Determinants of mortality in systemic sclerosis: a focused review

  • Dilli Ram Poudel
  • Divya Jayakumar
  • Abhijeet Danve
  • Shiv Tej Sehra
  • Chris T. Derk
Observational Research


Scleroderma (systemic sclerosis) is an autoimmune rheumatic disorder that is characterized by fibrosis, vascular dysfunction, and autoantibody production that involves most visceral organs. It is characterized by a high morbidity and mortality rate, mainly due to disease-related complications. Epidemiological data describing mortality and survival in this population have been based on both population and observational studies. Multiple clinical and non-clinical factors have been found to predict higher likelihood of death among thepatients. Here, we do an extensive review of the available literature, utilizing the PubMed database, to describe scleroderma and non-scleroderma related determinants of mortality in this population. We found that even though the mortality among the general population has declined, scleroderma continues to carry a very high morbidity and mortality rate, however we have made some slow progress in improving the mortality among scleroderma patients over the last few decades.


Mortality Predictor Systemic sclerosis Scleroderma Review Survival 


Compliance with ethical standards


No financial support received.

Conflict of interest

None of the authors have conflict of interest.


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Copyright information

© Springer-Verlag GmbH Germany 2017

Authors and Affiliations

  1. 1.Department of Internal MedicineReading Health SystemWest ReadingUSA
  2. 2.Department of Internal MedicineWestchester Medical CenterValhallaUSA
  3. 3.Department of MedicineYale University School of MedicineNew HavenUSA
  4. 4.Division of RheumatologyMt. Auburn HospitalCambridgeUSA
  5. 5.Division of RheumatologyUniversity of Pennsylvania School of MedicinePhiladelphiaUSA

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