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2014 ; 26
(5
): 562-9
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Hyperinflammation, rather than hemophagocytosis, is the common link between
macrophage activation syndrome and hemophagocytic lymphohistiocytosis
#MMPMID25022357
Weaver LK
; Behrens EM
Curr Opin Rheumatol
2014[Sep]; 26
(5
): 562-9
PMID25022357
show ga
PURPOSE OF REVIEW: Macrophage activation syndrome is the rheumatic
disease-associated member of a group of hyperinflammatory syndromes characterized
by uncontrolled cytokine storm. In this review, we highlight recent publications
related to the pathoetiology of hyperinflammatory syndromes with an emphasis on
how this new knowledge will guide our diagnosis, treatment, and future research
efforts to better understand these deadly conditions. RECENT FINDINGS: The
heterogeneity of clinical manifestations seen in patients with hyperinflammatory
syndromes continues to grow as novel genetic and immunotherapeutic triggers of
cytokine storm have been identified. Recent studies characterize unique cytokine
and gene expression profiles from patients with different hyperinflammatory
syndromes, whereas novel murine models begin to define networks of immune
dysregulation thought to drive excessive inflammation in cytokine storm. SUMMARY:
Emerging evidence suggests hypercytokinemia is the driving cause of
immunopathology and morbidity/mortality in hyperinflammatory syndromes.
Therefore, approaches to block cytokine function may be fruitful in treating
hyperinflammatory syndromes with less toxicity than current therapies. However,
not all hyperinflammatory syndromes result in the same pathogenic cytokine
profile, implying that a personalized approach will be required for effective use
of anticytokine therapies in the treatment of hyperinflammatory syndromes.