Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders

Abstract

Conclusion-MAS is a rare and potentially fatal complication of childhood rheumatic disorders. Most of our patients were female, and most cases were preceded by infection. Bone marrow studies support the diagnosis. Deranged renal function may be a poor prognostic sign. Aggressive early therapy is essential. (Arch Dis Child 2001;85:421-426)

Cytokine profile in adult-onset Still's disease: Comparison with systemic juvenile idiopathic arthritis

“…Furthermore, 26% of patients with s-JIA in this study developed MAS. This was in line with recent reports [39,40] rather than with an older report [41].…”

Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment

“…70 A similar benefit was reported for high dose steroids in nine children with MAS (seven with sJIA) in England. 3 More recently, glucocorticoids, along with other immunosuppressive therapy, were reported to be effective in 13 children with sJIA in China 71 and in 6 children with sJIA in India. 72 Hence, high dose steroids are routinely used to treat MAS as part of sJIA.…”

Síndrome de ativação macrofágica associada com artrite idiopática juvenil sistêmica

“…21 Evidence suggests that MAS etiopathogenesis is associated with a hereditary defect in immunoregulation, predisposing sufferers to a histiocytic proliferation in response to specific triggering agents such as viruses, 15,22 autoimmune diseases, 6,[22][23][24][25][26][27][28] immunodeficiencies (such as during the accelerated phase of the Chédiak-Higashi syndrome) 3 and drugs. 7,10 In the majority of studies of JIA, the criteria for MAS diagnosis are not defined. Ravelli 4 performed a retrospective study of 88 patients with JIA (72 reported in published literature and 16 new Italian cases).…”

“…3,12 Macrophage activation syndrome can be triggered by viral infectious agents such as: varicella-zoster, 13 hepatitis A, 14 Epstein-Barr 8 and coxsackie B, 15 in addition to therapy with gold, acetylsalicylic acid and other nonsteroidal antiinflammatory drugs, 6,12 methrotrexate, 10 sulfasalazine and penicillamine. 7 The etiopathogenesis of MAS is unknown. The macrophage activation liberates proteases which activate plasminogen, leading to the formation of plasmin with fibrin degradation, triggering fibrinolysis and DIVC.…”

“…6 This syndrome occurs primarily with the systemic form of JIA, and the average period of JIA prior to MAS onset is from 4.2 to 4.8 years. 6,7 Other names have been used in published literature for this syndrome when associated with JIA, such as: reactive hemophagocytic syndrome, 5,8 hemophagocytic lymphohistiocytosis 2 or disseminated intravascular coagulation (DIVC) with liver failure. 9 Patients with MAS present prolonged high fever, hepatomegaly and splenomegaly, bleeding, generalized adenomegaly, rash, jaundice, can develop acute liver failure, coma, DIVC and multiple organ failure.…”