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Quality of Life in Systemic Sclerosis

  • L. Mouthon
  • F. Rannou
  • A. Berezné
  • S. Poiraudeau
Reference work entry

Abstract:

 Systemic sclerosis (SSc) is a connective-tissue disease characterized by excessive collagen deposition and by vascular hyper-reactivity and obliterative microvascular phenomena. In addition to diminishing life expectancy, SSc is responsible for skin, tendon, joint, and vessel damage, which leads to disability, handicap, and worsening of quality of life.

Health related quality of life can be measured in systemic sclerosis patients by using the Medical Outcomes Study 36-Item Short-Form Health Survey and/or the World Health Organization Disability Assessment Schedule II. Health related quality of life has been reported to be compromised in patients with systemic sclerosis.

Not surprisingly, health related quality of life, when measured using the 36-Item Short-Form Health Survey, has been reported to be compromised in patients with systemic sclerosis, and the use of the physical component score of the 36-Item Short-Form Health Survey has been shown to discriminate between patients with limited cutaneous SSc and those with diffuse systemic sclerosis. However, the relevance of using the two aggregate scores, physical component score and the mental component score has not been demonstrated in SSc.

The Health Organization Disability Assessment Schedule II is a multidimensional instrument which has good ability to discriminate between subsets of systemic sclerosis ( Classification of Systemic Sclerosis) patients with less severe disease compared with those with more severe disease. In these patients, significant predictors of the Health Organization Disability Assessment Schedule II are skin scores, shortness of breath, number of gastrointestinal problems, fatigue, pain, and depression.

In systemic sclerosis patients health related quality of life is significantly impaired as compared with the healthy population as assessed by the 36-Item Short-Form Health Survey and/or the Health Organization Disability Assessment Schedule II. Whether one of the two or both of these tests are necessary remains to be determined.

Keywords

Idiopathic Pulmonary Fibrosis Interstitial Lung Disease Health Assessment Questionnaire Mental Component Score Physical Component Score 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

List of Abbreviations:

BDI

baseline dyspnea index

CHFS

 Cochin hand function scale

HAD

hospital anxiety and depression

HAQ

Health assessment questionnaire

HRQOL

Health related quality of life

ICF

International Classification of Functioning, Disability and Health

MACTAR

 McMaster-Toronto Arthritis Patient Preference Disability Questionnaire

MCS

mental component score

MHISS

Mouth Handicap In Systemic Sclerosis

MOS

Medical Outcomes Study

PCS

physical component score

SD

standard deviation

SF-36

36-Item Short-Form Health Survey

sHAQ

scleroderma HAQ

SSc

systemic sclerosis

lSSc

limited SSc

lcSSc

limited cutaneous SSc

dSSc

diffuse SSc

VAS

visual analog scale

WHODAS II

World Health Organization Disability Assessment Schedule II

Notes

Acknowledgements

We thank the Association des sclérodermiques de France for its support.

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Copyright information

© Springer Science+Business Media LLC 2010

Authors and Affiliations

  • L. Mouthon
    • 1
  • F. Rannou
    • 2
  • A. Berezné
    • 1
  • S. Poiraudeau
    • 2
  1. 1.Department of Internal MedicineParis Descartes University, Reference Center for Necrotizing Vasculitides and Systemic Sclerosis, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP)ParisFrance
  2. 2.Department of RehabilitationParis Descartes University, Cochin Hospital, AP-HP, INSERM Institut Féderatif de Recherche sur le Handicap (IFR 25)NashvilleParis

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