European multicentre study to define disease activity criteria for systemic sclerosis.* II: identification of disease activity variables and development of preliminary activity indexes

G. Valentini, A. Della Rossa, S. Bombardieri, W. Bencivelli, A. Silman, S. D'Angelo, M. Cerinic, J. Belch, C. Black, P. Bruhlmann, L. Czirjak, A. De Luca, A. Drosos, C. Ferri, A. Gabrielli, R. Giacomelli, G. Hayem, M. Inanc, N. McHugh, H. NielsenM. Rosada, R. Scorza, J. Stork, A. Sysa, F. H. J. van den Hoogen, P. Vlachoyiannopoulos

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    Abstract

    OBJECTIVE—To develop criteria for disease activity in systemic sclerosis (SSc) that are valid, reliable, and easy to use.

    METHODS—Investigators from 19 European centres completed a standardised clinical chart for a consecutive number of patients with SSc. Three protocol management members blindly evaluated each chart and assigned a disease activity score on a semiquantitative scale of 0-10. Two of them, in addition, gave a blinded, qualitative evaluation of disease activity ("inactive to moderately active" or "active to very active" disease). Both these evaluations were found to be reliable. A final disease activity score and qualitative evaluation of disease activity were arrived at by consensus for each patient; the former represented the gold standard for subsequent analyses. The correlations between individual items in the chart and this gold standard were then analysed.

    RESULTS—A total of 290 patients with SSc (117 with diffuse SSc (dSSc) and 173 with limited SSc (lSSc)) were enrolled in the study. The items (including ?-factors—that is, worsening according to the patient report) that were found to correlate with the gold standard on multiple regression were used to construct three separate 10-point indices of disease activity: (a) ?-cardiopulmonary (4.0), ?-skin (3.0), ?-vascular (2.0), and ?-articular/muscular (1.0) for patients with dSSc; (b) ?-skin (2.5), erythrocyte sedimentation rate (ESR) >30 mm/1st h (2.5), ?-cardiopulmonary (1.5), ?-vascular (1.0), arthritis (1.0), hypocomplementaemia (1.0), and scleredema (0.5) for lSSc; (c) ?-cardiopulmonary (2.0), ?-skin (2.0), ESR >30 mm/1st h (1.5), total skin score >20 (1.0), hypocomplementaemia (1.0), scleredema (0.5), digital necrosis (0.5),?-vascular (0.5), arthritis (0.5), TLCO <80% (0.5) for all patients with SSc. The three indexes were validated by the jackknife technique. Finally, receiver operating characteristic curves were constructed in order to define the value of the index with the best discriminant capacity for "active to very active" patients.

    CONCLUSIONS—Three feasible, reliable, and valid preliminary indices to define disease activity in SSc were constructed.

    Original languageEnglish
    Pages (from-to)592-598
    Number of pages7
    JournalAnnals of the Rheumatic Diseases
    Volume60
    Issue number6
    DOIs
    Publication statusPublished - Jun 2001

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    Systemic Scleroderma
    Multicenter Studies
    Skin
    Scleredema Adultorum
    Blood Vessels
    Blood Sedimentation
    Sedimentation
    Arthritis
    Diffuse Scleroderma
    ROC Curve
    Necrosis
    Joints

