Abstract
We studied the relationships between physician-assessed damage, severity and activity in a large, multicentre systemic sclerosis (SSc, scleroderma) cohort. We hypothesized that there is a relationship between disease activity and severity and damage, but that severity would be more strongly related to damage. A total of 520 SSc patients (87% women, mean age 56 years, mean 8.6 years disease duration, 39% diffuse SSc) were studied. The correlations between physician's global assessments of damage, activity and severity were determined overall and in a subset of early, diffuse SSc (n = 74). The mean (SD) patient global health score was 3.6 (2.4) on a 0–10 scale. Physician-rated severity, activity and damage were 2.8 (2.2), 2.3 (2.0), and 3.4 (2.4) respectively. Damage was more strongly related to severity (r = 0.744, P < 0.001) than activity (r = 0.596, P < 0.001). Damage was not related to disease duration (r = 0.046, P = 0.3). In early diffuse SSc, the correlations were: damage and severity 0.771 (P < 0.001), damage and activity 0.596 (P < 0.001), severity and activity 0.809 (P < 0.001). The relationships vary in the overall cohort versus the early diffuse SSc subset where activity, severity and damage appear more strongly related. Thus, the exact nature of the relationship between damage, activity and severity will depend upon the characteristics of the population studied.
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References
Varga J, Abraham D (2007) Systemic sclerosis: a prototypic multisystem fibrotic disorder. J Clin Invest 117(3):557–567
LeRoy EC, Black C, Fleischmajer R et al (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15(2):202–205
Symmons DP (1995) Disease assessment indices: activity, damage and severity. Baillieres Clin Rheumatol 9:267–285
Medsger TA Jr (2000) Assessment of damage and activity in systemic sclerosis. Curr Opin Rheumatol 12:545–548
Guillemin F (2003) Assessment of disease activity. Best Pract Res Clin Rheumatol 17:415–426
Vonk MC, van den Hoogen FHJ, van Riel PLCM, Valentini G (2007) What does the clinician need to improve patient care in systemic sclerosis? Ann Rheum Dis 66:1129–1131
Hudson M, Steele R, Baron M (2007) Update on indices of disease activity in systemic sclerosis. Semin Arthritis Rheum 37(2):93–98
Valentini G, Matucci Cerinic M (2007) Disease-specific indicators, guidelines and outcome measures in scleroderma. Clin Exp Rheumatol 25(Suppl 47):S159–S162
Ruof J, Bruhlmann P, Michel BA, Stucki G (1999) Development and validation of a self-administered systemic sclerosis questionnaire (SySQ). Rheumatology (Oxford) 38:535–542
Valentini G, Della Rossa A, Bombardieri S et al (2001) European multicentre study to define disease activity criteria for systemic sclerosis. II. Identification of disease activity variables and development of preliminary activity indexes. Ann Rheum Dis 60:592–598
Merkel PA, Herlyn K, Martin RW et al (2002) Measuring disease activity and functional status in patients with scleroderma and Raynaud’s phenomenon. Arthritis Rheum 46:2410–2420
Rodnan GP, Lipinski E, Luksick J (1979) Skin thickness and collagen content in progressive systemic sclerosis and localized scleroderma. Arthritis Rheum 22:130–144
Clements PJ, Lachenbruch PA, Ng SC, Simmons M, Sterz M, Furst DE (1990) Skin score: a semiquantitative measure of cutaneous involvement that improves prediction of prognosis in systemic sclerosis. Arthritis Rheum 33:1256–1263
Steen V, Medsger TA Jr (1997) The value of the Health Assessment Questionnaire and special patient-generated scales to demonstrate change in systemic sclerosis patients over time. Arthritis Rheum 40:1984–1991
Medsger TA Jr, Silman AJ, Steen VD et al (1999) A disease severity scale for systemic sclerosis. Development and testing. J Rheumatol 26:2159–2167
Geirsson AJ, Wollheim FA, Akesson A (2001) Disease severity of 100 patients with systemic sclerosis over a period of 14 years: using a modified Medsger scale. Ann Rheum Dis 60:1117–1122
