Development of the autoinflammatory disease damage index (ADDI)

Nienke M. Ter Haar; Kim V. Annink; Sulaiman M Al-Mayouf; Gayane Amaryan; Jordi Anton; Karyl S. Barron; Susanne M. Benseler; Paul A. Brogan; Luca Cantarini; Marco Cattalini; Alexis-Virgil Cochino; Fabrizio De Benedetti; Fatma Dedeoglu; Adriana Almeida de Jesus; Ornella Della Casa Alberighi; Erkan Demirkaya; Pavla Dolezalova; Karen L Durrant; Giovanna Fabio; Romina Gallizzi; Raphaela Goldbach-Mansky; Eric Hachulla; Veronique Hentgen; Troels Herlin; Michaël Hofer; Hal M Hoffman; Antonella Insalaco; Annette F Jansson; Tilmann Kallinich; Isabelle Koné-Paut; Anna Kozlova; Jasmin B. Kuemmerle-Deschner; Helen J. Lachmann; Ronald M Laxer; Alberto Martini; Susan Nielsen; Irina Nikishina; Amanda K Ombrello; Seza Ozen; Efimia Papadopoulou-Alataki; Pierre Quartier; Donato Rigante; Ricardo Russo; Anna Simon; Maria Trachana; Yosef Uziel; Angelo Ravelli; Marco Gattorno; Joost Frenkel

doi:10.1136/annrheumdis-2016-210092

Development of the autoinflammatory disease damage index (ADDI)

Nienke M. Ter Haar^*, Kim V. Annink, Sulaiman M Al-Mayouf, Gayane Amaryan, Jordi Anton, Karyl S. Barron, Susanne M. Benseler, Paul A. Brogan, Luca Cantarini, Marco Cattalini, Alexis-Virgil Cochino, Fabrizio De Benedetti, Fatma Dedeoglu, Adriana Almeida de Jesus, Ornella Della Casa Alberighi, Erkan Demirkaya, Pavla Dolezalova, Karen L Durrant, Giovanna Fabio, Romina GallizziRaphaela Goldbach-Mansky, Eric Hachulla, Veronique Hentgen, Troels Herlin, Michaël Hofer, Hal M Hoffman, Antonella Insalaco, Annette F Jansson, Tilmann Kallinich, Isabelle Koné-Paut, Anna Kozlova, Jasmin B. Kuemmerle-Deschner, Helen J. Lachmann, Ronald M Laxer, Alberto Martini, Susan Nielsen, Irina Nikishina, Amanda K Ombrello, Seza Ozen, Efimia Papadopoulou-Alataki, Pierre Quartier, Donato Rigante, Ricardo Russo, Anna Simon, Maria Trachana, Yosef Uziel, Angelo Ravelli, Marco Gattorno, Joost Frenkel

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

Abstract

Objectives Autoinflammatory diseases cause systemic inflammation that can result in damage to multiple organs. A validated instrument is essential to quantify damage in individual patients and to compare disease outcomes in clinical studies. Currently, there is no such tool. Our objective was to develop a common autoinflammatory disease damage index (ADDI) for familial Mediterranean fever, cryopyrin-associated periodic syndromes, tumour necrosis factor receptor-associated periodic fever syndrome and mevalonate kinase deficiency. Methods We developed the ADDI by consensus building. The top 40 enrollers of patients in the Eurofever Registry and 9 experts from the Americas participated in multiple rounds of online surveys to select items and definitions. Further, 22 (parents of) patients rated damage items and suggested new items. A consensus meeting was held to refine the items and definitions, which were then formally weighted in a scoring system derived using decision-making software, known as 1000minds. Results More than 80% of the experts and patients completed the online surveys. The preliminary ADDI contains 18 items, categorised in the following eight organ systems: Reproductive, renal/amyloidosis, developmental, serosal, neurological, ears, ocular and musculoskeletal damage. The categories renal/amyloidosis and neurological damage were assigned the highest number of points, serosal damage the lowest number of points. The involvement of (parents of) patients resulted in the inclusion of, for example, chronic musculoskeletal pain. Conclusions An instrument to measure damage caused by autoinflammatory diseases is developed based on consensus building. Patients fulfilled a significant role in this process.

Original language	English
Pages (from-to)	821-830
Number of pages	10
Journal	Annals of the Rheumatic Diseases
Volume	76
Issue number	5
DOIs	https://doi.org/10.1136/annrheumdis-2016-210092
Publication status	Published - 1 May 2017

Keywords

Familial Mediterranean Fever
Fever Syndromes
Outcomes research
Patient perspective

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10.1136/annrheumdis-2016-210092

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Ter Haar, N. M., Annink, K. V., Al-Mayouf, S. M., Amaryan, G., Anton, J., Barron, K. S., Benseler, S. M., Brogan, P. A., Cantarini, L., Cattalini, M., Cochino, A.-V., De Benedetti, F., Dedeoglu, F., de Jesus, A. A., Della Casa Alberighi, O., Demirkaya, E., Dolezalova, P., Durrant, K. L., Fabio, G., ... Frenkel, J. (2017). Development of the autoinflammatory disease damage index (ADDI). Annals of the Rheumatic Diseases, 76(5), 821-830. https://doi.org/10.1136/annrheumdis-2016-210092

