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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
  • CROGMacrophage activation syndrome

Recent clinical studies

Etiology

Minoia F, Davì S, Horne A, Demirkaya E, Bovis F, Li C, Lehmberg K, Weitzman S, Insalaco A, Wouters C, Shenoi S, Espada G, Ozen S, Anton J, Khubchandani R, Russo R, Pal P, Kasapcopur O, Miettunen P, Maritsi D, Merino R, Shakoory B, Alessio M, Chasnyk V, Sanner H, Gao YJ, Huasong Z, Kitoh T, Avcin T, Fischbach M, Frosch M, Grom A, Huber A, Jelusic M, Sawhney S, Uziel Y, 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
Arthritis Rheumatol 2014 Nov;66(11):3160-9. doi: 10.1002/art.38802. PMID: 25077692
Davì S, Minoia F, Pistorio A, Horne A, Consolaro A, Rosina S, Bovis F, Cimaz R, Gamir ML, Ilowite NT, Kone-Paut I, Feitosa de Oliveira SK, McCurdy D, Silva CA, Sztajnbok F, Tsitsami E, Unsal E, Weiss JE, Wulffraat N, Abinun M, Aggarwal A, Apaz MT, Astigarraga I, Corona F, Cuttica R, D'Angelo G, Eisenstein EM, Hashad S, Lepore L, Mulaosmanovic V, Nielsen S, Prahalad S, Rigante D, Stanevicha V, Sterba G, Susic G, Takei S, Trauzeddel R, Zletni M, Ruperto N, Martini A, Cron RQ, Ravelli A; Paediatric Rheumatology International Trials Organisation, the Childhood Arthritis and Rheumatology Research Alliance, the Pediatric Rheumatology Collaborative Study Group, and the Histiocyte Society
Arthritis Rheumatol 2014 Oct;66(10):2871-80. doi: 10.1002/art.38769. PMID: 25044674
Weaver LK, Behrens EM
Curr Opin Rheumatol 2014 Sep;26(5):562-9. doi: 10.1097/BOR.0000000000000093. PMID: 25022357Free PMC Article
Schulert GS, Grom AA
Best Pract Res Clin Rheumatol 2014 Apr;28(2):277-92. doi: 10.1016/j.berh.2014.03.002. PMID: 24974063Free PMC Article
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

Diagnosis

Diwan AG, Shewale R, Iyer S, Nisal A, Agrawa P
J Assoc Physicians India 2014 Jan;62(1):58-61. PMID: 25327097
Vastert SJ, Prakken BJ
Nat Rev Rheumatol 2014 Nov;10(11):640-2. Epub 2014 Sep 9 doi: 10.1038/nrrheum.2014.143. [Epub ahead of print] PMID: 25201384
Weaver LK, Behrens EM
Curr Opin Rheumatol 2014 Sep;26(5):562-9. doi: 10.1097/BOR.0000000000000093. PMID: 25022357Free PMC Article
Poddighe D, Cavagna L, Brazzelli V, Bruni P, Marseglia GL
Autoimmun Rev 2014 Nov;13(11):1142-8. Epub 2014 May 29 doi: 10.1016/j.autrev.2014.05.009. [Epub ahead of print] PMID: 24879966
Reddy VV, Myles A, Cheekatla SS, Singh S, Aggarwal A
Int J Rheum Dis 2014 Mar;17(3):261-7. Epub 2014 Jan 13 doi: 10.1111/1756-185X.12196. [Epub ahead of print] PMID: 24417800

