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Determinants of mortality in systemic sclerosis: a focused review

  • Observational Research
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Abstract

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.

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Authors and Affiliations

  1. Department of Internal Medicine, Reading Health System, 420 S Fifth Avenue, West Reading, PA, 19611, USA

    Dilli Ram Poudel

  2. Department of Internal Medicine, Westchester Medical Center, 100 Woods Rd, Valhalla, NY, 10595, USA

    Divya Jayakumar

  3. Department of Medicine, Yale University School of Medicine, 333 Cedar St, New Haven, CT, 06510, USA

    Abhijeet Danve

  4. Division of Rheumatology, Mt. Auburn Hospital, 330 Mt Auburn St, Cambridge, MA, 02138, USA

    Shiv Tej Sehra

  5. Division of Rheumatology, University of Pennsylvania School of Medicine, 3400 Spruce Street, Philadelphia, PA, 19104, USA

    Chris T. Derk

Authors
  1. Dilli Ram Poudel
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  2. Divya Jayakumar
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  3. Abhijeet Danve
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  4. Shiv Tej Sehra
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  5. Chris T. Derk
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Correspondence to Dilli Ram Poudel.

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Poudel, D.R., Jayakumar, D., Danve, A. et al. Determinants of mortality in systemic sclerosis: a focused review. Rheumatol Int 38, 1847–1858 (2018). https://doi.org/10.1007/s00296-017-3826-y

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  • DOI: https://doi.org/10.1007/s00296-017-3826-y

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