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title = "Development of the autoinflammatory disease damage index (ADDI)",

abstract = "Objectives Autoinflammatory diseases cause systemic inflammation that can result in damage to multiple organs. A validated instrument is essential to quantify damage in individual patients and to compare disease outcomes in clinical studies. Currently, there is no such tool. Our objective was to develop a common autoinflammatory disease damage index (ADDI) for familial Mediterranean fever, cryopyrin-associated periodic syndromes, tumour necrosis factor receptor-associated periodic fever syndrome and mevalonate kinase deficiency. Methods We developed the ADDI by consensus building. The top 40 enrollers of patients in the Eurofever Registry and 9 experts from the Americas participated in multiple rounds of online surveys to select items and definitions. Further, 22 (parents of) patients rated damage items and suggested new items. A consensus meeting was held to refine the items and definitions, which were then formally weighted in a scoring system derived using decision-making software, known as 1000minds. Results More than 80% of the experts and patients completed the online surveys. The preliminary ADDI contains 18 items, categorised in the following eight organ systems: Reproductive, renal/amyloidosis, developmental, serosal, neurological, ears, ocular and musculoskeletal damage. The categories renal/amyloidosis and neurological damage were assigned the highest number of points, serosal damage the lowest number of points. The involvement of (parents of) patients resulted in the inclusion of, for example, chronic musculoskeletal pain. Conclusions An instrument to measure damage caused by autoinflammatory diseases is developed based on consensus building. Patients fulfilled a significant role in this process.",

keywords = "Familial Mediterranean Fever, Fever Syndromes, Outcomes research, Patient perspective",

author = "{Ter Haar}, {Nienke M.} and Annink, {Kim V.} and Al-Mayouf, {Sulaiman M} and Gayane Amaryan and Jordi Anton and Barron, {Karyl S.} and Benseler, {Susanne M.} and Brogan, {Paul A.} and Luca Cantarini and Marco Cattalini and Alexis-Virgil Cochino and {De Benedetti}, Fabrizio and Fatma Dedeoglu and {de Jesus}, {Adriana Almeida} and {Della Casa Alberighi}, Ornella and Erkan Demirkaya and Pavla Dolezalova and Durrant, {Karen L} and Giovanna Fabio and Romina Gallizzi and Raphaela Goldbach-Mansky and Eric Hachulla and Veronique Hentgen and Troels Herlin and Micha{\"e}l Hofer and Hoffman, {Hal M} and Antonella Insalaco and Jansson, {Annette F} and Tilmann Kallinich and Isabelle Kon{\'e}-Paut and Anna Kozlova and Kuemmerle-Deschner, {Jasmin B.} and Lachmann, {Helen J.} and Laxer, {Ronald M} and Alberto Martini and Susan Nielsen and Irina Nikishina and Ombrello, {Amanda K} and Seza Ozen and Efimia Papadopoulou-Alataki and Pierre Quartier and Donato Rigante and Ricardo Russo and Anna Simon and Maria Trachana and Yosef Uziel and Angelo Ravelli and Marco Gattorno and Joost Frenkel",

note = "Publisher Copyright: {\textcopyright} 2017 Published by the BMJ Publishing Group Limited.",

year = "2017",

month = may,

day = "1",

doi = "10.1136/annrheumdis-2016-210092",

language = "English",

volume = "76",

pages = "821--830",

journal = "Annals of the Rheumatic Diseases",

issn = "0003-4967",

publisher = "Elsevier",

number = "5",

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Ter Haar, NM, Annink, KV, Al-Mayouf, SM, Amaryan, G, Anton, J, Barron, KS, Benseler, SM, Brogan, PA, Cantarini, L, Cattalini, M, Cochino, A-V, De Benedetti, F, Dedeoglu, F, de Jesus, AA, Della Casa Alberighi, O, Demirkaya, E, Dolezalova, P, Durrant, KL, Fabio, G, Gallizzi, R, Goldbach-Mansky, R, Hachulla, E, Hentgen, V, Herlin, T, Hofer, M, Hoffman, HM, Insalaco, A, Jansson, AF, Kallinich, T, Koné-Paut, I, Kozlova, A, Kuemmerle-Deschner, JB, Lachmann, HJ, Laxer, RM, Martini, A, Nielsen, S, Nikishina, I, Ombrello, AK, Ozen, S, Papadopoulou-Alataki, E, Quartier, P, Rigante, D, Russo, R, Simon, A, Trachana, M, Uziel, Y, Ravelli, A, Gattorno, M & Frenkel, J 2017, 'Development of the autoinflammatory disease damage index (ADDI)', Annals of the Rheumatic Diseases, vol. 76, no. 5, pp. 821-830. https://doi.org/10.1136/annrheumdis-2016-210092

TY - JOUR

T1 - Development of the autoinflammatory disease damage index (ADDI)

AU - Ter Haar, Nienke M.

AU - Annink, Kim V.

