Systematic review: macrophage activation syndrome in inflammatory bowel disease. Issue 11 (8th April 2013)

• Record Type: Journal Article
• Authors: Fries, W.; Cottone, M.; Cascio, A.
• Is Part Of: Alimentary pharmacology & therapeutics. Volume 37:Issue 11(2013)
• Page Start: 1033
• Page End: 1045
• Publication Date: 2013-04-08
• Subjects: Digestive organs -- Diseases -- Treatment -- Periodicals; Digestive organs -- Effect of drugs on -- Periodicals; Gastrointestinal system -- Diseases -- Treatment -- Periodicals; Gastrointestinal system -- Effect of drugs on -- Periodicals; 615.73
• DOI: 10.1111/apt.12305
• Languages: English
• ISSNs: 0269-2813

Abstract

<abstract abstract-type="main" id="apt12305-abs-0001"> <title>Summary</title> <sec id="apt12305-sec-0001" sec-type="section"> <title>Background</title> <p>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.</p> </sec> <sec id="apt12305-sec-0002" sec-type="section"> <title>Aim</title> <p>To identify all reports on MAS in IBD and to establish data on triggering agents, immunosuppression leading to MAS, and mortality.</p> </sec> <sec id="apt12305-sec-0003" sec-type="section"> <title>Methods</title> <p>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).</p> </sec> <sec id="apt12305-sec-0004" sec-type="section"> <title>Results</title> <p>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%.</p> </sec> <sec id="apt12305-sec-0005" sec-type="section"> <title>Conclusions</title> <p>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.</p> </sec> </abstract>