2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative

Angelo Ravelli, Francesca Minoia, Sergio Davì, Annacarin Horne, Francesca Bovis, Angela Pistorio, Maurizio Aricò, Tadej Avcin, Edward M. Behrens, Fabrizio De Benedetti, Lisa Filipovic, Alexei A. Grom, Jan Inge Henter, Norman Todd Ilowite, Michael B. Jordan, Raju Khubchandani, Toshiyuki Kitoh, Kai Lehmberg, Daniel J. Lovell, Paivi Miettunen & 16 others Kim E. Nichols, Seza Ozen, Jana Pachlopnik Schmid, Athimalaipet V. Ramanan, Ricardo Russo, Rayfel Schneider, Gary Sterba, Yosef Uziel, Carol Wallace, Carine Wouters, Nico Wulffraat, Erkan Demirkaya, Hermine I. Brunner, Alberto Martini, Nicolino Ruperto, Randy Q. Cron

Research output: Contribution to journalArticle

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Abstract

Objective To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). Methods A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of 28 experts was first asked to classify 428 patient profiles as having or not having MAS, based on clinical and laboratory features at the time of disease onset. The 428 profiles comprised 161 patients with systemic JIA-associated MAS and 267 patients with a condition that could potentially be confused with MAS (active systemic JIA without evidence of MAS, or systemic infection). Next, the ability of candidate criteria to classify individual patients as having MAS or not having MAS was assessed by evaluating the agreement between the classification yielded using the criteria and the consensus classification of the experts. The final criteria were selected in a consensus conference. Results Experts achieved consensus on the classification of 391 of the 428 patient profiles (91.4%). A total of 982 candidate criteria were tested statistically. The 37 best-performing criteria and 8 criteria obtained from the literature were evaluated at the consensus conference. During the conference, 82% consensus among experts was reached on the final MAS classification criteria. In validation analyses, these criteria had a sensitivity of 0.73 and a specificity of 0.99. Agreement between the classification (MAS or not MAS) obtained using the criteria and the original diagnosis made by the treating physician was high (κ = 0.76). Conclusion We have developed a set of classification criteria for MAS complicating systemic JIA and provided preliminary evidence of its validity. Use of these criteria will potentially improve understanding of MAS in systemic JIA and enhance efforts to discover effective therapies, by ensuring appropriate patient enrollment in studies.

Original languageEnglish (US)
Pages (from-to)566-576
Number of pages11
JournalArthritis and Rheumatology
Volume68
Issue number3
DOIs
StatePublished - Mar 1 2016

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Macrophage Activation Syndrome
Juvenile Arthritis
Rheumatology
Pediatrics

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy
  • Rheumatology
  • Medicine(all)

Cite this

2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis : A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. / Ravelli, Angelo; Minoia, Francesca; Davì, Sergio; Horne, Annacarin; Bovis, Francesca; Pistorio, Angela; Aricò, Maurizio; Avcin, Tadej; Behrens, Edward M.; De Benedetti, Fabrizio; Filipovic, Lisa; Grom, Alexei A.; Henter, Jan Inge; Ilowite, Norman Todd; Jordan, Michael B.; Khubchandani, Raju; Kitoh, Toshiyuki; Lehmberg, Kai; Lovell, Daniel J.; Miettunen, Paivi; Nichols, Kim E.; Ozen, Seza; Pachlopnik Schmid, Jana; Ramanan, Athimalaipet V.; Russo, Ricardo; Schneider, Rayfel; Sterba, Gary; Uziel, Yosef; Wallace, Carol; Wouters, Carine; Wulffraat, Nico; Demirkaya, Erkan; Brunner, Hermine I.; Martini, Alberto; Ruperto, Nicolino; Cron, Randy Q.

In: Arthritis and Rheumatology, Vol. 68, No. 3, 01.03.2016, p. 566-576.