Therapy

Minoia F, Davì S, Horne A, Demirkaya E, Bovis F, Li C, Lehmberg K, Weitzman S, Insalaco A, Wouters C, Shenoi S, Espada G, Ozen S, Anton J, Khubchandani R, Russo R, Pal P, Kasapcopur O, Miettunen P, Maritsi D, Merino R, Shakoory B, Alessio M, Chasnyk V, Sanner H, Gao YJ, Huasong Z, Kitoh T, Avcin T, Fischbach M, Frosch M, Grom A, Huber A, Jelusic M, Sawhney S, Uziel Y, 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
Arthritis Rheumatol 2014 Nov;66(11):3160-9. doi: 10.1002/art.38802. PMID: 25077692
Weaver LK, Behrens EM
Curr Opin Rheumatol 2014 Sep;26(5):562-9. doi: 10.1097/BOR.0000000000000093. PMID: 25022357Free PMC Article
Schulert GS, Grom AA
Best Pract Res Clin Rheumatol 2014 Apr;28(2):277-92. doi: 10.1016/j.berh.2014.03.002. PMID: 24974063Free PMC Article
Mukherjee D, Pal P, Kundu R, Niyogi P
Indian Pediatr 2014 Feb;51(2):148-9. PMID: 24632699
Akagi K, Kawai T, Watanabe N, Yokoyama M, Arai K, Harayama S, Oana S, Onodera M
J Pediatr Hematol Oncol 2014 Apr;36(3):e169-72. doi: 10.1097/MPH.0b013e31828e5dae. PMID: 23652865

Prognosis

Minoia F, Davì S, Horne A, Demirkaya E, Bovis F, Li C, Lehmberg K, Weitzman S, Insalaco A, Wouters C, Shenoi S, Espada G, Ozen S, Anton J, Khubchandani R, Russo R, Pal P, Kasapcopur O, Miettunen P, Maritsi D, Merino R, Shakoory B, Alessio M, Chasnyk V, Sanner H, Gao YJ, Huasong Z, Kitoh T, Avcin T, Fischbach M, Frosch M, Grom A, Huber A, Jelusic M, Sawhney S, Uziel Y, 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
Arthritis Rheumatol 2014 Nov;66(11):3160-9. doi: 10.1002/art.38802. PMID: 25077692
Souabni L, Dridi L, Ben Abdelghani K, Kassab S, Chekili S, Laater A, Zakraoui L
Pan Afr Med J 2014;17:94. Epub 2014 Feb 7 doi: 10.11604/pamj.2014.17.94.3386. PMID: 25018831Free PMC Article
Akagi K, Kawai T, Watanabe N, Yokoyama M, Arai K, Harayama S, Oana S, Onodera M
J Pediatr Hematol Oncol 2014 Apr;36(3):e169-72. doi: 10.1097/MPH.0b013e31828e5dae. PMID: 23652865
Banse C, Vittecoq O, Benhamou Y, Gauthier-Prieur M, Lequerré T, Lévesque H
Joint Bone Spine 2013 Dec;80(6):653-5. Epub 2013 Jun 7 doi: 10.1016/j.jbspin.2013.04.011. [Epub ahead of print] PMID: 23751410
Gorelik M, Fall N, Altaye M, Barnes MG, Thompson SD, Grom AA, Hirsch R
J Rheumatol 2013 Jul;40(7):1191-9. Epub 2013 May 15 doi: 10.3899/jrheum.121131. [Epub ahead of print] PMID: 23678162Free PMC Article

Clinical prediction guides

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
Choudhary S, Berwal PK, Khichar S, Baid P
Indian Pediatr 2014 May;51(5):403-4. PMID: 24953588
Mukherjee D, Pal P, Kundu R, Niyogi P
Indian Pediatr 2014 Feb;51(2):148-9. PMID: 24632699
Reddy VV, Myles A, Cheekatla SS, Singh S, Aggarwal A
Int J Rheum Dis 2014 Mar;17(3):261-7. Epub 2014 Jan 13 doi: 10.1111/1756-185X.12196. [Epub ahead of print] PMID: 24417800
Gorelik M, Fall N, Altaye M, Barnes MG, Thompson SD, Grom AA, Hirsch R
J Rheumatol 2013 Jul;40(7):1191-9. Epub 2013 May 15 doi: 10.3899/jrheum.121131. [Epub ahead of print] PMID: 23678162Free PMC Article

Recent systematic reviews

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
Atteritano M, David A, Bagnato G, Beninati C, Frisina A, Iaria C, Bagnato G, Cascio A
Eur Rev Med Pharmacol Sci 2012 Oct;16(10):1414-24. PMID: 23104659
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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