Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis

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http://hdl.handle.net/10138/162482

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Boom , V , Anton , J , Lahdenne , P , Quartier , P , Ravelli , A , Wulffraat , N M & Vastert , S J 2015 , ' Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis ' , Pediatric Rheumatology Online Journal , vol. 13 , 55 . https://doi.org/10.1186/s12969-015-0055-3

Title: Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis
Author: Boom, V.; Anton, J.; Lahdenne, P.; Quartier, P.; Ravelli, A.; Wulffraat, N. M.; Vastert, S. J.
Contributor organization: Children's Hospital
Clinicum
HUS Children and Adolescents
Date: 2015-12-03
Language: eng
Number of pages: 13
Belongs to series: Pediatric Rheumatology Online Journal
ISSN: 1546-0096
DOI: https://doi.org/10.1186/s12969-015-0055-3
URI: http://hdl.handle.net/10138/162482
Abstract: Background: Macrophage activation syndrome (MAS) is a severe and potentially lethal complication of several inflammatory diseases but seems particularly linked to systemic juvenile idiopathic arthritis (sJIA). Standardized diagnostic and treatment guidelines for MAS in sJIA are currently lacking. The aim of this systematic literature review was to evaluate currently available literature on diagnostic criteria for MAS in sJIA and provide an overview of possible biomarkers for diagnosis, disease activity and treatment response and recent advances in treatment. Methods: A systematic literature search was performed in MEDLINE, EMBASE and Cochrane. 495 papers were identified. Potentially relevant papers were selected by 3 authors after which full text screening was performed. All selected papers were evaluated by at least two independent experts for validity and level of evidence according to EULAR guidelines. Results: 27 papers were included: 7 on diagnosis, 9 on biomarkers and 11 on treatment. Systematic review of the literature confirmed that there are no validated diagnostic criteria for MAS in sJIA. The preliminary Ravelli criteria, with the addition of ferritin, performed well in a large retrospective case-control study. Recently, an international consortium lead by PRINTO proposed a new set of diagnostic criteria able to distinguish MAS from active sJIA and/or infection with superior performance. Other promising diagnostic biomarkers potentially distinguish MAS complicating sJIA from primary and virusassociated hemophagocytic lymphohistiocytosis. The highest level of evidence for treatment comes from case-series. High dose corticosteroids with or without cyclosporine A were frequently reported as first-line therapy. From the newer treatment modalities, promising responses have been reported with anakinra. Conclusion: MAS in sJIA seems to be diagnosed best by the recently proposed PRINTO criteria, although prospective validation is needed. Novel promising biomarkers for sJIA related MAS are in need of prospective validation as well, and are not widely available yet. Currently, treatment of MAS in sJIA relies more on experience than evidence based medicine. Taking into account the severity of MAS and the scarcity of evidence, early expert consultation is recommended as soon as MAS is suspected.
Subject: SJIA
MAS
Hemophagocytosis
Systemic
Arthritis
Complications
Diagnosis
Treatment
Biomarkers
HLH
FOLLISTATIN-LIKE PROTEIN-1
ACQUIRED HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS
KILLER-CELL DYSFUNCTION
RHEUMATOID-ARTHRITIS
SOLUBLE CD25
CYCLOSPORINE-A
INTERLEUKIN-18
ETANERCEPT
GUIDELINES
CHILDREN
3123 Gynaecology and paediatrics
Peer reviewed: Yes
Rights: cc_by
Usage restriction: openAccess
Self-archived version: publishedVersion


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