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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.

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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

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Acknowledgements

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

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© 2010 Springer Science+Business Media LLC

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Mouthon, L., Rannou, F., Berezné, A., Poiraudeau, S. (2010). Quality of Life in Systemic Sclerosis. In: Preedy, V.R., Watson, R.R. (eds) Handbook of Disease Burdens and Quality of Life Measures. Springer, New York, NY. https://doi.org/10.1007/978-0-387-78665-0_222

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  • DOI: https://doi.org/10.1007/978-0-387-78665-0_222

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-0-387-78664-3

  • Online ISBN: 978-0-387-78665-0

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