Adapting the MS score for detection of macrophage activation syndrome in adult-onset Still’s disease. Response to ‘Application of MS score in macrophage activation syndrome patients associated with adult onset Still’s disease’ by Wang et al

Published on Sep 1, 2021in Annals of the Rheumatic Diseases16.102
· DOI :10.1136/ANNRHEUMDIS-2019-216310
Francesca Minoia17
Estimated H-index: 17
(Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico),
Angelo Ravelli90
Estimated H-index: 90
(UniGe: University of Genoa)
We are grateful to Wang et al 1 for testing our diagnostic score for macrophage activation syndrome (MAS)2 in their patients with adult onset Still disease (AOSD). Because it is increasingly recognised that systemic juvenile idiopathic arthritis (sJIA) and AOSD represent the same disease occurring at different ages3 4 and considering that the two illnesses share a similar risk for MAS, it is important to investigate whether the current diagnostic tools are applicable to both conditions. Wang and colleagues evaluated retrospectively the capacity of the MAS/sJIA (MS) score to detect MAS in AOSD by comparing 60 patients with MAS, whose diagnosis was made by HLH-2004 criteria and confirmed by the caring rheumatologists, with 390 patients without MAS. They found that the application of the MS score with the cut-off of ≥ –2.1 obtained in our study yielded a maximum …
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We read with great interest the article by Minoia et al which named development and initial validation of the macrophage activation syndrome (MAS)/systemic juvenile idiopathic arthritis (sJIA) (MS) score for diagnosis of MAS in sJIA. MAS is a life-threatening complication of rheumatic disorders, including sJIA, adult-onset Still’s disease (AOSD) and lupus.1–4 Timely diagnosis and appropriate treatment of MAS are particularly important to improve the prognosis of MAS patients. At present, hemopha...
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Objective To develop and validate a diagnostic score that aids in identifying macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (sJIA). Methods The clinical and laboratory features of 362 patients with sJIA-associated MAS and 404 patients with active sJIA without evidence of MAS were collected in a multinational collaborative project. Eighty percent of the study population was used to develop the score and the remaining 20% constituted the validation sa...
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Abstract To compare pro-inflammatory cytokine profiles and kinetics in patients with adult-onset Still's disease (AOSD) to those in patients with systemic juvenile idiopathic arthritis (s-JIA), we analyzed serum cytokine concentrations in 33 patients with AOSD and 77 patients with s-JIA and compared them with clinical features. Patients with AOSD and s-JIA shared a common cytokine profile pattern of a significant increase in IL-18. Patients with AOSD were classified into two subgroups based on s...
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Background Adult-onset Still’s disease (AOSD), a rare autoinflammatory disorder, resembles systemic juvenile idiopathic arthritis (SJIA). The superimposable systemic clinical features of AOSD and SJIA suggest both clinical phenotypes represent the same disease continuum with different ages of onset. To further characterize the similarity between AOSD and SJIA at the molecular level, 2 previously identified response gene sets in SJIA were used to investigate how genes that respond to interleukin ...
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