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

Angelo Ravelli¹, Francesca Minoia², Sergio Davì², AnnaCarin Horne³, Francesca Bovis², Angela Pistorio², Maurizio Aricò⁴, Tadej Avcin⁵, Edward M Behrens⁶, Fabrizio De Benedetti⁷, Alexandra Filipovic⁸, Alexei A Grom⁹, Jan-Inge Henter³, Norman T Ilowite⁹, Michael B Jordan⁸, Raju Khubchandani¹⁰, Toshiyuki Kitoh¹¹, Kai Lehmberg¹², Daniel J Lovell⁸, Paivi Miettunen¹³, Kim E Nichols¹⁴, Seza Ozen¹⁵, Jana Pachlopnik Schmid¹⁶, Athimalaipet V Ramanan¹⁷, Ricardo Russo¹⁸, Rayfel Schneider¹⁹, Gary Sterba²⁰, Yosef Uziel²¹, Carol Wallace²², Carine Wouters²³, Nico Wulffraat²⁴, Erkan Demirkaya²⁵, Hermine I Brunner⁸, Alberto Martini¹, Nicolino Ruperto², Randy Q Cron²⁶

Affiliations

¹ Università degli Studi di Genova and Istituto Giannina Gaslini, Genoa, Italy; Istituto Giannina Gaslini, Genoa, Italy.
² Istituto Giannina Gaslini , Genoa , Italy.
³ Karolinska Institute , Karolinska University Hospital Solna , Stockholm , Sweden.
⁴ Azienda Sanitaria Provinciale 7 , Ragusa , Italy.
⁵ University Children's Hospital , Ljubljana , Slovenia.
⁶ The Children's Hospital of Philadelphia , Philadelphia, Pennsylvania , USA.
⁷ Ospedale Pediatrico Bambino Gesù , Rome , Italy.
⁸ Cincinnati Children's Hospital Medical Center , Cincinnati, Ohio , USA.
⁹ Albert Einstein College of Medicine and Children's Hospital at Montefiore , Bronx, New York , USA.
¹⁰ Jaslok Hospital and Research Centre , Mumbai, Maharashtra , India.
¹¹ Aichi Medical University , Nagakute , Japan.
¹² University Medical Center , Hamburg , Germany.
¹³ University of Calgary , Calgary, Alberta , Canada.
¹⁴ St Jude Children's Research Hospital , Memphis, Tennessee , USA.
¹⁵ Hacettepe University , Ankara , Turkey.
¹⁶ University Children's Hospital , Zurich , Switzerland.
¹⁷ Bristol Royal Hospital for Children , Bristol , UK.
¹⁸ Hospital de Pediatria Juan P Garrahan , Buenos Aires , Argentina.
¹⁹ University of Toronto and Hospital for Sick Children , Toronto, Ontario , Canada.
²⁰ Mount Sinai Medical Center , Miami Beach, Florida , USA.
²¹ Meir Medical Centre , Kfar Saba , Israel.
²² Seattle Children's Hospital and University of Washington , Seattle, Washington , USA.
²³ University Hospital Leuven , Leuven , Belgium.
²⁴ Wilhelmina Children's Hospital and University Medical Center Utrecht , Utrecht , The Netherlands.
²⁵ Gulhane Military Medical Faculty, Ankara , Turkey.
²⁶ University of Alabama at Birmingham , Birmingham, Alabama , USA.

PMID: 26848401
PMCID: PMC4731834
DOI: 10.1136/rmdopen-2015-000161

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

Angelo Ravelli et al. RMD Open. 2016.

. 2016 Jan 19;2(1):e000161.

doi: 10.1136/rmdopen-2015-000161. eCollection 2016.

Authors

Affiliations

¹ Università degli Studi di Genova and Istituto Giannina Gaslini, Genoa, Italy; Istituto Giannina Gaslini, Genoa, Italy.
² Istituto Giannina Gaslini , Genoa , Italy.
³ Karolinska Institute , Karolinska University Hospital Solna , Stockholm , Sweden.
⁴ Azienda Sanitaria Provinciale 7 , Ragusa , Italy.
⁵ University Children's Hospital , Ljubljana , Slovenia.
⁶ The Children's Hospital of Philadelphia , Philadelphia, Pennsylvania , USA.
⁷ Ospedale Pediatrico Bambino Gesù , Rome , Italy.
⁸ Cincinnati Children's Hospital Medical Center , Cincinnati, Ohio , USA.
⁹ Albert Einstein College of Medicine and Children's Hospital at Montefiore , Bronx, New York , USA.
¹⁰ Jaslok Hospital and Research Centre , Mumbai, Maharashtra , India.
¹¹ Aichi Medical University , Nagakute , Japan.
¹² University Medical Center , Hamburg , Germany.
¹³ University of Calgary , Calgary, Alberta , Canada.
¹⁴ St Jude Children's Research Hospital , Memphis, Tennessee , USA.
¹⁵ Hacettepe University , Ankara , Turkey.
¹⁶ University Children's Hospital , Zurich , Switzerland.
¹⁷ Bristol Royal Hospital for Children , Bristol , UK.
¹⁸ Hospital de Pediatria Juan P Garrahan , Buenos Aires , Argentina.
¹⁹ University of Toronto and Hospital for Sick Children , Toronto, Ontario , Canada.
²⁰ Mount Sinai Medical Center , Miami Beach, Florida , USA.
²¹ Meir Medical Centre , Kfar Saba , Israel.
²² Seattle Children's Hospital and University of Washington , Seattle, Washington , USA.
²³ University Hospital Leuven , Leuven , Belgium.
²⁴ Wilhelmina Children's Hospital and University Medical Center Utrecht , Utrecht , The Netherlands.
²⁵ Gulhane Military Medical Faculty, Ankara , Turkey.
²⁶ University of Alabama at Birmingham , Birmingham, Alabama , USA.

PMID: 26848401
PMCID: PMC4731834
DOI: 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.

Keywords: Epidemiology; Juvenile Idiopathic Arthritis; Outcomes research.

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

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

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