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: To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). 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. 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). 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.
authors: Università degli Studi di Genova and Istituto Giannina Gaslini, Genoa, Italy.
authors: Angelo
authors: A
authors: Ravelli
authors: Istituto Giannina Gaslini, Genoa, Italy.
authors: Francesca
authors: F
authors: Minoia
authors: Istituto Giannina Gaslini, Genoa, Italy.
authors: Sergio
authors: S
authors: Davì
authors: Karolinska Institute and Karolinska University Hospital Solna, Stockholm, Sweden.
authors: AnnaCarin
authors: A
authors: Horne
authors: Istituto Giannina Gaslini, Genoa, Italy.
authors: Francesca
authors: F
authors: Bovis
authors: Istituto Giannina Gaslini, Genoa, Italy.
authors: Angela
authors: A
authors: Pistorio
authors: Azienda Sanitaria Provinciale 7, Ragusa, Italy.
authors: Maurizio
authors: M
authors: Aricò
authors: University Children's Hospital, Ljubljana, Slovenia.
authors: Tadej
authors: T
authors: Avcin
authors: The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
authors: Edward M
authors: EM
authors: Behrens
authors: Ospedale Pediatrico Bambino Gesù, Rome, Italy.
authors: Fabrizio
authors: F
authors: De Benedetti
authors: Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
authors: Lisa
authors: L
authors: Filipovic
authors: Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
authors: Alexei A
authors: AA
authors: Grom
authors: Karolinska Institute and Karolinska University Hospital Solna, Stockholm, Sweden.
authors: Jan-Inge
authors: JI
authors: Henter
authors: Albert Einstein College of Medicine and Children's Hospital at Montefiore, Bronx, New York, USA.
authors: Norman T
authors: NT
authors: Ilowite
authors: Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
authors: Michael B
authors: MB
authors: Jordan
authors: Jaslok Hospital and Research Centre, Mumbai, India.
authors: Raju
authors: R
authors: Khubchandani
authors: Aichi Medical University, Nagakute, Japan.
authors: Toshiyuki
authors: T
authors: Kitoh
authors: University Medical Center, Hamburg, Germany.
authors: Kai
authors: K
authors: Lehmberg
authors: Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
authors: Daniel J
authors: DJ
authors: Lovell
authors: University of Calgary, Calgary, Alberta, Canada.
authors: Paivi
authors: P
authors: Miettunen
authors: St Jude Children's Research Hospital, Memphis, Tennessee, USA.
authors: Kim E
authors: KE
authors: Nichols
authors: Hacettepe University, Ankara, Turkey.
authors: Seza
authors: S
authors: Ozen
authors: University Children's Hospital, Zurich, Switzerland.
authors: Jana
authors: J
authors: Pachlopnik Schmid
authors: Bristol Royal Hospital for Children, Bristol, UK.
authors: Athimalaipet V
authors: AV
authors: Ramanan
authors: Hospital de Pediatria Juan P. Garrahan, Buenos Aires, Argentina.
authors: Ricardo
authors: R
authors: Russo
authors: University of Toronto and Hospital for Sick Children, Toronto, Ontario, Canada.
authors: Rayfel
authors: R
authors: Schneider
authors: Mount Sinai Medical Center, Miami Beach, Florida, USA.
authors: Gary
authors: G
authors: Sterba
authors: Meir Medical Centre, Kfar Saba, Israel.
authors: Yosef
authors: Y
authors: Uziel
authors: Seattle Children's Hospital and University of Washington, Seattle, USA.
authors: Carol
authors: C
authors: Wallace
authors: University Hospital Leuven, Leuven, Belgium.
authors: Carine
authors: C
authors: Wouters
authors: Wilhelmina Children's Hospital and University Medical Center Utrecht, Uthrecht, The Netherlands.
authors: Nico
authors: N
authors: Wulffraat
authors: Gulhane Military Medical Faculty, Ankara, Turkey.
authors: Erkan
authors: E
authors: Demirkaya
authors: Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
authors: Hermine I
authors: HI
authors: Brunner
authors: Università degli Studi di Genova and Istituto Giannina Gaslini, Genoa, Italy.
authors: Alberto
authors: A
authors: Martini
authors: Istituto Giannina Gaslini, Genoa, Italy.
authors: Nicolino
authors: N
authors: Ruperto
authors: University of Alabama at Birmingham, Birmingham, UK.
authors: Randy Q
authors: RQ
authors: Cron
mesh_terms: D001171:Arthritis, Juvenile / Q000150:complications*; D002648:Child; D003697:Delphi Technique; D005060:Europe; D006801:Humans; D016015:Logistic Models; D055501:Macrophage Activation Syndrome / Q000145:classification* / Q000150:complications / Q000175:diagnosis; D015203:Reproducibility of Results; D012219:Rheumatology; D012955:Societies, Medical; D014481:United States
keywords: Adult Onset Still's Disease; Amyloidosis; Analgesics; Ankylosing Spondylitis; Ant-CCP