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. Miettunen & 99 others Kim 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

Fingerprint

Macrophage Activation Syndrome
Juvenile Arthritis
Clinical Laboratory Techniques
Ferritins
Aspartate Aminotransferases
Platelet Count
Blood Sedimentation
Fibrinogen
Early Diagnosis
Neutrophils
Cell Count
Prospective Studies

ASJC Scopus subject areas

  • Rheumatology
  • Immunology
  • Immunology and Allergy

Cite this

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

In: RMD Open, Vol. 2, No. 1, e000161, 2016.

Research output: Contribution to journalArticle

Ravelli, A, Minoia, F, Davì, S, Horne, A, Bovis, F, Pistorio, A, Aricò, M, Avcin, T, Behrens, EM, De Benedetti, F, Filipovic, A, Grom, AA, Henter, JI, Ilowite, NT, Jordan, MB, Khubchandani, R, Kitoh, T, Lehmberg, K, Lovell, DJ, Miettunen, PM, Nichols, KE, Ozen, S, Schmid, JP, Ramanan, A, Russo, R, Schneider, R, Sterba, G, Uziel, Y, Wallace, C, Wouters, C, Wulffraat, N, Demirkaya, E, Brunner, H, Martini, A, Ruperto, N, Cron, RQ, Angioloni, S, Pallotti, C, Pesce, M, Rinaldi, M, Villa, L, Abinun, M, Aggarwal, A, Akikusa, J, Al-Mayouf, S, Alessio, M, Anton, J, Apaz, MT, Astigarraga, I, Ayaz, NA, Barone, P, Bica, B, Bolt, I, Breda, L, Chasnyk, V, Cimaz, R, Corona, F, Cuttica, R, D'Angelo, G, Davidsone, Z, De Cunto, C, De Inocencio, J, Eisenstein, E, Enciso, S, Espada, G, Fischbach, M, Frosch, M, Gallizzi, R, Gamir, ML, Gao, YJ, Griffin, T, Hashad, S, Hennon, T, Horneff, G, Huasong, Z, Huber, A, Insalaco, A, Ioseliani, M, Jelusic-Drazic, M, Jeng, M, Kapovic, AM, Kasapcopur, O, Koné-Paut, I, De Oliveira, SKF, Lattanzi, B, Lepore, L, Li, C, Lipton, JM, Magni Manzoni, S, Maritsi, D, McCurdy, D, Merino, R, Mulaosmanovic, V, Nielsen, S, Pal, P, Prahalad, S, Rigante, D, Rumba-Rozenfelde, I, Magalhaes, CS, Sanner, H, Sawhney, S, Sewairi, WM, Shakoory, B, Shenoi, S, Clovis, AS, Stanevicha, V, Stine, KC, Susic, G, Sztajnbok, F, Takei, S, Tezer, H, Trauzeddel, R, Tsitsami, E, Unsal, E, Vougiouka, O, Weaver, LK, Weiss, JE, Weitzman, S & Zletni, M 2016, 'Expert consensus on dynamics of laboratory tests for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis', RMD Open, vol. 2, no. 1, e000161. https://doi.org/10.1136/rmdopen-2015-000161
Ravelli, Angelo ; Minoia, Francesca ; Davì, Sergio ; Horne, Annacarin ; Bovis, Francesca ; Pistorio, Angela ; Aricò, Maurizio ; Avcin, Tadej ; Behrens, E. M. ; De Benedetti, Fabrizio ; Filipovic, Alexandra ; Grom, Alexei A. ; Henter, Jan Inge ; Ilowite, Norman T. ; Jordan, Michael B. ; Khubchandani, Raju ; Kitoh, Toshiyuki ; Lehmberg, Kai ; Lovell, Daniel J. ; Miettunen, Paivi M. ; Nichols, Kim E. ; Ozen, Seza ; Schmid, Jana Pachlopnik ; Ramanan, Athimalaipet ; Russo, Ricardo ; Schneider, Rayfel ; Sterba, Gary ; Uziel, Yosef ; Wallace, Carol ; Wouters, Carine ; Wulffraat, Nico ; Demirkaya, Erkan ; Brunner, Hermine ; Martini, Alberto ; Ruperto, Nicolino ; Cron, Randy Q. ; Angioloni, Simona ; Pallotti, Chiara ; Pesce, Michele ; Rinaldi, Mariangela ; Villa, L. ; Abinun, Mario ; Aggarwal, Amita ; Akikusa, Jonathan ; Al-Mayouf, Sulaiman ; Alessio, M. ; Anton, Jordi ; Apaz, Maria Teresa ; Astigarraga, Itziar ; Ayaz, Nuray A. ; Barone, Patrizia ; Bica, Blanca ; Bolt, Isabel ; Breda, L. ; Chasnyk, Vyacheslav ; Cimaz, Rolando ; Corona, Fabrizia ; Cuttica, Ruben ; D'Angelo, Gianfranco ; Davidsone, Zane ; De Cunto, Carmen ; De Inocencio, Jaime ; Eisenstein, Eli ; Enciso, Sandra ; Espada, Graciela ; Fischbach, Michel ; Frosch, Michael ; Gallizzi, Romina ; Gamir, Maria Luz ; Gao, Yi Jin ; Griffin, Thomas ; Hashad, Soad ; Hennon, Teresa ; Horneff, Gerd ; Huasong, Zeng ; Huber, Adam ; Insalaco, Antonella ; Ioseliani, Maka ; Jelusic-Drazic, Marija ; Jeng, Michael ; Kapovic, Ageza M. ; Kasapcopur, Ozgur ; Koné-Paut, Isabelle ; De Oliveira, Sheila Knupp Feitosa ; Lattanzi, B. ; Lepore, L. ; Li, Caifeng ; Lipton, Jeffrey M. ; Magni Manzoni, Silvia ; Maritsi, Despoina ; McCurdy, Deborah ; Merino, Rosa ; Mulaosmanovic, Velma ; Nielsen, Susan ; Pal, Priyankar ; Prahalad, Sampath ; Rigante, D. ; Rumba-Rozenfelde, Ingrida ; Magalhaes, Claudia S. ; Sanner, Helga ; Sawhney, Sujata ; Sewairi, Wafaa M. ; Shakoory, Bita ; Shenoi, Susan ; Clovis, Artur Silva ; Stanevicha, Valda ; Stine, Kimo C. ; Susic, Gordana ; Sztajnbok, Flavio ; Takei, Syuji ; Tezer, Hasan ; Trauzeddel, Ralf ; Tsitsami, Elena ; Unsal, Erbil ; Vougiouka, Olga ; Weaver, Lehn K. ; Weiss, Jennifer E. ; Weitzman, Sheila ; Zletni, Mabruka. / Expert consensus on dynamics of laboratory tests for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. In: RMD Open. 2016 ; Vol. 2, No. 1.
@article{95fd00873e434c0b879c3d4671e88273,
title = "Expert consensus on dynamics of laboratory tests for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis",
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.",
author = "Angelo Ravelli and Francesca Minoia and Sergio Dav{\`i} and Annacarin Horne and Francesca Bovis and Angela Pistorio and Maurizio Aric{\`o} and Tadej Avcin and Behrens, {E. M.} and {De Benedetti}, Fabrizio and Alexandra Filipovic and Grom, {Alexei A.} and Henter, {Jan Inge} and Ilowite, {Norman T.} and Jordan, {Michael B.} and Raju Khubchandani and Toshiyuki Kitoh and Kai Lehmberg and Lovell, {Daniel J.} and Miettunen, {Paivi M.} and Nichols, {Kim E.} and Seza Ozen and Schmid, {Jana Pachlopnik} and Athimalaipet Ramanan and Ricardo Russo and Rayfel Schneider and Gary Sterba and Yosef Uziel and Carol Wallace and Carine Wouters and Nico Wulffraat and Erkan Demirkaya and Hermine Brunner and Alberto Martini and Nicolino Ruperto and Cron, {Randy Q.} and Simona Angioloni and Chiara Pallotti and Michele Pesce and Mariangela Rinaldi and L. Villa and Mario Abinun and Amita Aggarwal and Jonathan Akikusa and Sulaiman Al-Mayouf and M. Alessio and Jordi Anton and Apaz, {Maria Teresa} and Itziar Astigarraga and Ayaz, {Nuray A.} and Patrizia Barone and Blanca Bica and Isabel Bolt and L. Breda and Vyacheslav Chasnyk and Rolando Cimaz and Fabrizia Corona and Ruben Cuttica and Gianfranco D'Angelo and Zane Davidsone and {De Cunto}, Carmen and {De Inocencio}, Jaime and Eli Eisenstein and Sandra Enciso and Graciela Espada and Michel Fischbach and Michael Frosch and Romina Gallizzi and Gamir, {Maria Luz} and Gao, {Yi Jin} and Thomas Griffin and Soad Hashad and Teresa Hennon and Gerd Horneff and Zeng Huasong and Adam Huber and Antonella Insalaco and Maka Ioseliani and Marija Jelusic-Drazic and Michael Jeng and Kapovic, {Ageza M.} and Ozgur Kasapcopur and Isabelle Kon{\'e}-Paut and {De Oliveira}, {Sheila Knupp Feitosa} and B. Lattanzi and L. Lepore and Caifeng Li and Lipton, {Jeffrey M.} and {Magni Manzoni}, Silvia and Despoina Maritsi and Deborah McCurdy and Rosa Merino and Velma Mulaosmanovic and Susan Nielsen and Priyankar Pal and Sampath Prahalad and D. Rigante and Ingrida Rumba-Rozenfelde and Magalhaes, {Claudia S.} and Helga Sanner and Sujata Sawhney and Sewairi, {Wafaa M.} and Bita Shakoory and Susan Shenoi and Clovis, {Artur Silva} and Valda Stanevicha and Stine, {Kimo C.} and Gordana Susic and Flavio Sztajnbok and Syuji Takei and Hasan Tezer and Ralf Trauzeddel and Elena Tsitsami and Erbil Unsal and Olga Vougiouka and Weaver, {Lehn K.} and Weiss, {Jennifer E.} and Sheila Weitzman and Mabruka Zletni",
year = "2016",
doi = "10.1136/rmdopen-2015-000161",
language = "English",
volume = "2",
journal = "RMD Open",
issn = "2056-5933",
publisher = "BMJ Publishing Group",
number = "1",

