Scleroderma: From Pathogenesis to Comprehensive Management by John Varga, Christopher P. Denton, Fredrick M. Wigley

By John Varga, Christopher P. Denton, Fredrick M. Wigley

Comprised of the authoritative paintings of foreign specialists, this fully-updated moment version of Scleroderma builds upon the well-regarded procedure within the first version to supply built-in, concise, and updated synthesis of present innovations of pathogenesis and glossy ways to administration of systemic sclerosis (scleroderma). With a multidisciplinary method of accomplished care, this ebook is well available for health and wellbeing care execs in lots of fields. the recent version contains wide up to date fabric in keeping with significant advancements within the box, with new chapters on custom-made medication, melanoma issues, international views on scleroderma, and extra. It provides a succinct and considerate synthesis of present pathomechanistic strategies, supplying a necessary reference software for uncomplicated and translational investigators operating within the box. Scleroderma: From Pathogenesis to accomplished administration serves as a vital, all-inclusive source for rheumatologists, pulmonologists, cardiologists, gastroenterologists, nephrologists and all these curious about the care of scleroderma patients.

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Extra resources for Scleroderma: From Pathogenesis to Comprehensive Management

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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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