Weathering a Macrophage Storm

In rheumatology circles, macrophage activation syndrome (MAS) describes a potentially life-threatening complication of systemic inflammatory disorders, frequently making matters worse for those with systemic juvenile idiopathic arthritis (sJIA) and its adult counterpart, adult-onset Still disease (AOSD). A dysfunctional immune response results in continuous activation and expansion of T lymphocytes and macrophages, leading to an overproduction of proinflammatory cytokines (a cytokine storm) and resulting in multiorgan dysfunction. Like the related disorder, secondary hemophagocytic lymphohistiocytosis (HLH), MAS features include high, unremitting fever, hyperferritinemia, hepatosplenomegaly, lymphadenopathy, pancytopenia, and hypofibrinogenemia. Other laboratory abnormalities include elevated liver enzymes, D-dimers, lactate dehydrogenase, and triglycerides (TGC). Soluble interleukin 2 receptor α (sCD25) may be elevated, but testing is often not available at an on-site laboratory and therefore is not routinely done at the time of diagnosis1,2,3,4. Histopathology often reveals characteristic increased hemophagocytic activity in the bone marrow (and other tissues), with positive CD163 (histiocyte) staining, although this feature is often not present in initial stages and is neither highly sensitive nor specific for MAS5,6. Most commonly studied as a complication of sJIA, the reported prevalence of MAS is estimated to be about 10%; however, reports suggest subclinical MAS may be present in 30%–40% of patients with sJIA7,8. There are several diagnostic challenges in the early recognition of MAS, particularly in distinguishing it from a flare of sJIA or AOSD. Moreover, there is no single pathognomonic feature of MAS, and many clinical features and laboratory abnormalities overlap with those of systemic inflammatory disorders, making it difficult to distinguish the underlying disease from the life-threatening comorbidity1,2,3,4. Further, until very recently, there were no universal validated criteria to aid in diagnosis. However, patient data–based classification criteria … Address correspondence to Dr. R.Q. Cron, Children’s of Alabama, Division of Rheumatology, 1600 7th Ave. S., CPP #M10, Birmingham, Alabama 35233-1711, USA; E-mail: rcron{at}peds.uab.edu

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