Systematic review: macrophage activationsyndrome in inflammatory bowel disease

Mario Cottone, Antonio Cascio, Cascio, Walter Fries

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32 Citazioni (Scopus)

Abstract

BACKGROUND: Recently, there have been increasingly frequent reports on the occurrence of macrophage activation syndrome (MAS) in patients with inflammatory bowel disease (IBD). Clinically, MAS is characterized mainly by fever, hepatosplenomegaly, cytopenia, and elevated circulating ferritin and CD25. Mortality, even if diagnosed rapidly, is high.AIM: To identify all reports on MAS in IBD and to establish data on triggering agents, immunosuppression leading to MAS, and mortality.METHODS: A language unrestricted search on Pubmed and Scopus relating to the past 30 years was carried out by matching the following search-terms: h(a)emophagocytic lymphohistiocytosis OR h(a)emophagocytic lymphohistiocytic syndrome OR macrophage activation syndrome OR opportunistic infections OR cytomegalovirus OR Epstein-Barr virus AND Crohn's disease OR ulcerative colitis OR inflammatory bowel disease(s).RESULTS: Fifty cases were identified with an overall mortality of 30%. Virus-related MAS associated with cytomegalovirus or Epstein-Barr virus infections represents the main type of MAS, but in isolated cases bacterial infections precipitated the syndrome. In four cases (8%), a lymphoma was present at the time of MAS diagnosis or developed shortly thereafter. Thiopurine monotherapy was given before MAS onset in 56% of the patients, whereas multiple immunosuppression, including biologics, was administered to 24%.CONCLUSIONS: In IBD patients, the syndrome appears to be triggered by infections, but genetic susceptibility may contribute to its development. Since immunosuppressive therapy represents the backbone of therapeutic interventions in IBD, with the risk of new, or the reactivation of latent infections, even more frequent cases of macrophage activation syndrome may be expected.
Lingua originaleEnglish
pagine (da-a)1033-1045
Numero di pagine13
RivistaALIMENTARY PHARMACOLOGY & THERAPEUTICS
Volume37
Stato di pubblicazionePublished - 2013

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Macrophage Activation Syndrome
Inflammatory Bowel Diseases
Macrophages
Cytomegalovirus
Immunosuppression
Mortality
Epstein-Barr Virus Infections
Opportunistic Infections
Virus Diseases
Genetic Predisposition to Disease
Immunosuppressive Agents
Ferritins
Infection
Biological Products
Human Herpesvirus 4
Ulcerative Colitis
Bacterial Infections
PubMed
Crohn Disease
Lymphoma

All Science Journal Classification (ASJC) codes

  • Hepatology
  • Gastroenterology
  • Pharmacology (medical)

Cita questo

@article{326b0bf253114754ba5d782a7daed371,
title = "Systematic review: macrophage activationsyndrome in inflammatory bowel disease",
abstract = "BACKGROUND: Recently, there have been increasingly frequent reports on the occurrence of macrophage activation syndrome (MAS) in patients with inflammatory bowel disease (IBD). Clinically, MAS is characterized mainly by fever, hepatosplenomegaly, cytopenia, and elevated circulating ferritin and CD25. Mortality, even if diagnosed rapidly, is high.AIM: To identify all reports on MAS in IBD and to establish data on triggering agents, immunosuppression leading to MAS, and mortality.METHODS: A language unrestricted search on Pubmed and Scopus relating to the past 30 years was carried out by matching the following search-terms: h(a)emophagocytic lymphohistiocytosis OR h(a)emophagocytic lymphohistiocytic syndrome OR macrophage activation syndrome OR opportunistic infections OR cytomegalovirus OR Epstein-Barr virus AND Crohn's disease OR ulcerative colitis OR inflammatory bowel disease(s).RESULTS: Fifty cases were identified with an overall mortality of 30{\%}. Virus-related MAS associated with cytomegalovirus or Epstein-Barr virus infections represents the main type of MAS, but in isolated cases bacterial infections precipitated the syndrome. In four cases (8{\%}), a lymphoma was present at the time of MAS diagnosis or developed shortly thereafter. Thiopurine monotherapy was given before MAS onset in 56{\%} of the patients, whereas multiple immunosuppression, including biologics, was administered to 24{\%}.CONCLUSIONS: In IBD patients, the syndrome appears to be triggered by infections, but genetic susceptibility may contribute to its development. Since immunosuppressive therapy represents the backbone of therapeutic interventions in IBD, with the risk of new, or the reactivation of latent infections, even more frequent cases of macrophage activation syndrome may be expected.",
author = "Mario Cottone and Antonio Cascio and Cascio and Walter Fries",
year = "2013",
language = "English",
volume = "37",
pages = "1033--1045",
journal = "ALIMENTARY PHARMACOLOGY & THERAPEUTICS",
issn = "0269-2813",