    Cite this

    Valentini, G. ; Della Rossa, A. ; Bombardieri, S. ; Bencivelli, W. ; Silman, A. ; D'Angelo, S. ; Cerinic, M. ; Belch, J. ; Black, C. ; Bruhlmann, P. ; Czirjak, L. ; De Luca, A. ; Drosos, A. ; Ferri, C. ; Gabrielli, A. ; Giacomelli, R. ; Hayem, G. ; Inanc, M. ; McHugh, N. ; Nielsen, H. ; Rosada, M. ; Scorza, R. ; Stork, J. ; Sysa, A. ; van den Hoogen, F. H. J. ; Vlachoyiannopoulos, P. / European multicentre study to define disease activity criteria for systemic sclerosis.* II : identification of disease activity variables and development of preliminary activity indexes. In: Annals of the Rheumatic Diseases. 2001 ; Vol. 60, No. 6. pp. 592-598.
    @article{901f176fdfec43879b399daedad4e69e,
    title = "European multicentre study to define disease activity criteria for systemic sclerosis.* II: identification of disease activity variables and development of preliminary activity indexes",
    abstract = "OBJECTIVE—To develop criteria for disease activity in systemic sclerosis (SSc) that are valid, reliable, and easy to use. METHODS—Investigators from 19 European centres completed a standardised clinical chart for a consecutive number of patients with SSc. Three protocol management members blindly evaluated each chart and assigned a disease activity score on a semiquantitative scale of 0-10. Two of them, in addition, gave a blinded, qualitative evaluation of disease activity ({"}inactive to moderately active{"} or {"}active to very active{"} disease). Both these evaluations were found to be reliable. A final disease activity score and qualitative evaluation of disease activity were arrived at by consensus for each patient; the former represented the gold standard for subsequent analyses. The correlations between individual items in the chart and this gold standard were then analysed. RESULTS—A total of 290 patients with SSc (117 with diffuse SSc (dSSc) and 173 with limited SSc (lSSc)) were enrolled in the study. The items (including ?-factors—that is, worsening according to the patient report) that were found to correlate with the gold standard on multiple regression were used to construct three separate 10-point indices of disease activity: (a) ?-cardiopulmonary (4.0), ?-skin (3.0), ?-vascular (2.0), and ?-articular/muscular (1.0) for patients with dSSc; (b) ?-skin (2.5), erythrocyte sedimentation rate (ESR) >30 mm/1st h (2.5), ?-cardiopulmonary (1.5), ?-vascular (1.0), arthritis (1.0), hypocomplementaemia (1.0), and scleredema (0.5) for lSSc; (c) ?-cardiopulmonary (2.0), ?-skin (2.0), ESR >30 mm/1st h (1.5), total skin score >20 (1.0), hypocomplementaemia (1.0), scleredema (0.5), digital necrosis (0.5),?-vascular (0.5), arthritis (0.5), TLCO <80{\%} (0.5) for all patients with SSc. The three indexes were validated by the jackknife technique. Finally, receiver operating characteristic curves were constructed in order to define the value of the index with the best discriminant capacity for {"}active to very active{"} patients. CONCLUSIONS—Three feasible, reliable, and valid preliminary indices to define disease activity in SSc were constructed.",
    author = "G. Valentini and {Della Rossa}, A. and S. Bombardieri and W. Bencivelli and A. Silman and S. D'Angelo and M. Cerinic and J. Belch and C. Black and P. Bruhlmann and L. Czirjak and {De Luca}, A. and A. Drosos and C. Ferri and A. Gabrielli and R. Giacomelli and G. Hayem and M. Inanc and N. McHugh and H. Nielsen and M. Rosada and R. Scorza and J. Stork and A. Sysa and {van den Hoogen}, {F. H. J.} and P. Vlachoyiannopoulos",
    year = "2001",
    month = "6",
    doi = "10.1136/ard.60.6.592",
    language = "English",
    volume = "60",
    pages = "592--598",
    journal = "Annals of the Rheumatic Diseases",
    issn = "0003-4967",
    publisher = "BMJ Publishing Group",
    number = "6",

    }

    Valentini, G, Della Rossa, A, Bombardieri, S, Bencivelli, W, Silman, A, D'Angelo, S, Cerinic, M, Belch, J, Black, C, Bruhlmann, P, Czirjak, L, De Luca, A, Drosos, A, Ferri, C, Gabrielli, A, Giacomelli, R, Hayem, G, Inanc, M, McHugh, N, Nielsen, H, Rosada, M, Scorza, R, Stork, J, Sysa, A, van den Hoogen, FHJ & Vlachoyiannopoulos, P 2001, 'European multicentre study to define disease activity criteria for systemic sclerosis.* II: identification of disease activity variables and development of preliminary activity indexes', Annals of the Rheumatic Diseases, vol. 60, no. 6, pp. 592-598. https://doi.org/10.1136/ard.60.6.592

    European multicentre study to define disease activity criteria for systemic sclerosis.* II : identification of disease activity variables and development of preliminary activity indexes. / Valentini, G.; Della Rossa, A.; Bombardieri, S.; Bencivelli, W.; Silman, A.; D'Angelo, S.; Cerinic, M. ; Belch, J.; Black, C.; Bruhlmann, P.; Czirjak, L.; De Luca, A.; Drosos, A.; Ferri, C.; Gabrielli, A.; Giacomelli, R.; Hayem, G.; Inanc, M.; McHugh, N.; Nielsen, H.; Rosada, M.; Scorza, R.; Stork, J.; Sysa, A.; van den Hoogen, F. H. J.; Vlachoyiannopoulos, P.

    In: Annals of the Rheumatic Diseases, Vol. 60, No. 6, 06.2001, p. 592-598.

    Research output: Contribution to journalArticle

    TY - JOUR

    T1 - European multicentre study to define disease activity criteria for systemic sclerosis.* II

    T2 - identification of disease activity variables and development of preliminary activity indexes

    AU - Valentini, G.

    AU - Della Rossa, A.

    AU - Bombardieri, S.

    AU - Bencivelli, W.

    AU - Silman, A.

    AU - D'Angelo, S.

    AU - Cerinic, M.

    AU - Belch, J.

    AU - Black, C.

    AU - Bruhlmann, P.

    AU - Czirjak, L.

    AU - De Luca, A.

    AU - Drosos, A.

    AU - Ferri, C.

    AU - Gabrielli, A.

    AU - Giacomelli, R.

    AU - Hayem, G.

    AU - Inanc, M.

    AU - McHugh, N.

    AU - Nielsen, H.

    AU - Rosada, M.

    AU - Scorza, R.

    AU - Stork, J.

    AU - Sysa, A.

    AU - van den Hoogen, F. H. J.

    AU - Vlachoyiannopoulos, P.

    PY - 2001/6

    Y1 - 2001/6

    N2 - OBJECTIVE—To develop criteria for disease activity in systemic sclerosis (SSc) that are valid, reliable, and easy to use. METHODS—Investigators from 19 European centres completed a standardised clinical chart for a consecutive number of patients with SSc. Three protocol management members blindly evaluated each chart and assigned a disease activity score on a semiquantitative scale of 0-10. Two of them, in addition, gave a blinded, qualitative evaluation of disease activity ("inactive to moderately active" or "active to very active" disease). Both these evaluations were found to be reliable. A final disease activity score and qualitative evaluation of disease activity were arrived at by consensus for each patient; the former represented the gold standard for subsequent analyses. The correlations between individual items in the chart and this gold standard were then analysed. RESULTS—A total of 290 patients with SSc (117 with diffuse SSc (dSSc) and 173 with limited SSc (lSSc)) were enrolled in the study. The items (including ?-factors—that is, worsening according to the patient report) that were found to correlate with the gold standard on multiple regression were used to construct three separate 10-point indices of disease activity: (a) ?-cardiopulmonary (4.0), ?-skin (3.0), ?-vascular (2.0), and ?-articular/muscular (1.0) for patients with dSSc; (b) ?-skin (2.5), erythrocyte sedimentation rate (ESR) >30 mm/1st h (2.5), ?-cardiopulmonary (1.5), ?-vascular (1.0), arthritis (1.0), hypocomplementaemia (1.0), and scleredema (0.5) for lSSc; (c) ?-cardiopulmonary (2.0), ?-skin (2.0), ESR >30 mm/1st h (1.5), total skin score >20 (1.0), hypocomplementaemia (1.0), scleredema (0.5), digital necrosis (0.5),?-vascular (0.5), arthritis (0.5), TLCO <80% (0.5) for all patients with SSc. The three indexes were validated by the jackknife technique. Finally, receiver operating characteristic curves were constructed in order to define the value of the index with the best discriminant capacity for "active to very active" patients. CONCLUSIONS—Three feasible, reliable, and valid preliminary indices to define disease activity in SSc were constructed.

    AB - OBJECTIVE—To develop criteria for disease activity in systemic sclerosis (SSc) that are valid, reliable, and easy to use. METHODS—Investigators from 19 European centres completed a standardised clinical chart for a consecutive number of patients with SSc. Three protocol management members blindly evaluated each chart and assigned a disease activity score on a semiquantitative scale of 0-10. Two of them, in addition, gave a blinded, qualitative evaluation of disease activity ("inactive to moderately active" or "active to very active" disease). Both these evaluations were found to be reliable. A final disease activity score and qualitative evaluation of disease activity were arrived at by consensus for each patient; the former represented the gold standard for subsequent analyses. The correlations between individual items in the chart and this gold standard were then analysed. RESULTS—A total of 290 patients with SSc (117 with diffuse SSc (dSSc) and 173 with limited SSc (lSSc)) were enrolled in the study. The items (including ?-factors—that is, worsening according to the patient report) that were found to correlate with the gold standard on multiple regression were used to construct three separate 10-point indices of disease activity: (a) ?-cardiopulmonary (4.0), ?-skin (3.0), ?-vascular (2.0), and ?-articular/muscular (1.0) for patients with dSSc; (b) ?-skin (2.5), erythrocyte sedimentation rate (ESR) >30 mm/1st h (2.5), ?-cardiopulmonary (1.5), ?-vascular (1.0), arthritis (1.0), hypocomplementaemia (1.0), and scleredema (0.5) for lSSc; (c) ?-cardiopulmonary (2.0), ?-skin (2.0), ESR >30 mm/1st h (1.5), total skin score >20 (1.0), hypocomplementaemia (1.0), scleredema (0.5), digital necrosis (0.5),?-vascular (0.5), arthritis (0.5), TLCO <80% (0.5) for all patients with SSc. The three indexes were validated by the jackknife technique. Finally, receiver operating characteristic curves were constructed in order to define the value of the index with the best discriminant capacity for "active to very active" patients. CONCLUSIONS—Three feasible, reliable, and valid preliminary indices to define disease activity in SSc were constructed.

    U2 - 10.1136/ard.60.6.592

    DO - 10.1136/ard.60.6.592

    M3 - Article

    C2 - 11350848

    VL - 60

    SP - 592

    EP - 598

    JO - Annals of the Rheumatic Diseases

    JF - Annals of the Rheumatic Diseases

    SN - 0003-4967

    IS - 6

    ER -