 2015 Oct;160(2):277-81. Epub 2015 Jun 20 doi: 10.1016/j.clim.2015.06.005. [Epub ahead of print] PMID: 26101092
Minoia F, Davì S, Horne A, Bovis F, Demirkaya E, Akikusa J, Ayaz NA, Al-Mayouf SM, Barone P, Bica B, Bolt I, Breda L, De Cunto C, Enciso S, Gallizzi R, Griffin T, Hennon T, Horneff G, Jeng M, Kapovic AM, Lipton JM, Magni Manzoni S, Rumba-Rozenfelde I, Magalhaes CS, Sewairi WM, Stine KC, Vougiouka O, Weaver LK, Davidsone Z, De Inocencio J, Ioseliani M, Lattanzi B, Tezer H, Buoncompagni A, Picco P, Ruperto N, Martini A, Cron RQ, Ravelli A; Pediatric Rheumatology International Trials Organization; Childhood Arthritis and Rheumatology Research Alliance; Pediatric Rheumatology Collaborative Study Group; Histiocyte Society
J Rheumatol 2015 Jun;42(6):994-1001. Epub 2015 Apr 15 doi: 10.3899/jrheum.141261. [Epub ahead of print] PMID: 25877504
Kostik MM, Dubko MF, Masalova VV, Snegireva LS, Kornishina TL, Chikova IA, Likhacheva TS, Isupova EA, Glebova NI, Kuchinskaya EM, Balbotkina EV, Buchinskaya NV, Kalashnikova OV, Chasnyk VG
Semin Arthritis Rheum 2015 Feb;44(4):417-22. Epub 2014 Sep 11 doi: 10.1016/j.semarthrit.2014.09.004. [Epub ahead of print] PMID: 25300700

Diagnosis

Ravelli A, Minoia F, Davì S, Horne A, Bovis F, Pistorio A, Aricò M, Avcin T, Behrens EM, De Benedetti F, Filipovic L, Grom AA, Henter JI, Ilowite NT, Jordan MB, Khubchandani R, Kitoh T, Lehmberg K, Lovell DJ, Miettunen P, Nichols KE, Ozen S, Pachlopnik Schmid J, Ramanan AV, Russo R, Schneider R, Sterba G, Uziel Y, Wallace C, Wouters C, Wulffraat N, Demirkaya E, Brunner HI, Martini A, Ruperto N, Cron RQ; Paediatric Rheumatology International Trials Organisation; Childhood Arthritis and Rheumatology Research Alliance; Pediatric Rheumatology Collaborative Study Group; Histiocyte Society
Ann Rheum Dis 2016 Mar;75(3):481-9. doi: 10.1136/annrheumdis-2015-208982. PMID: 26865703
Cron RQ, Davi S, Minoia F, Ravelli A
Expert Rev Clin Immunol 2015;11(9):1043-53. Epub 2015 Jun 16 doi: 10.1586/1744666X.2015.1058159. [Epub ahead of print] PMID: 26082353
Minoia F, Davì S, Horne A, Bovis F, Demirkaya E, Akikusa J, Ayaz NA, Al-Mayouf SM, Barone P, Bica B, Bolt I, Breda L, De Cunto C, Enciso S, Gallizzi R, Griffin T, Hennon T, Horneff G, Jeng M, Kapovic AM, Lipton JM, Magni Manzoni S, Rumba-Rozenfelde I, Magalhaes CS, Sewairi WM, Stine KC, Vougiouka O, Weaver LK, Davidsone Z, De Inocencio J, Ioseliani M, Lattanzi B, Tezer H, Buoncompagni A, Picco P, Ruperto N, Martini A, Cron RQ, Ravelli A; Pediatric Rheumatology International Trials Organization; Childhood Arthritis and Rheumatology Research Alliance; Pediatric Rheumatology Collaborative Study Group; Histiocyte Society
J Rheumatol 2015 Jun;42(6):994-1001. Epub 2015 Apr 15 doi: 10.3899/jrheum.141261. [Epub ahead of print] PMID: 25877504
Kostik MM, Dubko MF, Masalova VV, Snegireva LS, Kornishina TL, Chikova IA, Likhacheva TS, Isupova EA, Glebova NI, Kuchinskaya EM, Balbotkina EV, Buchinskaya NV, Kalashnikova OV, Chasnyk VG
Semin Arthritis Rheum 2015 Feb;44(4):417-22. Epub 2014 Sep 11 doi: 10.1016/j.semarthrit.2014.09.004. [Epub ahead of print] PMID: 25300700
Wang W, Gong F, Zhu W, Fu S, Zhang Q
Semin Arthritis Rheum 2015 Feb;44(4):405-10. Epub 2014 Aug 7 doi: 10.1016/j.semarthrit.2014.07.007. [Epub ahead of print] PMID: 25200945

