A novel assessment tool for clinical care of patients with autoinflammatory disease: juvenile autoinflammatory disease multidimensional assessment report

作者：Konukbay D; Gattorno M; Yildiz D; Frenkel J; Acikel C; Sozeri B; Makay B; Ayaz N Aktay; Barut K; Kisaarslan A; Bilginer Y; Karaman D; Peru H; Simsek D; Aydog O; Unsal E; Gunduz Z; Fidanci B E; Kone Paut I; Kasapcopur O; Ravelli A; Ozen S; Demirkaya E^*

来源：Clinical and Experimental Rheumatology, 2016, 34(6): S129-S135.

• auto-inflammatory disease
• outcome instrument
• multidimensional
• proxy report
• familial Mediterranean fever

Objective. To develop and test a new multidimensional questionnaire for assessment of children with auto-inflammatory disease (AID) such as FMF, PFAPA, HIDS, TRAPS in standard clinical care.
Methods. The juvenile auto-inflammatory disease multidimensional assessment report (JAIMAR) includes 16 parent or patient-centered measures and four dimensions that assess functional status, pain, therapeutic compliance and health-related quality of life (physical, social, school, emotional status) with disease outcome. It is proposed for use as both a proxy-report and a patient self report, with the suggested age range of 8-18 years for use as a self-report.
Results. 250 children with FMF were included in the study. Total of 179 forms were filled tip by parents and patients, and 71 forms were filled up by parents having children less than 8 years. Completing and scoring the JAIMAR can be done in 15 minutes. For the JAIMAR's dimensions, the Cronbach's alpha coefficient for internal consistency was between 0.507-0.998. There was a significant and a positive correlation between the test-retest scale scores (ICC=0.607-0.966). Concerning construct validity, all factors loadings were above 030. For the criterion validity, the correlation level between each dimension and the related scale ranged from medium (r=0329, p<0.0001) to large (r=0.894, p<0.0001). The parents' proxy-reported and children's self reported data were outstandingly concordant (r=0.770-0.989).
Conclusion. The development of the JAIMAR introduces a new and multidimensional approach in paediatric rheumatology practice. It is a new tool for children with auto-inflammatory disease and it may help enhance their quality of care.

• 出版日期2016-12