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The development of systemic sclerosis classification criteria

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Abstract

Systemic sclerosis (SSc) is a rare connective tissue disorder whose aetiology remains obscure, although environmental and genetic influences are likely to play a role. Disease registries have contributed to enhancing our understanding of this debilitating illness, but without sensitive, specific, and extensively validated classification criteria, accurate comparison between registries and the identification of patients suitable for clinical trials can be problematic. The American College of Rheumatology (ACR) criteria, published in 1980, have become outdated as our understanding of disease specific autoantibodies and nailfold capillaroscopy has improved. In addition, the sensitivity of the ACR criteria is low with respect to limited SSc. Although subsequent classification systems have been proposed, none has gained universal approval. The two- versus three-subset disease model remains a point of debate. Newly derived criteria are likely to draw upon the older classification systems as well as incorporating up-to-date diagnostic techniques and biomarkers. Validation will be critical before their use becomes widespread.

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Abbreviations

ACR:

American College of Rheumatology

anti-CENP:

anti-centromere protein

CTD:

connective tissue disease

dcSSc:

diffuse cutaneous systemic sclerosis

lcSSc:

limited cutaneous systemic sclerosis

PM/DM:

polymyositis/dermatomyositis

SARD:

systemic autoimmune rheumatic diseases

SLE:

systemic lupus erythematosus

SSc:

systemic sclerosis

topo I:

topoisomerase I

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Authors and Affiliations

  1. Faculty of Medicine, University of Calgary, 3330 Hospital Dr NW, T2N 4N1, Calgary, AB, Canada

    Jennifer G. Walker, Sharon LeClercq, Steven M. Edworthy & Marvin J. Fritzler

  2. University of Western Ontario, London, ON, Canada

    Janet Pope

  3. McGill University, Montreal, Quebec, Canada

    Murray Baron, Marie Hudson & Suzanne Taillefer

  4. Department of Internal Medicine, Lviv Medical University, Lviv, Ukraine

    Oleg Nadashkevich

Authors
  1. Jennifer G. Walker
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  2. Janet Pope
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  3. Murray Baron
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  4. Sharon LeClercq
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  5. Marie Hudson
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  6. Suzanne Taillefer
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  7. Steven M. Edworthy
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  8. Oleg Nadashkevich
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  9. Marvin J. Fritzler
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Corresponding author

Correspondence to Marvin J. Fritzler.

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Walker, J.G., Pope, J., Baron, M. et al. The development of systemic sclerosis classification criteria. Clin Rheumatol 26, 1401–1409 (2007). https://doi.org/10.1007/s10067-007-0537-x

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  • DOI: https://doi.org/10.1007/s10067-007-0537-x

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