OBJECTIVE: To develop criteria for the classification of @entity15835 ( @entity15835 ) in @entity1 with @entity4932 ( @entity4932 ). METHODS: A multistep process, based on a combination of expert consensus and analysis of real @entity1 data, was conducted. A panel of 28 experts was first asked to classify 428 @entity1 profiles as having or not having @entity15835 , based on clinical and laboratory features at the time of disease onset. The 428 profiles comprised 161 @entity1 with systemic @entity4932 -associated @entity15835 and 267 @entity1 with a condition that could potentially be confused with @entity15835 (active systemic @entity4932 without evidence of @entity15835 , or @entity281 ). Next, the ability of candidate criteria to classify individual @entity1 as having @entity15835 or not having @entity15835 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 @entity1 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 @entity15835 classification criteria. In validation analyses, these criteria had a sensitivity of 0.73 and a specificity of 0.99. Agreement between the classification ( @entity15835 or not @entity15835 ) obtained using the criteria and the original diagnosis made by the treating physician was high (k   =   0.76). CONCLUSION: We have developed a set of classification criteria for @entity15835 complicating systemic @entity4932 and provided preliminary evidence of its validity. Use of these criteria will potentially improve understanding of @entity15835 in systemic @entity4932 and enhance efforts to discover effective therapies, by ensuring appropriate @entity1 enrollment in studies.

2016 Classification Criteria for Macrophage Activation Syndrome Complicating XXXX : A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity281 :: ('MESH:D007239', 'Disease') :: ['systemic infection'] @entity15835 :: ('MESH:D055501', 'Disease') :: ['macrophage activation syndrome', 'MAS'] @entity4932 :: ('MESH:D001171', 'Disease') :: ['JIA', 'systemic juvenile idiopathic arthritis']
@entity4932 :: (MESH:D001171,Disease) :: ['Systemic Juvenile Idiopathic Arthritis']
