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Cited by 773

The DOI matches its Crossref record; 36 of the 36 references it cites were found in a registry or an index.

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

Open access
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 T., 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., on behalf of the Paediatric Rheumatology International Trials Organisation, the Childhood Arthritis and Rheumatology Research Alliance, the Pediatric Rheumatology Collaborative Study Group, and the Histiocyte Society·2016·Arthritis & Rheumatology
DOI 10.1002/art.39332
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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.

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Research works printed but not linked: 5

  1. Ravelli A, 2008, Pediatric rheumatology, 55
  2. 10.1007/s00296-010-1650-8 · doi:10.1007/s00296-010-1650-8
  3. 10.1002/acr.20418 · doi:10.1002/acr.20418
  4. Arico M, 1996, Hemophagocytic lymphohistiocytosis: report of 122 children from the International Registry, Leukemia, 10, 197
  5. 10.1111/j.1651-2227.1986.tb10158.x · doi:10.1111/j.1651-2227.1986.tb10158.x

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Metadata

Authors
Ravelli, A., Minoia, F., Davì, S., Horne, A., Bovis, F., Pistorio, A., Aricò, M., Avcin, T., Behrens, E. M., De Benedetti, F., Filipovic, L., Grom, A. A., Henter, J., Ilowite, N. T., Jordan, M. B., Khubchandani, R., Kitoh, T., Lehmberg, K., Lovell, D. J., Miettunen, P., Nichols, K. E., Ozen, S., Pachlopnik Schmid, J., Ramanan, A. V., Russo, R., Schneider, R., Sterba, G., Uziel, Y., Wallace, C., Wouters, C., Wulffraat, N., Demirkaya, E., Brunner, H. I., Martini, A., Ruperto, N., Cron, R. Q., & on behalf of the Paediatric Rheumatology International Trials Organisation, T. C. A. A. R. R. A.
Venue
Arthritis & Rheumatology
Publisher
Wiley
Published
2016-02-09
Volume
68
Issue
3
Pages
566-576
ISSN
  • 2326-5191 (ISSN-L)
  • 2326-5205
DOI
10.1002/art.39332
OpenAlex
W2265918137
Cited by
773
References
36
FWCI
26.68
Type
journal article
Language
en
Open access
bronze
Licence
onlinelibrary.wiley.com/termsAndConditions#vor (vor)
Funders
  • American College of Rheumatology
  • European League Against Rheumatism
  • Paediatric Rheumatology International Trials Organisation

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CoChat. (2026). Citation check: 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. https://app.cochat.ai/papers/10.1002/art.39332
The paper
Ravelli, A., Minoia, F., Davì, S., Horne, A., Bovis, F., Pistorio, A., Aricò, M., Avcin, T., Behrens, E. M., De Benedetti, F., Filipovic, L., Grom, A. A., Henter, J., Ilowite, N. T., Jordan, M. B., Khubchandani, R., Kitoh, T., Lehmberg, K., Lovell, D. J., . . . on behalf of the Paediatric Rheumatology International Trials Organisation, T. C. A. A. R. R. A. (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 & Rheumatology. https://doi.org/10.1002/art.39332

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Bibliographic data: Semantic Scholar, OpenAlex, Crossref.

Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.