Application of MS score in macrophage activation syndrome patients associated with adult onset Still’s disease

Published on Sep 1, 2021in Annals of the Rheumatic Diseases16.102
· DOI :10.1136/ANNRHEUMDIS-2019-216286
Ran Wang5
Estimated H-index: 5
(SJTU: Shanghai Jiao Tong University),
Ting Li39
Estimated H-index: 39
(SJTU: Shanghai Jiao Tong University)
+ 5 AuthorsQiong Fu12
Estimated H-index: 12
(SJTU: Shanghai Jiao Tong University)
Sources
Abstract
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, hemophagocytic lymphohistiocytosis (HLH)-2004 and HLH-2009 criteria are widely used to identify MAS associated with AOSD. Hemophagocytic syndrome diagnostic (HS) score was developed previously to facilitate MAS recognition, but still requires validation.5 In 2019, Francesca Minoia et al reported a MS score for classification of sJIA-associated MAS patients.6 Considering that sJIA and AOSD are …
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References6
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#1Francesca Minoia (Istituto Giannina Gaslini)H-Index: 17
#2Francesca Bovis (UniGe: University of Genoa)H-Index: 23
Last. Angelo Ravelli (UniGe: University of Genoa)H-Index: 90
view all 17 authors...
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...
42 CitationsSource
#1F. Parisi (UCSC: Catholic University of the Sacred Heart)H-Index: 4
Last. Elisa GremeseH-Index: 34
view all 5 authors...
RATIONALE: Myocarditis is a rare but potentially fatal complication of Still's disease (about 7% of total cases). PATIENT CONCERNS: A 42-year-old woman was admitted to our ward with high-grade fever, rash and polyarthralgia, lasting since 4 weeks and rapidly complicated by MAS and acute heart failure. DIAGNOSES: Adult Onset Still's Disease rapidly developping macrophage activation syndrome and disseminated intravascular coagulopathy, further complicated by iperacute myocarditis with cardiac arre...
35 CitationsSource
#1Claudia BracagliaH-Index: 21
#2Giusi PrencipeH-Index: 17
Last. Fabrizio De BenedettiH-Index: 57
view all 3 authors...
Background Macrophage activation syndrome (MAS) is a severe complication of rheumatic disease in childhood, particularly in systemic Juvenile Idiopathic Arthritis (sJIA). It is characterize by an uncontrolled activation and proliferation of T lymphocytes and macrophages.
80 CitationsSource
#1Philippe Guilpain (University of Montpellier)H-Index: 21
#2Alain Le Quellec (University of Montpellier)H-Index: 19
Adult onset Still’s disease (AOSD) is a rare inflammatory disorder that remains poorly understood. Its pathophysiology is yet to be completely elucidated, but is known to consist mainly on a cytokine cascade, responsible for the systemic manifestations. AOSD diagnosis is usually difficult and delayed, with physicians having to rule out several other conditions, including cancer or infectious diseases. Prognosis is heterogeneous and difficult to establish, ranging from benign outcome to chronic d...
10 CitationsSource
#1Mariam Aysha Siddiqui (U of C: University of Chicago)H-Index: 1
#2Michael S. Putman (U of C: University of Chicago)H-Index: 8
Last. Anisha Bharadwaj Dua (U of C: University of Chicago)H-Index: 6
view all 3 authors...
Adult-onset Still’s disease (AOSD) – a multi-systemic inflammatory condition characterized by high fevers, polyarthritis, an evanescent rash, and pharyngitis – has been a challenging condition to diagnose expediently and treat effectively. Questions remain regarding the underlying pathophysiology and etiology of AOSD. Pathognomonic diagnostic tests and reliable biomarkers remain undiscovered. Over the past decade, important progress has been made. Diagnostic criteria employing glycosylated ferri...