Therapy

Shakoory B, Carcillo JA, Chatham WW, Amdur RL, Zhao H, Dinarello CA, Cron RQ, Opal SM
Crit Care Med 2016 Feb;44(2):275-81. doi: 10.1097/CCM.0000000000001402. PMID: 26584195
Grom AA, Ilowite NT, Pascual V, Brunner HI, Martini A, Lovell D, Ruperto N; Paediatric Rheumatology International Trials Organisation and the Pediatric Rheumatology Collaborative Study Group, Leon K, Lheritier K, Abrams K
Arthritis Rheumatol 2016 Jan;68(1):218-28. doi: 10.1002/art.39407. PMID: 26314396
Wagner-Weiner L
Pediatr Ann 2015 Jun;44(6):e142-7. doi: 10.3928/00904481-20150611-09. PMID: 26114369
Green GL, Aframian A, Vinayakam P, Cornell MS
J Pediatr Orthop B 2015 Jul;24(4):330-5. doi: 10.1097/BPB.0000000000000156. PMID: 25714936
Park JH, Kim SH, Kim HJ, Lee SJ, Jeong DC, Kim SY
J Perinatol 2015 Feb;35(2):158-60. doi: 10.1038/jp.2014.207. PMID: 25627282

Prognosis

Shimizu M, Nakagishi Y, Inoue N, Mizuta M, Ko G, Saikawa Y, Kubota T, Yamasaki Y, Takei S, Yachie A
Clin Immunol 2015 Oct;160(2):277-81. Epub 2015 Jun 20 doi: 10.1016/j.clim.2015.06.005. [Epub ahead of print] PMID: 26101092
Minoia F, Davì S, Horne A, Bovis F, Demirkaya E, Akikusa J, Ayaz NA, Al-Mayouf SM, Barone P, Bica B, Bolt I, Breda L, De Cunto C, Enciso S, Gallizzi R, Griffin T, Hennon T, Horneff G, Jeng M, Kapovic AM, Lipton JM, Magni Manzoni S, Rumba-Rozenfelde I, Magalhaes CS, Sewairi WM, Stine KC, Vougiouka O, Weaver LK, Davidsone Z, De Inocencio J, Ioseliani M, Lattanzi B, Tezer H, Buoncompagni A, Picco P, Ruperto N, Martini A, Cron RQ, Ravelli A; Pediatric Rheumatology International Trials Organization; Childhood Arthritis and Rheumatology Research Alliance; Pediatric Rheumatology Collaborative Study Group; Histiocyte Society
J Rheumatol 2015 Jun;42(6):994-1001. Epub 2015 Apr 15 doi: 10.3899/jrheum.141261. [Epub ahead of print] PMID: 25877504
Rigante D, Emmi G, Fastiggi M, Silvestri E, Cantarini L
Clin Rheumatol 2015 Aug;34(8):1333-9. Epub 2015 Apr 8 doi: 10.1007/s10067-015-2923-0. [Epub ahead of print] PMID: 25846831
Green GL, Aframian A, Vinayakam P, Cornell MS
J Pediatr Orthop B 2015 Jul;24(4):330-5. doi: 10.1097/BPB.0000000000000156. PMID: 25714936
Kostik MM, Dubko MF, Masalova VV, Snegireva LS, Kornishina TL, Chikova IA, Likhacheva TS, Isupova EA, Glebova NI, Kuchinskaya EM, Balbotkina EV, Buchinskaya NV, Kalashnikova OV, Chasnyk VG
Semin Arthritis Rheum 2015 Feb;44(4):417-22. Epub 2014 Sep 11 doi: 10.1016/j.semarthrit.2014.09.004. [Epub ahead of print] PMID: 25300700

