Introduction to section VIII: Management and outcome assessment

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

Research output: Chapter in Book/Report/Conference proceedingChapter


Scleroderma has complex pathogenesis, and its protean clinical and pathological manifestations reflect a disease process affecting multiple organs. In addition, scleroderma encompasses a series of related but distinct entities that are characterized by different course, severity, response to therapy, and outcomes. Scleroderma causes pain, disfigurement, functional impairment, and a substantial decrease in life expectancy. The survival of patients with scleroderma has shown improvement during the past two decades. This improvement in survival, as well as in disease-associated morbidity and disability, is due to advances in diagnosis and early recognition of internal organ involvement, as well as successful management of selected complications such as pulmonary arterial hypertension, Raynaud phenomenon, gastroesophageal reflux disease, ischemic digital ulcers, and scleroderma renal crisis. Additionally, recognition of prognostic risk factors, such as scleroderma-associated autoantibodies, has permitted better risk stratification, which is indispensible for both more effective clinical trials, as well for optimized patient management. Risk stratification in scleroderma enables the selection of more precise therapies targeted to the individual scleroderma patient while reducing potential drug toxicities.

Original languageEnglish (US)
Title of host publicationScleroderma
Subtitle of host publicationFrom Pathogenesis to Comprehensive Management
PublisherSpringer International Publishing
Number of pages2
ISBN (Electronic)9783319314075
ISBN (Print)9783319314051
StatePublished - Jan 1 2016


  • Classification
  • Clinical trials
  • Disease-modifying therapy
  • Integrated management
  • Multidisciplinary care
  • Risk stratification
  • Screening

ASJC Scopus subject areas

  • Medicine(all)


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