}

TY - JOUR

T1 - Expert consensus on dynamics of laboratory tests for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

AU - Ravelli, Angelo

AU - Minoia, Francesca

AU - Davì, Sergio

AU - Horne, Annacarin

AU - Bovis, Francesca

AU - Pistorio, Angela

AU - Aricò, Maurizio

AU - Avcin, Tadej

AU - Behrens, E. M.

AU - De Benedetti, Fabrizio

AU - Filipovic, Alexandra

AU - Grom, Alexei A.

AU - Henter, Jan Inge

AU - Ilowite, Norman T.

AU - Jordan, Michael B.

AU - Khubchandani, Raju

AU - Kitoh, Toshiyuki

AU - Lehmberg, Kai

AU - Lovell, Daniel J.

AU - Miettunen, Paivi M.

AU - Nichols, Kim E.

AU - Ozen, Seza

AU - Schmid, Jana Pachlopnik

AU - Ramanan, Athimalaipet

AU - Russo, Ricardo

AU - Schneider, Rayfel

AU - Sterba, Gary

AU - Uziel, Yosef

AU - Wallace, Carol

AU - Wouters, Carine

AU - Wulffraat, Nico

AU - Demirkaya, Erkan

AU - Brunner, Hermine

AU - Martini, Alberto

AU - Ruperto, Nicolino

AU - Cron, Randy Q.

AU - Angioloni, Simona

AU - Pallotti, Chiara

AU - Pesce, Michele

AU - Rinaldi, Mariangela

AU - Villa, L.

AU - Abinun, Mario

AU - Aggarwal, Amita

AU - Akikusa, Jonathan

AU - Al-Mayouf, Sulaiman

AU - Alessio, M.

AU - Anton, Jordi

AU - Apaz, Maria Teresa

AU - Astigarraga, Itziar

AU - Ayaz, Nuray A.

AU - Barone, Patrizia

AU - Bica, Blanca

AU - Bolt, Isabel

AU - Breda, L.

AU - Chasnyk, Vyacheslav

AU - Cimaz, Rolando

AU - Corona, Fabrizia

AU - Cuttica, Ruben

AU - D'Angelo, Gianfranco

AU - Davidsone, Zane

AU - De Cunto, Carmen

AU - De Inocencio, Jaime

AU - Eisenstein, Eli

AU - Enciso, Sandra

AU - Espada, Graciela

AU - Fischbach, Michel

AU - Frosch, Michael

AU - Gallizzi, Romina

AU - Gamir, Maria Luz

AU - Gao, Yi Jin

AU - Griffin, Thomas

AU - Hashad, Soad

AU - Hennon, Teresa

AU - Horneff, Gerd

AU - Huasong, Zeng

AU - Huber, Adam

AU - Insalaco, Antonella

AU - Ioseliani, Maka

AU - Jelusic-Drazic, Marija

AU - Jeng, Michael

AU - Kapovic, Ageza M.

AU - Kasapcopur, Ozgur

AU - Koné-Paut, Isabelle

AU - De Oliveira, Sheila Knupp Feitosa

AU - Lattanzi, B.

AU - Lepore, L.

AU - Li, Caifeng

AU - Lipton, Jeffrey M.

AU - Magni Manzoni, Silvia

AU - Maritsi, Despoina

AU - McCurdy, Deborah

AU - Merino, Rosa

AU - Mulaosmanovic, Velma

AU - Nielsen, Susan

AU - Pal, Priyankar

AU - Prahalad, Sampath

AU - Rigante, D.

AU - Rumba-Rozenfelde, Ingrida

AU - Magalhaes, Claudia S.

AU - Sanner, Helga

AU - Sawhney, Sujata

AU - Sewairi, Wafaa M.

AU - Shakoory, Bita

AU - Shenoi, Susan

AU - Clovis, Artur Silva

AU - Stanevicha, Valda

AU - Stine, Kimo C.

AU - Susic, Gordana

AU - Sztajnbok, Flavio

AU - Takei, Syuji

AU - Tezer, Hasan

AU - Trauzeddel, Ralf

AU - Tsitsami, Elena

AU - Unsal, Erbil

AU - Vougiouka, Olga

AU - Weaver, Lehn K.

AU - Weiss, Jennifer E.

AU - Weitzman, Sheila

AU - Zletni, Mabruka

PY - 2016

Y1 - 2016

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=84988448976&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84988448976&partnerID=8YFLogxK

U2 - 10.1136/rmdopen-2015-000161

DO - 10.1136/rmdopen-2015-000161

M3 - Article

VL - 2

JO - RMD Open

JF - RMD Open

SN - 2056-5933

IS - 1

M1 - e000161

ER -