AU - Al-Mayouf, Sulaiman M

AU - Amaryan, Gayane

AU - Anton, Jordi

AU - Barron, Karyl S.

AU - Benseler, Susanne M.

AU - Brogan, Paul A.

AU - Cantarini, Luca

AU - Cattalini, Marco

AU - Cochino, Alexis-Virgil

AU - De Benedetti, Fabrizio

AU - Dedeoglu, Fatma

AU - de Jesus, Adriana Almeida

AU - Della Casa Alberighi, Ornella

AU - Demirkaya, Erkan

AU - Dolezalova, Pavla

AU - Durrant, Karen L

AU - Fabio, Giovanna

AU - Gallizzi, Romina

AU - Goldbach-Mansky, Raphaela

AU - Hachulla, Eric

AU - Hentgen, Veronique

AU - Herlin, Troels

AU - Hofer, Michaël

AU - Hoffman, Hal M

AU - Insalaco, Antonella

AU - Jansson, Annette F

AU - Kallinich, Tilmann

AU - Koné-Paut, Isabelle

AU - Kozlova, Anna

AU - Kuemmerle-Deschner, Jasmin B.

AU - Lachmann, Helen J.

AU - Laxer, Ronald M

AU - Martini, Alberto

AU - Nielsen, Susan

AU - Nikishina, Irina

AU - Ombrello, Amanda K

AU - Ozen, Seza

AU - Papadopoulou-Alataki, Efimia

AU - Quartier, Pierre

AU - Rigante, Donato

AU - Russo, Ricardo

AU - Simon, Anna

AU - Trachana, Maria

AU - Uziel, Yosef

AU - Ravelli, Angelo

AU - Gattorno, Marco

AU - Frenkel, Joost

PY - 2017/5/1

Y1 - 2017/5/1

N2 - Objectives Autoinflammatory diseases cause systemic inflammation that can result in damage to multiple organs. A validated instrument is essential to quantify damage in individual patients and to compare disease outcomes in clinical studies. Currently, there is no such tool. Our objective was to develop a common autoinflammatory disease damage index (ADDI) for familial Mediterranean fever, cryopyrin-associated periodic syndromes, tumour necrosis factor receptor-associated periodic fever syndrome and mevalonate kinase deficiency. Methods We developed the ADDI by consensus building. The top 40 enrollers of patients in the Eurofever Registry and 9 experts from the Americas participated in multiple rounds of online surveys to select items and definitions. Further, 22 (parents of) patients rated damage items and suggested new items. A consensus meeting was held to refine the items and definitions, which were then formally weighted in a scoring system derived using decision-making software, known as 1000minds. Results More than 80% of the experts and patients completed the online surveys. The preliminary ADDI contains 18 items, categorised in the following eight organ systems: Reproductive, renal/amyloidosis, developmental, serosal, neurological, ears, ocular and musculoskeletal damage. The categories renal/amyloidosis and neurological damage were assigned the highest number of points, serosal damage the lowest number of points. The involvement of (parents of) patients resulted in the inclusion of, for example, chronic musculoskeletal pain. Conclusions An instrument to measure damage caused by autoinflammatory diseases is developed based on consensus building. Patients fulfilled a significant role in this process.

AB - Objectives Autoinflammatory diseases cause systemic inflammation that can result in damage to multiple organs. A validated instrument is essential to quantify damage in individual patients and to compare disease outcomes in clinical studies. Currently, there is no such tool. Our objective was to develop a common autoinflammatory disease damage index (ADDI) for familial Mediterranean fever, cryopyrin-associated periodic syndromes, tumour necrosis factor receptor-associated periodic fever syndrome and mevalonate kinase deficiency. Methods We developed the ADDI by consensus building. The top 40 enrollers of patients in the Eurofever Registry and 9 experts from the Americas participated in multiple rounds of online surveys to select items and definitions. Further, 22 (parents of) patients rated damage items and suggested new items. A consensus meeting was held to refine the items and definitions, which were then formally weighted in a scoring system derived using decision-making software, known as 1000minds. Results More than 80% of the experts and patients completed the online surveys. The preliminary ADDI contains 18 items, categorised in the following eight organ systems: Reproductive, renal/amyloidosis, developmental, serosal, neurological, ears, ocular and musculoskeletal damage. The categories renal/amyloidosis and neurological damage were assigned the highest number of points, serosal damage the lowest number of points. The involvement of (parents of) patients resulted in the inclusion of, for example, chronic musculoskeletal pain. Conclusions An instrument to measure damage caused by autoinflammatory diseases is developed based on consensus building. Patients fulfilled a significant role in this process.

KW - Familial Mediterranean Fever

KW - Fever Syndromes

KW - Outcomes research

KW - Patient perspective

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U2 - 10.1136/annrheumdis-2016-210092

DO - 10.1136/annrheumdis-2016-210092

M3 - Article

C2 - 27811147

SN - 0003-4967

VL - 76

SP - 821

EP - 830

JO - Annals of the Rheumatic Diseases

JF - Annals of the Rheumatic Diseases

IS - 5

ER -

Development of the autoinflammatory disease damage index (ADDI)

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