8 CitationsSource
#1Laurence Fardet (UPMC: Pierre-and-Marie-Curie University)H-Index: 27
#2Lionel GalicierH-Index: 67
Last. Gilles Hejblum (UPMC: Pierre-and-Marie-Curie University)H-Index: 20
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Objective Because it has no unique clinical, biologic, or histologic features, reactive hemophagocytic syndrome may be difficult to distinguish from other diseases such as severe sepsis or hematologic malignancies. This study was undertaken to develop and validate a diagnostic score for reactive hemophagocytic syndrome. Methods A multicenter retrospective cohort of 312 patients who were judged by experts to have reactive hemophagocytic syndrome (n = 162), were judged by experts to not have react...
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Cited By6
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#1Francesca Minoia (Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico)H-Index: 17
#2Angelo Ravelli (UniGe: University of Genoa)H-Index: 90
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...
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#1Erdal Sag (Hacettepe University)H-Index: 10
#2Armagan Keskin (Hacettepe University)
Last. Seza Ozen (Hacettepe University)H-Index: 81
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Macrophage activation syndrome (MAS) is a devastating complication of systemic JIA (sJIA), seen in approximately 10-25% of the sJIA patients. A number of criteria have been proposed to differentiate between activation of sJIA and MAS, including HScore and the recently proposed MS-score. This is the first study comparing the performances of MS-score and HScore for the diagnosis of MAS in sJIA patients. Systemic JIA patients followed at Hacettepe University Pediatric Rheumatology Unit were include...
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#1Lei Zhang (Zhengzhou University)H-Index: 3
#2Xiaopei Yang (Zhengzhou University)H-Index: 2
Last. Shengyun Liu (Zhengzhou University)H-Index: 7
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Background: Adult-onset Still’s disease (AOSD) is a rare multisystemic auto-inflammatory disease.Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic disorders, including sJIA, adult-onset Still’s disease (AOSD) and lupus. Many tools have been developed for the diagnosis of MAS, including HScore and MS score. Although some authors have tried to apply these tools for the diagnosis of ASOD-associated MAS, there are no studies comparing the diagnostic ability of HSco...
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#1Francesca Minoia (Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico)H-Index: 17
#2Angelo Ravelli (UniGe: University of Genoa)H-Index: 90
We thank Zhang et al 1 for their interest in our MAS/sJIA (MS) score for diagnosis of macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis (sJIA).2 Considering that sJIA and adult-onset Still’s disease (AOSD) are nowadays thought to constitute a continuum of a single disease entity3 4 and that they share a similar risk for MAS, it is worth evaluating whether the proposed diagnostic tools are suitable to detect MAS in both illnesses. Zhang et al 1 compared the diagnostic...
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#1Ran Wang (SJTU: Shanghai Jiao Tong University)H-Index: 5
#2Ting Li (SJTU: Shanghai Jiao Tong University)H-Index: 39
Last. Qiong Fu (SJTU: Shanghai Jiao Tong University)H-Index: 12
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To explore the clinical features, treatments, and prognostic factors of adult-onset Still’s disease (AOSD)-associated macrophage activation syndrome (MAS), we conducted a multicenter retrospective clinical study of AOSD-associated MAS patients. AOSD patients were collected from six tertiary hospitals in China. Medical charts were reviewed and clinical information was recorded and analyzed. There were 447 AOSD patients enrolled into this retrospective clinical study. Among them, 55 were diagnosed...
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#1Qiongyi Hu (SJTU: Shanghai Jiao Tong University)H-Index: 12
#2Mengyan Wang (SJTU: Shanghai Jiao Tong University)H-Index: 3
Last. Chengde Yang (SJTU: Shanghai Jiao Tong University)H-Index: 20
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Adult-onset Still’s disease (AOSD) is an autoinflammatory disease characterised by spiking fever, rash, polyarthralgia, sore throat and even life-threatening complications, such as macrophage activation syndrome and fulminant hepatitis. Excessive and inappropriate production of cytokines is a cornerstone in AOSD pathogenesis.1 Unlike anakinra and tocilizumab, Janus kinases (JAK) inhibitors block the proinflammatory effect of a wide range of cytokines. This range of activity could be beneficial i...
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