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TY - JOUR

T1 - Systematic review: macrophage activationsyndrome in inflammatory bowel disease

AU - Cottone, Mario

AU - Cascio, Antonio

AU - Cascio, null

AU - Fries, Walter

PY - 2013

Y1 - 2013

N2 - BACKGROUND: Recently, there have been increasingly frequent reports on the occurrence of macrophage activation syndrome (MAS) in patients with inflammatory bowel disease (IBD). Clinically, MAS is characterized mainly by fever, hepatosplenomegaly, cytopenia, and elevated circulating ferritin and CD25. Mortality, even if diagnosed rapidly, is high.AIM: To identify all reports on MAS in IBD and to establish data on triggering agents, immunosuppression leading to MAS, and mortality.METHODS: A language unrestricted search on Pubmed and Scopus relating to the past 30 years was carried out by matching the following search-terms: h(a)emophagocytic lymphohistiocytosis OR h(a)emophagocytic lymphohistiocytic syndrome OR macrophage activation syndrome OR opportunistic infections OR cytomegalovirus OR Epstein-Barr virus AND Crohn's disease OR ulcerative colitis OR inflammatory bowel disease(s).RESULTS: Fifty cases were identified with an overall mortality of 30%. Virus-related MAS associated with cytomegalovirus or Epstein-Barr virus infections represents the main type of MAS, but in isolated cases bacterial infections precipitated the syndrome. In four cases (8%), a lymphoma was present at the time of MAS diagnosis or developed shortly thereafter. Thiopurine monotherapy was given before MAS onset in 56% of the patients, whereas multiple immunosuppression, including biologics, was administered to 24%.CONCLUSIONS: In IBD patients, the syndrome appears to be triggered by infections, but genetic susceptibility may contribute to its development. Since immunosuppressive therapy represents the backbone of therapeutic interventions in IBD, with the risk of new, or the reactivation of latent infections, even more frequent cases of macrophage activation syndrome may be expected.

AB - BACKGROUND: Recently, there have been increasingly frequent reports on the occurrence of macrophage activation syndrome (MAS) in patients with inflammatory bowel disease (IBD). Clinically, MAS is characterized mainly by fever, hepatosplenomegaly, cytopenia, and elevated circulating ferritin and CD25. Mortality, even if diagnosed rapidly, is high.AIM: To identify all reports on MAS in IBD and to establish data on triggering agents, immunosuppression leading to MAS, and mortality.METHODS: A language unrestricted search on Pubmed and Scopus relating to the past 30 years was carried out by matching the following search-terms: h(a)emophagocytic lymphohistiocytosis OR h(a)emophagocytic lymphohistiocytic syndrome OR macrophage activation syndrome OR opportunistic infections OR cytomegalovirus OR Epstein-Barr virus AND Crohn's disease OR ulcerative colitis OR inflammatory bowel disease(s).RESULTS: Fifty cases were identified with an overall mortality of 30%. Virus-related MAS associated with cytomegalovirus or Epstein-Barr virus infections represents the main type of MAS, but in isolated cases bacterial infections precipitated the syndrome. In four cases (8%), a lymphoma was present at the time of MAS diagnosis or developed shortly thereafter. Thiopurine monotherapy was given before MAS onset in 56% of the patients, whereas multiple immunosuppression, including biologics, was administered to 24%.CONCLUSIONS: In IBD patients, the syndrome appears to be triggered by infections, but genetic susceptibility may contribute to its development. Since immunosuppressive therapy represents the backbone of therapeutic interventions in IBD, with the risk of new, or the reactivation of latent infections, even more frequent cases of macrophage activation syndrome may be expected.

UR - http://hdl.handle.net/10447/73448

M3 - Article

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JO - ALIMENTARY PHARMACOLOGY & THERAPEUTICS

JF - ALIMENTARY PHARMACOLOGY & THERAPEUTICS

SN - 0269-2813

ER -