Clinical prediction guides

Cifaldi L, Prencipe G, Caiello I, Bracaglia C, Locatelli F, De Benedetti F, Strippoli R
Arthritis Rheumatol 2015 Nov;67(11):3037-46. doi: 10.1002/art.39295. PMID: 26251193
Shimizu M, Nakagishi Y, Inoue N, Mizuta M, Ko G, Saikawa Y, Kubota T, Yamasaki Y, Takei S, Yachie A
Clin Immunol 2015 Oct;160(2):277-81. Epub 2015 Jun 20 doi: 10.1016/j.clim.2015.06.005. [Epub ahead of print] PMID: 26101092
Rigante D, Emmi G, Fastiggi M, Silvestri E, Cantarini L
Clin Rheumatol 2015 Aug;34(8):1333-9. Epub 2015 Apr 8 doi: 10.1007/s10067-015-2923-0. [Epub ahead of print] PMID: 25846831
Ruscitti P, Cipriani P, Di Benedetto P, Ciccia F, Liakouli V, Carubbi F, Berardicurti O, Rizzo A, Triolo G, Giacomelli R
Autoimmun Rev 2015 May;14(5):429-37. Epub 2015 Jan 17 doi: 10.1016/j.autrev.2015.01.004. [Epub ahead of print] PMID: 25599955
Kostik MM, Dubko MF, Masalova VV, Snegireva LS, Kornishina TL, Chikova IA, Likhacheva TS, Isupova EA, Glebova NI, Kuchinskaya EM, Balbotkina EV, Buchinskaya NV, Kalashnikova OV, Chasnyk VG
Semin Arthritis Rheum 2015 Feb;44(4):417-22. Epub 2014 Sep 11 doi: 10.1016/j.semarthrit.2014.09.004. [Epub ahead of print] PMID: 25300700

Recent systematic reviews

Ravelli A, Minoia F, Davì S, Horne A, Bovis F, Pistorio A, Aricò M, Avcin T, Behrens EM, De Benedetti F, Filipovic L, Grom AA, Henter JI, Ilowite NT, Jordan MB, Khubchandani R, Kitoh T, Lehmberg K, Lovell DJ, Miettunen P, Nichols KE, Ozen S, Pachlopnik Schmid J, Ramanan AV, Russo R, Schneider R, Sterba G, Uziel Y, Wallace C, Wouters C, Wulffraat N, Demirkaya E, Brunner HI, Martini A, Ruperto N, Cron RQ; Paediatric Rheumatology International Trials Organisation; Childhood Arthritis and Rheumatology Research Alliance; Pediatric Rheumatology Collaborative Study Group; Histiocyte Society
Ann Rheum Dis 2016 Mar;75(3):481-9. doi: 10.1136/annrheumdis-2015-208982. PMID: 26865703
Correll CK, Binstadt BA
Pediatr Res 2014 Jan;75(1-2):176-83. Epub 2013 Nov 8 doi: 10.1038/pr.2013.187. [Epub ahead of print] PMID: 24213625
Fries W, Cottone M, Cascio A
Aliment Pharmacol Ther 2013 Jun;37(11):1033-45. Epub 2013 Apr 8 doi: 10.1111/apt.12305. [Epub ahead of print] PMID: 23565820
de Boysson H, Février J, Nicolle A, Auzary C, Geffray L
Clin Rheumatol 2013 Jan;32(1):141-7. Epub 2012 Oct 30 doi: 10.1007/s10067-012-2105-2. [Epub ahead of print] PMID: 23108887
Cascio A, Pernice LM, Barberi G, Delfino D, Biondo C, Beninati C, Mancuso G, Rodriguez-Morales AJ, Iaria C
Eur Rev Med Pharmacol Sci 2012 Oct;16(10):1324-37. PMID: 23104648

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