Dataset Information

In silico validation of the Autoinflammatory Disease Damage Index.

ABSTRACT:

Introduction

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.

Methods

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.

Results

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.

Conclusion

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.

SUBMITTER: Ter Haar NM

PROVIDER: S-EPMC8411437 | biostudies-literature | 2018 Nov

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

In silico validation of the Autoinflammatory Disease Damage Index.

Ter Haar Nienke M NM van Delft Amber Laetitia Justine ALJ Annink Kim Valerie KV van Stel Henk H Al-Mayouf Sulaiman M SM Amaryan Gayane G Anton Jordi J Barron Karyl S KS Benseler Susanne S Brogan Paul A PA Cantarini Luca L Cattalini Marco M Cochino Alexis-Virgil AV de Benedetti Fabrizio F Dedeoglu Fatma F de Jesus Adriana Almeida AA Demirkaya Erkan E Dolezalova Pavla P Durrant Karen L KL Fabio Giovanna G Gallizzi Romina R Goldbach-Mansky Raphaela R Hachulla Eric E Hentgen Veronique V Herlin Troels T Hofer Michaël M Hoffman Hal M HM Insalaco Antonella A Jansson Annette F AF Kallinich Tilmann T Kone-Paut Isabelle I Kozlova Anna A Kuemmerle-Deschner Jasmin Beate JB Lachmann Helen J HJ Laxer Ronald M RM Martini Alberto A Nielsen Susan S Nikishina Irina I Ombrello Amanda K AK Özen Seza S Papadopoulou-Alataki Efimia E Quartier Pierre P Rigante Donato D Russo Ricardo R Simon Anna A Trachana Maria M Uziel Yosef Y Ravelli Angelo A Schulert Grant G Gattorno Marco M Frenkel Joost J

Annals of the rheumatic diseases 20180804 11

<h4>Introduction</h4>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 ...[more]

PMID: 30077992

Similar Datasets

Project description:Background & aimsThe Lémann Index is a tool measuring cumulative structural bowel damage in Crohn's disease (CD). We reported on its validation and updating.MethodsThis was an international, multicenter, prospective, cross-sectional observational study. At each center, 10 inclusions, stratified by CD duration and location, were planned. For each patient, the digestive tract was divided into 4 organs, upper tract, small bowel, colon/rectum, anus, and subsequently into segments, explored systematically by magnetic resonance imaging and by endoscopies in relation to disease location. For each segment, investigators retrieved information on previous surgical procedures, identified predefined strictures and penetrating lesions of maximal severity (grades 1-3) at each organ investigational method (gastroenterologist and radiologist for magnetic resonance imaging), provided segmental damage evaluation ranging from 0.0 to 10.0 (complete resection). Organ resection-free cumulative damage evaluation was then calculated from the sum of segmental damages. Then investigators provided a 0-10 global damage evaluation from the 4-organ standardized cumulative damage evaluations. Simple linear regressions of investigator damage evaluations on their corresponding Lémann Index were studied, as well as calibration plots. Finally, updated Lémann Index was derived through multiple linear mixed models applied to combined development and validation samples.ResultsIn 15 centers, 134 patients were included. Correlation coefficients between investigator damage evaluations and Lémann Indexes were >0.80. When analyzing data in 272 patients from both samples and 27 centers, the unbiased correlation estimates were 0.89, 0,97, 0,94, 0.81, and 0.91 for the 4 organs and globally, and stable when applied to one sample or the other.ConclusionsThe updated Lémann Index is a well-established index to assess cumulative bowel damage in CD that can be used in epidemiological studies and disease modification trials.

Dataset Information

In silico validation of the Autoinflammatory Disease Damage Index.

Introduction

Methods

Results

Conclusion

Publications

In silico validation of the Autoinflammatory Disease Damage Index.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets