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Annals of the Rheumatic Diseases logoLink to Annals of the Rheumatic Diseases
. 2001 Jun;60(6):585–591. doi: 10.1136/ard.60.6.585

European multicentre study to define disease activity criteria for systemic sclerosis.* I. Clinical and epidemiological features of 290 patients from 19 centres

R Della 1, G Valentini 1, S Bombardieri 1, W Bencivelli 1, A Silman 1, S D'Angelo 1, M Cerinic 1, J Belch 1, C Black 1, R Becvar 1, P Bruhlman 1, F Cozzi 1, L Czirjak 1, A Drosos 1, B Dziankowska 1, C Ferri 1, A Gabrielli 1, R Giacomelli 1, G Hayem 1, M Inanc 1, N McHugh 1, H Nielsen 1, R Scorza 1, E Tirri 1, F H J van den Hoogen 1, P Vlachoyiannopoulo 1
PMCID: PMC1753678  PMID: 11350847

Abstract

OBJECTIVE—To investigate the existence of differences among European referral centres for systemic sclerosis (SSc) in the pattern of attendance and referral and in the clinical and therapeutical approaches.
METHODS—In 1995 the European Scleroderma Study Group initiated a multicentre prospective one year study whose aim was to define the disease activity criteria in SSc. During the study period each participating European centre was asked to enrol consecutive patients satisfying American College of Rheumatology criteria for SSc and to fill out for each of them a standardised clinical chart. Patients from various centres were compared and differences in epidemiological, clinical, and therapeutical aspects were analysed.
RESULTS—Nineteen different medical research centres consecutively recruited 290 patients. The patients could be divided into two subgroups: 173 with the limited (lSSc) and 117 with the diffuse (dSSc) form of the disease. The clinical and serological findings for the series of 290 patients seemed to be similar to data previously reported. However, when the data were analysed to elicit any differences between the participating centres, a high degree of variability emerged, in both epidemiological and clinical features and in the diagnostic and therapeutic approaches to the disease.
CONCLUSIONS—The clinical approach to SSc, not only in different countries but also in different centres within the same country, is not yet standardised. To overcome this problem, it will be necessary for the scientific community to draw up a standardised procedure for the management of patients with SSc. This would provide a common research tool for different centres engaged in research on this complex disease.



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Figure 1  .

Figure 1  

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Distribution of the patients by (A) age at enrolment and (B) disease duration among the different centres. In the box plots the 10th, 25th, 50th (median), 75th, and 90th centiles for each variable are shown. Values above the 90th and below the 10th centiles are plotted as points.

Figure 2  .

Figure 2  

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Cumulative prevalence of organ involvement based on data from the patient history section—that is, collected at the time of entry into the study.

Figure 3  .

Figure 3  

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Distribution of the total skin score among the different centres. In the box plots the 10th, 25th, 50th (median), 75th, and 90th centiles for each variable are shown. Values above the 90th and below the 10th centiles are plotted as points.

Figure 4  .

Figure 4  

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Distribution of clinical features among the different centres as emerged by data collected in section II (clinical and instrumental investigations carried out at the entry into the study). In the box plots the 10th, 25th, 50th (median), 75th, 90th centiles for each variable are shown. Values above the 90th and below the 10th centiles are plotted as points.

Figure 5  .

Figure 5  

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Drug treatment being used in the patient series at the time of enrolment.

Figure 6  .

Figure 6  

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Treatments used by the different centres. In the box plots the 10th, 25th, 50th (median), 75th, and 90th centiles of each variable are shown. Values above the 90th and below the 10th centiles are plotted as points.

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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