The Washington Manual Pulmonary Medicine Subspecialty by A Shifren, Et Al

By A Shifren, Et Al

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Appreciation of the need for cohort enrichment and clear definition of endpoints has emerged from expert discussion over the past three decades and represents an important achievement in recent scleroderma history. Classification of SSc was first attempted in 1980 [2] and subset criteria were later developed. The new classification criteria were developed by rigorous methodology and with strong international collaboration and were published in 2013 [6]. These reflected expert practice and a growing understanding of disease heterogeneity, although attempts to define robust criteria for early diagnosis of classification of milder disease proved challenging and no real agreement was reached until much more recently when evidence-based approaches such as the prospective VEDOSS project developed [7].

Mayes, MD, MPH Division of Rheumatology and Clinical Immunogenetics, University of Texas Health Science Center – Houston, Houston, TX 77030, USA © Springer Science+Business Media New York 2017 J. Varga et al. 1007/978-3-319-31407-5_2 dard classification system for SSc prior to 1980 makes it problematic to interpret occurrence figures for SSc in reports prior to this time. Reported prevalence figures for definite SSc vary greatly from 30 cases/million (New Zealand, 1979) [5] to 580 cases/million (Alberta, Canada, 1994–2007) [6].

The qualifying term “progressive” was later considered to be inaccurate in some cases that either remained stable or improved or has generally been dropped. It does however serve to highlight the potential severity of the worst forms of the disease. The potential importance of subtypes of scleroderma began in 1964, when Winterbauer reported cases with the CRST (calcinosis, Raynaud’s phenomenon, sclerodactyly, and telangiectasias) syndrome. A similar group of patients was reported in 1920, named after the authors, the Thibierge-Weissenbach syndrome.

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