Gelber AC, Wigley FM (2002) Disease severity as a predictor of outcome in scleroderma. Lancet 359:277–279
Arkachaisri T, Pino S (2008) Localized scleroderma severity index and global assessments: a pilot study of outcome instruments. J Rheumatol 35:650–657
World Health Organization Disability Assessment Schedule II (WHODAS II) (2001) URL http://www.who.int/icidh/whodas/index.html
Ostojic P, Damjanov N (2006) The Scleroderma Assessment Questionnaire (SAQ). A new self-assessment questionnaire for evaluation of disease status in patients with systemic sclerosis. Z Rheumatol 65:168–175
Clements PJ, Seibold JR, Furst DE et al (2004) High-dose versus low-dose D-penicillamine in early diffuse systemic sclerosis trial: lessons learned. Semin Arthritis Rheum 33:249–263
Poole JL, Steen VD (1991) The use of the health assessment questionnaire (HAQ) to determine physical disability in systemic sclerosis. Arthritis Care Res 4:27–31
Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee (1980) Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 23:581–590
Valentini G, Bencivelli W, Bombardieri S et al (2003) European Scleroderma Study Group to define disease activity criteria for systemic sclerosis. III. Assessment of the construct validity of the preliminary activity criteria. Ann Rheum Dis 62:901–903
Clements PJ (2000) Systemic sclerosis (scleroderma) and related disorders: clinical aspects. Baillieres Clin Rheumatol 14:1–16
Derk CT, Jimenez SA (2003) Systemic sclerosis: current views of its pathogenesis. Autoimmun Rev 2:181–191
Denton CP, Black CM (2004) Scleroderma—clinical and pathological advances. Best Pract Res Clin Rheumatol 18:271–290
Johnson SR, Carette S, Dunne JV (2006) Scleroderma: health services utilization from patients’ perspective. J Rheumatol 33(6):1123–1127
Steen VD, Medsger TA Jr, Osial TA, Ziegler GL, Shapiro AP, Rodnan GP (1984) Factors predicting the development of renal involvement in progressive systemic sclerosis. Am J Med 76:779–786
Steen VD, Conte C, Owens GR, Medsger TA Jr (1994) Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 37:1283–1289
Ioannidis JPA, Vlachoyiannopoulos PG, Haidich A-B et al (2005) Mortality in systemic sclerosis: an international meta-analysis of individual patient data. Am J Med 118:2–10
Steen VD, Medsger TA Jr (2007) Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis 66:940–944
Henness S, Wigley FM (2007) Current drug therapy for scleroderma and secondary Raynaud’s phenomenon: evidence-based review. Curr Opin Rheumatol 19:611–618
Matucci-Cerinic M, Steen VD, Furst DE, Seibold JR (2007) Clinical trials in systemic sclerosis: lessons learned and outcomes. Arthritis Res Ther 9(Suppl):S7
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We thank the Canadian Institutes of Health Research and the Scleroderma Society of Canada for grant funding.
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The Canadian Scleroderma Research Group (CSRG) Investigators are listed in Appendix.
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The Canadian Scleroderma Research Group (CSRG) Investigators:
M. Abu-Hakima, Calgary, Alberta; M. Baron, Montreal, Quebec; P. Docherty, Moncton, New Brunswick; M. Fritzler, Advanced Diagnostics Laboratory, Calgary, Alberta; N. Jones, Edmonton, Alberta; E. Kaminska, Hamilton, Ontario; N. Khalidi, Hamilton, Ontario; S. LeClercq, Calgary, Alberta; S. Ligier, Montreal, Quebec; J. Markland, Saskatoon, Saskatchewan; A. Masetto, Sherbrooke, Quebec; J-P. Mathieu, Montreal, Quebec; J. Pope, London, Ontario; D. Robinson, Winnipeg, Manitoba; D. Smith, Ottawa, Ontario; E. Sutton, Halifax, Nova Scotia.
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Fan, X., Pope, J., The Canadian Scleroderma Research Group. et al. What is the relationship between disease activity, severity and damage in a large Canadian systemic sclerosis cohort? Results from the Canadian Scleroderma Research Group (CSRG). Rheumatol Int 30, 1205–1210 (2010). https://doi.org/10.1007/s00296-009-1129-7
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DOI: https://doi.org/10.1007/s00296-009-1129-7