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

Angelo Ravelli, Francesca Minoia, Sergio Davì, Annacarin Horne, Francesca Bovis, Angela Pistorio, Maurizio Aricò, Tadej Avcin, E. M. Behrens, Fabrizio De Benedetti, Alexandra Filipovic, Alexei A. Grom, Jan Inge Henter, Norman T. Ilowite, Michael B. Jordan, Raju Khubchandani, Toshiyuki Kitoh, Kai Lehmberg, Daniel J. Lovell, Paivi M. MiettunenKim E. Nichols, Seza Ozen, Jana Pachlopnik Schmid, Athimalaipet Ramanan, Ricardo Russo, Rayfel Schneider, Gary Sterba, Yosef Uziel, Carol Wallace, Carine Wouters, Nico Wulffraat, Erkan Demirkaya, Hermine Brunner, Alberto Martini, Nicolino Ruperto, Randy Q. Cron, Simona Angioloni, Chiara Pallotti, Michele Pesce, Mariangela Rinaldi, L. Villa, Mario Abinun, Amita Aggarwal, Jonathan Akikusa, Sulaiman Al-Mayouf, M. Alessio, Jordi Anton, Maria Teresa Apaz, Itziar Astigarraga, Nuray A. Ayaz, Patrizia Barone, Blanca Bica, Isabel Bolt, L. Breda, Vyacheslav Chasnyk, Rolando Cimaz, Fabrizia Corona, Ruben Cuttica, Gianfranco D'Angelo, Zane Davidsone, Carmen De Cunto, Jaime De Inocencio, Eli Eisenstein, Sandra Enciso, Graciela Espada, Michel Fischbach, Michael Frosch, Romina Gallizzi, Maria Luz Gamir, Yi Jin Gao, Thomas Griffin, Soad Hashad, Teresa Hennon, Gerd Horneff, Zeng Huasong, Adam Huber, Antonella Insalaco, Maka Ioseliani, Marija Jelusic-Drazic, Michael Jeng, Ageza M. Kapovic, Ozgur Kasapcopur, Isabelle Koné-Paut, Sheila Knupp Feitosa De Oliveira, B. Lattanzi, L. Lepore, Caifeng Li, Jeffrey M. Lipton, Silvia Magni Manzoni, Despoina Maritsi, Deborah McCurdy, Rosa Merino, Velma Mulaosmanovic, Susan Nielsen, Priyankar Pal, Sampath Prahalad, D. Rigante, Ingrida Rumba-Rozenfelde, Claudia S. Magalhaes, Helga Sanner, Sujata Sawhney, Wafaa M. Sewairi, Bita Shakoory, Susan Shenoi, Artur Silva Clovis, Valda Stanevicha, Kimo C. Stine, Gordana Susic, Flavio Sztajnbok, Syuji Takei, Hasan Tezer, Ralf Trauzeddel, Elena Tsitsami, Erbil Unsal, Olga Vougiouka, Lehn K. Weaver, Jennifer E. Weiss, Sheila Weitzman, Mabruka Zletni

Research output: Contribution to journalArticle

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.

Original languageEnglish
Article numbere000161
JournalRMD Open
Volume2
Issue number1
DOIs
Publication statusPublished - 2016

ASJC Scopus subject areas

  • Rheumatology
  • Immunology
  • Immunology and Allergy

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    Ravelli, A., Minoia, F., Davì, S., Horne, A., Bovis, F., Pistorio, A., Aricò, M., Avcin, T., Behrens, E. M., De Benedetti, F., Filipovic, A., Grom, A. A., Henter, J. I., Ilowite, N. T., Jordan, M. B., Khubchandani, R., Kitoh, T., Lehmberg, K., Lovell, D. J., ... Zletni, M. (2016). Expert consensus on dynamics of laboratory tests for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. RMD Open, 2(1), [e000161]. https://doi.org/10.1136/rmdopen-2015-000161