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, L, Grom, AA, Henter, JI, Ilowite, NT, Jordan, MB, Khubchandani, R, Kitoh, T, Lehmberg, K, Lovell, DJ, Miettunen, P, Nichols, KE, Ozen, S, Pachlopnik Schmid, J, Ramanan, AV, Russo, R, Schneider, R, Sterba, G, Uziel, Y, Wallace, C, Wouters, C, Wulffraat, N, Demirkaya, E, Brunner, HI, Martini, A, Ruperto, N & Cron, RQ 2016, '2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative', Arthritis and Rheumatology, vol. 68, no. 3, pp. 566-576. https://doi.org/10.1002/art.39332
Ravelli, Angelo ; Minoia, Francesca ; Davì, Sergio ; Horne, Annacarin ; Bovis, Francesca ; Pistorio, Angela ; Aricò, Maurizio ; Avcin, Tadej ; Behrens, Edward M. ; De Benedetti, Fabrizio ; Filipovic, Lisa ; Grom, Alexei A. ; Henter, Jan Inge ; Ilowite, Norman Todd ; Jordan, Michael B. ; Khubchandani, Raju ; Kitoh, Toshiyuki ; Lehmberg, Kai ; Lovell, Daniel J. ; Miettunen, Paivi ; Nichols, Kim E. ; Ozen, Seza ; Pachlopnik Schmid, Jana ; Ramanan, Athimalaipet V. ; Russo, Ricardo ; Schneider, Rayfel ; Sterba, Gary ; Uziel, Yosef ; Wallace, Carol ; Wouters, Carine ; Wulffraat, Nico ; Demirkaya, Erkan ; Brunner, Hermine I. ; Martini, Alberto ; Ruperto, Nicolino ; Cron, Randy Q. / 2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis : A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. In: Arthritis and Rheumatology. 2016 ; Vol. 68, No. 3. pp. 566-576.
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title = "2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative",
abstract = "Objective To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). Methods A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of 28 experts was first asked to classify 428 patient profiles as having or not having MAS, based on clinical and laboratory features at the time of disease onset. The 428 profiles comprised 161 patients with systemic JIA-associated MAS and 267 patients with a condition that could potentially be confused with MAS (active systemic JIA without evidence of MAS, or systemic infection). Next, the ability of candidate criteria to classify individual patients as having MAS or not having MAS was assessed by evaluating the agreement between the classification yielded using the criteria and the consensus classification of the experts. The final criteria were selected in a consensus conference. Results Experts achieved consensus on the classification of 391 of the 428 patient profiles (91.4{\%}). A total of 982 candidate criteria were tested statistically. The 37 best-performing criteria and 8 criteria obtained from the literature were evaluated at the consensus conference. During the conference, 82{\%} consensus among experts was reached on the final MAS classification criteria. In validation analyses, these criteria had a sensitivity of 0.73 and a specificity of 0.99. Agreement between the classification (MAS or not MAS) obtained using the criteria and the original diagnosis made by the treating physician was high (κ = 0.76). Conclusion We have developed a set of classification criteria for MAS complicating systemic JIA and provided preliminary evidence of its validity. Use of these criteria will potentially improve understanding of MAS in systemic JIA and enhance efforts to discover effective therapies, by ensuring appropriate patient enrollment in studies.",
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, {Edward M.} and {De Benedetti}, Fabrizio and Lisa Filipovic and Grom, {Alexei A.} and Henter, {Jan Inge} and Ilowite, {Norman Todd} and Jordan, {Michael B.} and Raju Khubchandani and Toshiyuki Kitoh and Kai Lehmberg and Lovell, {Daniel J.} and Paivi Miettunen and Nichols, {Kim E.} and Seza Ozen and {Pachlopnik Schmid}, Jana and Ramanan, {Athimalaipet V.} 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 Brunner, {Hermine I.} and Alberto Martini and Nicolino Ruperto and Cron, {Randy Q.}",
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pages = "566--576",
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TY - JOUR

T1 - 2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

T2 - A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative

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, Edward M.

AU - De Benedetti, Fabrizio

AU - Filipovic, Lisa

AU - Grom, Alexei A.

AU - Henter, Jan Inge

AU - Ilowite, Norman Todd

AU - Jordan, Michael B.

AU - Khubchandani, Raju

AU - Kitoh, Toshiyuki

AU - Lehmberg, Kai

AU - Lovell, Daniel J.

AU - Miettunen, Paivi

AU - Nichols, Kim E.

AU - Ozen, Seza

AU - Pachlopnik Schmid, Jana

AU - Ramanan, Athimalaipet V.

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 I.

AU - Martini, Alberto

AU - Ruperto, Nicolino

AU - Cron, Randy Q.

PY - 2016/3/1

Y1 - 2016/3/1

N2 - Objective To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). Methods A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of 28 experts was first asked to classify 428 patient profiles as having or not having MAS, based on clinical and laboratory features at the time of disease onset. The 428 profiles comprised 161 patients with systemic JIA-associated MAS and 267 patients with a condition that could potentially be confused with MAS (active systemic JIA without evidence of MAS, or systemic infection). Next, the ability of candidate criteria to classify individual patients as having MAS or not having MAS was assessed by evaluating the agreement between the classification yielded using the criteria and the consensus classification of the experts. The final criteria were selected in a consensus conference. Results Experts achieved consensus on the classification of 391 of the 428 patient profiles (91.4%). A total of 982 candidate criteria were tested statistically. The 37 best-performing criteria and 8 criteria obtained from the literature were evaluated at the consensus conference. During the conference, 82% consensus among experts was reached on the final MAS classification criteria. In validation analyses, these criteria had a sensitivity of 0.73 and a specificity of 0.99. Agreement between the classification (MAS or not MAS) obtained using the criteria and the original diagnosis made by the treating physician was high (κ = 0.76). Conclusion We have developed a set of classification criteria for MAS complicating systemic JIA and provided preliminary evidence of its validity. Use of these criteria will potentially improve understanding of MAS in systemic JIA and enhance efforts to discover effective therapies, by ensuring appropriate patient enrollment in studies.

AB - Objective To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). Methods A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of 28 experts was first asked to classify 428 patient profiles as having or not having MAS, based on clinical and laboratory features at the time of disease onset. The 428 profiles comprised 161 patients with systemic JIA-associated MAS and 267 patients with a condition that could potentially be confused with MAS (active systemic JIA without evidence of MAS, or systemic infection). Next, the ability of candidate criteria to classify individual patients as having MAS or not having MAS was assessed by evaluating the agreement between the classification yielded using the criteria and the consensus classification of the experts. The final criteria were selected in a consensus conference. Results Experts achieved consensus on the classification of 391 of the 428 patient profiles (91.4%). A total of 982 candidate criteria were tested statistically. The 37 best-performing criteria and 8 criteria obtained from the literature were evaluated at the consensus conference. During the conference, 82% consensus among experts was reached on the final MAS classification criteria. In validation analyses, these criteria had a sensitivity of 0.73 and a specificity of 0.99. Agreement between the classification (MAS or not MAS) obtained using the criteria and the original diagnosis made by the treating physician was high (κ = 0.76). Conclusion We have developed a set of classification criteria for MAS complicating systemic JIA and provided preliminary evidence of its validity. Use of these criteria will potentially improve understanding of MAS in systemic JIA and enhance efforts to discover effective therapies, by ensuring appropriate patient enrollment in studies.

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U2 - 10.1002/art.39332

DO - 10.1002/art.39332

M3 - Article

VL - 68

SP - 566

EP - 576

JO - Arthritis and Rheumatology

JF - Arthritis and Rheumatology

SN - 2326-5191

IS - 3

ER -