Quality of Life in Systemic Sclerosis

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


 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.


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:


baseline dyspnea index


 Cochin hand function scale


hospital anxiety and depression


Health assessment questionnaire


Health related quality of life


International Classification of Functioning, Disability and Health


 McMaster-Toronto Arthritis Patient Preference Disability Questionnaire


mental component score


Mouth Handicap In Systemic Sclerosis


Medical Outcomes Study


physical component score


standard deviation


36-Item Short-Form Health Survey


scleroderma HAQ


systemic sclerosis


limited SSc


limited cutaneous SSc


diffuse SSc


visual analog scale


World Health Organization Disability Assessment Schedule II



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


  1. Tamby MC, Chanseaud Y, Guillevin L, et al. (2003). Autoimmun Rev. 2: 152–157.PubMedCrossRefGoogle Scholar
  2. LeRoy EC, Medsger TA, Jr. (2001). J Rheumatol. 28: 1573–1576.PubMedGoogle Scholar
  3. Masi AT. (1988). J Rheumatol. 15: 894–898.PubMedGoogle Scholar
  4. Hachulla E, Gressin V, Guillevin L, et al. (2005). Arthritis Rheum. 52: 3792–3800.PubMedCrossRefGoogle Scholar
  5. Steen VD, Medsger TA Jr. (2000). Arthritis Rheum. 43: 2437–2444.PubMedCrossRefGoogle Scholar
  6. Steen VD, Medsger TA. (2000). Ann Intern Med. 133: 600–603.PubMedGoogle Scholar
  7. Bouros D, Wells AU, Nicholson AG, et al. (2002). Am J Respir Crit Care Med. 165: 1581–1586.PubMedCrossRefGoogle Scholar
  8. Poole JL, Steen VD. (1991). Arthritis Care Res. 4: 27–31.PubMedCrossRefGoogle Scholar
  9. Guyatt GH, Feeny DH, Patrick DL. (1993). Ann Intern Med. 118: 622–629.PubMedGoogle Scholar
  10. O'Reilly S, Dogherty M. (2003). Signs, symptoms and laboratory tests. K. Brandt, M. Dogherty, and L. Lohmander, (Ed). Osteoarthritis. 2nd Edn, In:Oxford University Press: New-York. p. 197–210.Google Scholar
  11. Xie F, Li SC, Fong KY, et al. (2006). Osteoarthritis Cartilage. 14: 224–230.PubMedCrossRefGoogle Scholar
  12. Ware J, Snow K, Kosinski M, et al. (1993). SF-36 Health Survey: manual and interpretation guide. The Health Institute, New England Medical Center.Boston:Google Scholar
  13. Kosinski M, Keller SD, Hatoum HT, et al. (1999). Med Care. 37: MS10–MS22.PubMedCrossRefGoogle Scholar
  14. Patrick DL, Deyo RA. (1989). Med Care. 27: S217–S232.PubMedCrossRefGoogle Scholar
  15. Ware JE Jr., Sherbourne CD. (1992). Med Care. 30: 473–483.PubMedCrossRefGoogle Scholar
  16. Kosinski M, Keller SD, Ware JE Jr., et al. (1999). Med Care. 37: MS23–MS39.PubMedCrossRefGoogle Scholar
  17. Del Rosso A, Boldrini M, D'Agostino D, et al. (2004). Arthritis Rheum. 51: 475–481.PubMedCrossRefGoogle Scholar
  18. Danieli E, Airo P, Bettoni L, et al. (2005). Clin Rheumatol. 24: 48–54.PubMedCrossRefGoogle Scholar
  19. Georges C, Chassany O, Mouthon L, et al. (2004). Rev Med Interne. 25: 16–21.PubMedCrossRefGoogle Scholar
  20. Khanna D, Furst DE, Clements PJ, et al. (2005). J Rheumatol. 32: 832–840.PubMedGoogle Scholar
  21. Khanna D, Clements PJ, Furst DE, et al. (2005). Arthritis Rheum. 52: 592–600.PubMedCrossRefGoogle Scholar
