Cloak and dagger: the case for adult onset still disease and hemophagocytic lymphohistiocytosis |
| |
Authors: | Francisco Hélder C Félix Luzia Kalyne A M Leal Juvenia Bezerra Fontenele |
| |
Institution: | 1.Peter Pan Pediatric Hemato-Oncology Unit,Hospital Infantil Albert Sabin,Fortaleza,Brazil;2.Department of Pharmacy, Faculdade de Farmácia, Ondontologia e Enfermagem,Universidade Federal do Ceará,Fortaleza,Brazil;3.Department of Physiology and Pharmacology, Faculdade de Medicina,Universidade Federal do Ceará,Fortaleza,Brazil |
| |
Abstract: | Adult onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. Systemic onset juvenile idiopathic
arthritis (SoJIA) is the preferred nomenclature of Still’s disease. Strong association with so-called macrophage activation
syndrome (MAS) may provide a clue to the understanding of the distinctive pathogenetic features of SoJIA. MAS is a severe,
potentially life-threatening complication characterized by the excessive activation of well-differentiated macrophages. It
is more appropriately named autoimmune disease associated reactive hemophagocytic lymphohistiocytosis (ReHLH), a subset of
a histiocytic disorder: class II histiocytosis hemophagocytic lymphohistiocytosis (HLH). The relation of SoJIA with HLH is
still under debate. We propose that MAS, HLH, SoJIA, and AOSD are indeed the same disease, in different clinical presentations
that may be classified based on severity and laboratory findings, but with essentially the same physiopathogenesis. We propose
that the case described by Hong & Lee (Rheumatol Int 2008) was actually an AOSD-associated MAS/RHS/ReHLH fulminant disease. |
| |
Keywords: | Hemophagocytic lymphohistiocytosis Juvenile idiopathic arthritis |
本文献已被 SpringerLink 等数据库收录! |
|