期刊
JOURNAL OF RHEUMATOLOGY
卷 40, 期 6, 页码 761-767出版社
J RHEUMATOL PUBL CO
DOI: 10.3899/jrheum.121233
关键词
MACROPHAGE ACTIVATION SYNDROME; JUVENILE IDIOPATHIC ARTHRITIS; HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSES; ETIOLOGY
类别
Macrophage activation syndrome (MAS) is a potentially fatal complication of rheumatic diseases. The condition is considered part of secondary hemophagocytic lymphohistiocytoses (HLH). There are similarities in genetic background, pathogenesis, and clinical and laboratory features with primary HLH (p-HLH). We describe findings in mouse models of secondary HLH, comparing them with models of p-HLH and the cellular and molecular mechanisms involved, and relate them to recent findings in patients with secondary HLH. A multilayer model is presented in which background inflammation, infections, and genetics all contribute in different proportions and in several ways. Once the threshold has been reached, inflammatory cytokines are the final effectors, independent of the interplay between different upstream pathogenic factors.
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