Paediatrics Publications

Title

Expert consensus on dynamics of laboratory tests for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

Authors

Angelo Ravelli, Università degli Studi di Genova
Francesca Minoia, Istituto Giannina Gaslini
Sergio Davì, Istituto Giannina Gaslini
Anna Carin Horne, Karolinska Universitetssjukhuset
Francesca Bovis, Istituto Giannina Gaslini
Angela Pistorio, Istituto Giannina Gaslini
Maurizio Aricò, Azienda Sanitaria Provinciale
Tadej Avcin, University Children's Hospital, Ljubljana
Edward M. Behrens, The Children's Hospital of Philadelphia
Fabrizio De Benedetti, IRCCS Ospedale Pediatrico Bambino Gesù
Alexandra Filipovic, Cincinnati Children's Hospital Medical Center
Alexei A. Grom, The Childen's Hospital at Montefiore
Jan Inge Henter, Karolinska Universitetssjukhuset
Norman T. Ilowite, The Childen's Hospital at Montefiore
Michael B. Jordan, Cincinnati Children's Hospital Medical Center
Raju Khubchandani, Jaslok Hospital and Research Centre
Toshiyuki Kitoh, Aichi Medical University
Kai Lehmberg, Universitätsklinikum Hamburg-Eppendorf
Daniel J. Lovell, Cincinnati Children's Hospital Medical Center
Paivi Miettunen, University of Calgary
Kim E. Nichols, St. Jude Children's Research Hospital
Seza Ozen, Hacettepe Üniversitesi
Jana Pachlopnik Schmid, Kinderspital Zürich
Athimalaipet V. Ramanan, Bristol Royal Hospital for Children
Ricardo Russo, Fundacion Hospital de Pediatria Professor Dr. Juan P. Garrahan
Rayfel Schneider, Hospital for Sick Children University of Toronto
Gary Sterba, Mount Sinai Medical Center Miami Beach
Yosef Uziel, Meir Medical Center
Carol Wallace, University of Washington

Document Type

Article

Publication Date

1-1-2016

Journal

RMD Open

Volume

2

Issue

1

URL with Digital Object Identifier

10.1136/rmdopen-2015-000161

Abstract

Objective: To identify which laboratory tests that change over time are most valuable for the timely diagnosis of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (sJIA). Methods: A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of experts was first asked to evaluate 115 profiles of patients with MAS, which included the values of laboratory tests at the pre-MAS visit and at MAS onset, and the change in values between the two time points. The experts were asked to choose the 5 laboratory tests in which change was most important for the diagnosis of MAS and to rank the 5 selected tests in order of importance. The relevance of change in laboratory parameters was further discussed and ranked by the same experts at a consensus conference. Results: Platelet count was the most frequently selected test, followed by ferritin level, aspartate aminotransferase (AST), white cell count, neutrophil count, and fibrinogen and erythrocyte sedimentation rate. Ferritin was most frequently assigned the highest score. At the end of the process, platelet count, ferritin level and AST were the laboratory tests in which the experts found change over time to be most important. Conclusions: We identified the laboratory tests in which change over time is most valuable for the early diagnosis of MAS in sJIA. The dynamics of laboratory values during the course of MAS should be further scrutinised in a prospective study in order to establish the optimal cut-off values for their variation.

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