naa a22 4500 530185237 CHVBK 20181026074443.0 cr unu---uuuuu 181026e201811 xx s 000 0 eng 10.1136/annrheumdis-2018-213725 doi (SERVAL)BIB_2962AA9B1BC8 30077992 pmid In silico validation of the Autoinflammatory Disease Damage Index [Elektronische Daten] [N.M. Ter Haar, ALJ van Delft, K.V. Annink, H. van Stel, S.M. Al-Mayouf, G. Amaryan, J. Anton, K.S. Barron, S. Benseler, P.A. Brogan, L. Cantarini, M. Cattalini, A.V. Cochino, F. de Benedetti, F. Dedeoglu, A.A. de Jesus, E. Demirkaya, P. Dolezalova, K.L. Durrant, G. Fabio, R. Gallizzi, R. Goldbach-Mansky, E. Hachulla, V. Hentgen, T. Herlin, M. Hofer, H.M. Hoffman, A. Insalaco, A.F. Jansson, T. Kallinich, I. Kone-Paut, A. Kozlova, J.B. Kuemmerle-Deschner, H.J. Lachmann, R.M. Laxer, A. Martini, S. Nielsen, I. Nikishina, A.K. Ombrello, S. Özen, E. Papadopoulou-Alataki, P. Quartier, D. Rigante, R. Russo, A. Simon, M. Trachana, Y. Uziel, A. Ravelli, G. Schulert, M. Gattorno, J. Frenkel] Autoinflammatory diseases can cause irreversible tissue damage due to systemic inflammation. Recently, the Autoinflammatory Disease Damage Index (ADDI) was developed. The ADDI is the first instrument to quantify damage in familial Mediterranean fever, cryopyrin-associated periodic syndromes, mevalonate kinase deficiency and tumour necrosis factor receptor-associated periodic syndrome. The aim of this study was to validate this tool for its intended use in a clinical/research setting. The ADDI was scored on paper clinical cases by at least three physicians per case, independently of each other. Face and content validity were assessed by requesting comments on the ADDI. Reliability was tested by calculating the intraclass correlation coefficient (ICC) using an 'observer-nested-within-subject' design. Construct validity was determined by correlating the ADDI score to the Physician Global Assessment (PGA) of damage and disease activity. Redundancy of individual items was determined with Cronbach's alpha. The ADDI was validated on a total of 110 paper clinical cases by 37 experts in autoinflammatory diseases. This yielded an ICC of 0.84 (95% CI 0.78 to 0.89). The ADDI score correlated strongly with PGA-damage (r=0.92, 95% CI 0.88 to 0.95) and was not strongly influenced by disease activity (r=0.395, 95% CI 0.21 to 0.55). After comments from disease experts, some item definitions were refined. The interitem correlation in all different categories was lower than 0.7, indicating that there was no redundancy between individual damage items. The ADDI is a reliable and valid instrument to quantify damage in individual patients and can be used to compare disease outcomes in clinical studies. Ter Haar N.M. aut van Delft ALJ aut Annink K.V. aut van Stel H. aut Al-Mayouf S.M. aut Amaryan G. aut Anton J. aut Barron K.S. aut Benseler S. aut Brogan P.A. aut Cantarini L. aut Cattalini M. aut Cochino A.V. aut de Benedetti F. aut Dedeoglu F. aut de Jesus A.A. aut Demirkaya E. aut Dolezalova P. aut Durrant K.L. aut Fabio G. aut Gallizzi R. aut Goldbach-Mansky R. aut Hachulla E. aut Hentgen V. aut Herlin T. aut Hofer M. aut Hoffman H.M. aut Insalaco A. aut Jansson A.F. aut Kallinich T. aut Kone-Paut I. aut Kozlova A. aut Kuemmerle-Deschner J.B. aut Lachmann H.J. aut Laxer R.M. aut Martini A. aut Nielsen S. aut Nikishina I. aut Ombrello A.K. aut Özen S. aut Papadopoulou-Alataki E. aut Quartier P. aut Rigante D. aut Russo R. aut Simon A. aut Trachana M. aut Uziel Y. aut Ravelli A. aut Schulert G. aut Gattorno M. aut Frenkel J. aut Annals of the rheumatic diseases 77/11(2018-11), 1599-1605 77:11<1599-1605 2018 77 1 article serval SERVAL 700 1- Ter Haar N.M. aut SERVAL 700 1- van Delft ALJ aut SERVAL 700 1- Annink K.V. aut SERVAL 700 1- van Stel H. aut SERVAL 700 1- Al-Mayouf S.M. aut SERVAL 700 1- Amaryan G. aut SERVAL 700 1- Anton J. aut SERVAL 700 1- Barron K.S. aut SERVAL 700 1- Benseler S. aut SERVAL 700 1- Brogan P.A. aut SERVAL 700 1- Cantarini L. aut SERVAL 700 1- Cattalini M. aut SERVAL 700 1- Cochino A.V. aut SERVAL 700 1- de Benedetti F. aut SERVAL 700 1- Dedeoglu F. aut SERVAL 700 1- de Jesus A.A. aut SERVAL 700 1- Demirkaya E. aut SERVAL 700 1- Dolezalova P. aut SERVAL 700 1- Durrant K.L. aut SERVAL 700 1- Fabio G. aut SERVAL 700 1- Gallizzi R. aut SERVAL 700 1- Goldbach-Mansky R. aut SERVAL 700 1- Hachulla E. aut SERVAL 700 1- Hentgen V. aut SERVAL 700 1- Herlin T. aut SERVAL 700 1- Hofer M. aut SERVAL 700 1- Hoffman H.M. aut SERVAL 700 1- Insalaco A. aut SERVAL 700 1- Jansson A.F. aut SERVAL 700 1- Kallinich T. aut SERVAL 700 1- Kone-Paut I. aut SERVAL 700 1- Kozlova A. aut SERVAL 700 1- Kuemmerle-Deschner J.B. aut SERVAL 700 1- Lachmann H.J. aut SERVAL 700 1- Laxer R.M. aut SERVAL 700 1- Martini A. aut SERVAL 700 1- Nielsen S. aut SERVAL 700 1- Nikishina I. aut SERVAL 700 1- Ombrello A.K. aut SERVAL 700 1- Özen S. aut SERVAL 700 1- Papadopoulou-Alataki E. aut SERVAL 700 1- Quartier P. aut SERVAL 700 1- Rigante D. aut SERVAL 700 1- Russo R. aut SERVAL 700 1- Simon A. aut SERVAL 700 1- Trachana M. aut SERVAL 700 1- Uziel Y. aut SERVAL 700 1- Ravelli A. aut SERVAL 700 1- Schulert G. aut SERVAL 700 1- Gattorno M. aut SERVAL 700 1- Frenkel J. aut SERVAL 773 0- Annals of the rheumatic diseases 77/11(2018-11), 1599-1605 77:11<1599-1605 2018 77 BK010053 XK010053 XK010000 SERVAL SERVAL SERVAL article