  22. Merkel PA, Clements PJ, Reveille JD, et al. (2003). Report from OMERACT 6. J Rheumatol. 30: 1630–1647.Google Scholar
  23. Khanna D, Ahmed M, Furst DE, et al. (2007). Arthritis Rheum. 57: 86–93.PubMedCrossRefGoogle Scholar
  24. Rannou F, Poiraudeau S, Berezné A, et al. (2007). Arthritis Rheum. 57(1): 94–102. [Epub ahead of print]. PubMedCrossRefGoogle Scholar
  25. Bowling A, Bond M, Jenkinson C, et al. (1999). J Public Health Med. 21: 255–270.PubMedCrossRefGoogle Scholar
  26. McHorney CA, Ware JE Jr., Raczek AE. (1993). Med Care. 31: 247–263.PubMedCrossRefGoogle Scholar
  27. International Classification of Functioning, Disability, and Health., in World Health Organization, n. ed., Editor. 2001: Geneva.Google Scholar
  28. van Tubergen A, Landewe R, Heuft-Dorenbosch L, et al. (2003). Ann Rheum Dis. 62: 140–145.PubMedCrossRefGoogle Scholar
  29. Chwastiak LA, Von Korff M. (2003). J Clin Epidemiol. 56: 507–514.PubMedCrossRefGoogle Scholar
  30. Hudson M, Steele R, Taillefer S, et al. (2008). Arthritis Rheum. 59: 270–278.PubMedCrossRefGoogle Scholar
  31. Hudson M, Thombs BD, Steele R, et al. (2008). Arthritis Rheum. 59: 279–284.PubMedCrossRefGoogle Scholar
  32. Khanna D, Yan X, Tashkin DP, et al. (2007). Arthritis Rheum. 56: 1676–1684.PubMedCrossRefGoogle Scholar
  33. Kaplan RM, Atkins CJ, Timms R. (1984). J Chronic Dis. 37: 85–95.PubMedCrossRefGoogle Scholar
  34. Jones PW, Quirk FH, Baveystock CM, et al. (1992). Am Rev Respir Dis. 145: 1321–1327.PubMedGoogle Scholar
  35. Jones PW, Quirk FH, Baveystock CM. (1991). Respir Med. 85(B): 25–31; discussion 33–7.PubMedCrossRefGoogle Scholar
  36. Nishiyama O, Taniguchi H, Kondoh Y, et al. (2005). Respir Med. 99: 408–414.PubMedCrossRefGoogle Scholar
  37. Chang JA, Curtis JR, Patrick DL, et al. (1999). Chest. 116: 1175–1182.PubMedCrossRefGoogle Scholar
  38. Tzanakis N, Samiou M, Lambiri I, et al. (2005). Eur J Intern Med. 16: 105–112.PubMedCrossRefGoogle Scholar
  39. Beretta L, Santaniello A, Lemos A, Masciocchi M, Scorza R. (2007). Rheumatology (Oxford) 46 2: 296–301.Google Scholar
  40. Poole JL, William CA, Bloch DA, et al. (1995). Arthritis Care Res. 8: 189–193.PubMedCrossRefGoogle Scholar
  41. Merkel PA, Herlyn K, Martin RW, et al. (2002). Arthritis Rheum. 46: 2410–2420.PubMedCrossRefGoogle Scholar
  42. Steen VD, Medsger TA. (1997). Arthritis Rheum. 40: 1984–1991.PubMedCrossRefGoogle Scholar
  43. Clements PJ, Wong WK, Hurwitz EL, et al. (2001). Arthritis Rheum. 44: 653–61.PubMedCrossRefGoogle Scholar
  44. Brower LM, Poole JL. (2004). Arthritis Rheum. 51: 805–809.PubMedCrossRefGoogle Scholar
  45. Mouthon L, Rannou F, Berezne A, et al. (2007). Ann Rheum Dis. 66: 1651–1655.PubMedCrossRefGoogle Scholar
  46. Tugwell P, Bombardier C, Buchanan WW, et al. J Rheumatol. 14: 446–451.Google Scholar
  47. Hewlett S, Smith AP, Kirwan JR. (2001). Ann Rheum Dis. 60: 928–933.PubMedCrossRefGoogle Scholar
  48. Verhoeven AC, Boers M, van der Liden S. (2000). J Rheumatol. 27: 2801–2809.PubMedGoogle Scholar
  49. Mouthon L, Rannou F, Berezne A, et al. (2008). Arthritis Rheum. 59: 968–973.PubMedCrossRefGoogle Scholar

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

Personalised recommendations