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 T.
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.
AU - Paediatric Rheumatology International Trials Organisation, the Childhood Arthritis andRheumatology Research Alliance, the Pediatric Rheumatology Collaborative Study Group,and the Histiocyte Societ
AU - Dāvidsone, Zane
N1 - Publisher Copyright:
© 2015, American College of Rheumatology.
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.
UR - http://www.scopus.com/inward/record.url?scp=84963788143&partnerID=8YFLogxK
U2 - 10.1002/art.39332
DO - 10.1002/art.39332
M3 - Article
C2 - 26314788
AN - SCOPUS:84963788143
SN - 2326-5191
VL - 68
SP - 566
EP - 576
JO - Arthritis and Rheumatology
JF - Arthritis and Rheumatology
IS - 3
ER -