Systematic review: macrophage activation syndrome in inflammatory bowel disease

Abstract

SUMMARY BackgroundRecently, 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.

Infection is the major trigger of hemophagocytic syndrome in adult patients treated with biological therapies

“…In addition, we found a specific microorganism distribution according to the underlying disease: 80% of viral infections were reported in patients with CD and 82% of non-viral infections in patients with inflammatory arthritis. In patients with inflammatory bowel disease, HLH has been associated more frequently with viral infections than with bacterial infections [50,51] with a reported overall mortality of viral-related HLH of 30% [52]. In patients with inflammatory arthritis, the predominant role of intracellular bacteria can be linked to the predominant use of anti-TNF agents, which have been closely associated with an enhanced risk of tuberculosis and other infections by intracellular bacteria [48,49].…”

Lymphoma Presenting as Secondary HLH: A Review With a Tale of Two Cases

“…13,16 Autoimmune diseases that have been associated with HLH include systemic lupus erythematosus, rheumatoid arthritis, Crohn’s disease, scleroderma, and Sjogren disease especially when these patients are given immunosuppressive therapy. 17–20 In a retrospective study by Fries et al, 50 cases of macrophage activation syndrome (MAS), which is considered equivalent to secondary HLH, were found to be associated with inflammatory bowel disease, with most patients suffering from Crohn’s disease. All except 5 (10%) received immunosuppressive therapy and viral infections especially EBV and CMV were detected in 39 patients (78%).…”

“…Thiopurines, specifically azathioprine or mercaptopurine, were most commonly associated with the development of HLH, but anti-tumor necrosis factor (TNF) therapy, specifically infliximab, has also been reported. 20 …”

En mann i 30-årene med ulcerøs kolitt og pancytopeni

“…Man antar at kombinasjonen av kronisk inflammasjon og immunsuppresjon, primaert i form av tiopuriner, men også kombinasjon av tiopuriner og biologiske legemidler eller steroider, kan disponere for utvikling av hemofagocytisk lymfohistiocytose (18,19). I forbindelse med akutt virusinfeksjon, som cytomegalovirus eller Epstein-Barr-virus, kan azatioprin bidra til å utløse sekundaer hemofagocytisk lymfohistiocytose hos pasienter med inflammatorisk tarmsykdom.…”