Journal article

Health Assessment Questionnaire-Disability Index (HAQ-DI) use in modelling disease progression in diffuse cutaneous systemic sclerosis: an analysis from the EUSTAR database.

  • Allanore Y Service de Rhumatologie, Cochin Hospital, APHP, Paris Descartes University, 27 rue du Faubourg Saint Jacques, 75014, Paris, France. yannick.allanore@aphp.fr.
  • Bozzi S Sanofi R&D, Chilly-Mazarin, France.
  • Terlinden A Sanofi R&D, Chilly-Mazarin, France.
  • Huscher D Institute of Biometry and Clinical Epidemiology and Berlin Institute of Health, Charité - Universitaetsmedizin, Berlin, Germany.
  • Amand C Sanofi R&D, Chilly-Mazarin, France.
  • Soubrane C Sanofi R&D, Chilly-Mazarin, France.
  • Siegert E Charité University Hospital, Berlin, Germany.
  • Czirják L University of Pécs, Medical School, Pécs, Hungary.
  • Carreira PE Hospital University 12 October, Madrid, Spain.
  • Hachulla E University of Lille, Claude Huriez' Hospital, Lille Cedex, France.
  • Zanatta E University of Padova, Padova, Italy.
  • Li M Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, P.R. China.
  • Airò P Rheumatology and Clinical Immunology, Spedali Civili, Brescia, Italy.
  • Mendoza FA Jefferson Institute of Molecular Medicine, Thomas Jefferson University, Philadelphia, PA, USA.
  • Rosato E La Sapienza University, Polyclinic Umberto I, Rome, Italy.
  • Distler O Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
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  • 2020-10-29
Published in:
  • Arthritis research & therapy. - 2020
English BACKGROUND
Patients with diffuse cutaneous systemic sclerosis (dcSSc) have a poor prognosis. The importance of monitoring subjective measures of functioning and disability, such as the Health Assessment Questionnaire-Disability Index (HAQ-DI), is important as dcSSc is rated by patients as worse than diabetes or hemodialysis for quality of life impairment. This European Scleroderma Trials and Research (EUSTAR) database analysis was undertaken to examine the importance of impaired functionality in dcSSc prognosis. The primary objectives were to identify predictors of death and HAQ-DI score progression over 1 year. HAQ-DI score, major advanced organ involvement, and death rate were also used to develop a comprehensive model to predict lifetime dcSSc progression.


METHODS
This was an observational, longitudinal study in patients with dcSSc registered in EUSTAR. Death and HAQ-DI scores were, respectively, analyzed by Cox regression and linear regression analyses in relation to baseline covariates. A microsimulation Markov model was developed to estimate/predict natural progression of dcSSc over a patient's lifetime.


RESULTS
The analysis included dcSSc patients with (N = 690) and without (N = 4132) HAQ-DI score assessments from the EUSTAR database. Baseline HAQ-DI score, corticosteroid treatment, and major advanced organ involvement were predictive of death on multivariable analysis; a 1-point increase in baseline HAQ-DI score multiplied the risk of death by 2.7 (p <  0.001) and multiple advanced major organ involvement multiplied the risk of death by 2.8 (p <  0.05). Multivariable analysis showed that baseline modified Rodnan Skin Score (mRSS) and baseline HAQ-DI score were associated with HAQ-DI score progression at 1 year (p <  0.05), but there was no association between baseline organ involvement and HAQ-DI score progression at 1 year. HAQ-DI score, major advanced organ involvement, and death were successfully used to model long-term disease progression in dcSSc.


CONCLUSIONS
HAQ-DI score and major advanced organ involvement were comparable predictors of mortality risk in dcSSc. Baseline mRSS and baseline HAQ-DI score were predictive of HAQ-DI score progression at 1 year, indicating a correlation between these endpoints in monitoring disease progression. It is hoped that this EUSTAR analysis may change physician perception about the importance of the HAQ-DI score in dcSSc.
Language
  • English
Open access status
gold
Identifiers
Persistent URL
https://folia.unifr.ch/global/documents/26115
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