Comorbidity burden in systemic sclerosis: beyond disease-specific complications

  • Eleni Pagkopoulou
  • Alexandra Arvanitaki
  • Dimitrios Daoussis
  • Alexandros Garyfallos
  • George Kitas
  • Theodoros DimitroulasEmail author


Systemic sclerosis (SSc) is a chronic, systemic disease characterized by fibrosis of the skin and internal organs, vasculopathy, and auto-immune activation. On the top of severe organ involvement such as interstitial lung and myocardial fibrosis, pulmonary hypertension, and renal crisis, individuals diagnosed with SSc may suffer from a number of comorbidities. This is a narrative review according to published recommendations and we searched the online databases MEDLINE and EMBASE using as key words the following terms: systemic sclerosis, scleroderma, myocardial fibrosis in combination with micro- and macro-vascular disease, cardiac involvement, atherosclerosis, cardiovascular disease and coronary arteries, infections, cancer, depression, osteoporosis, and dyslipidemia. Although data are usually inconclusive it appears that comorbidities with significant impact on life expectancy, namely cardiovascular disease, infections, and cancer as well as phycological disorders affecting emotional and mental health are highly prevalent in SSc population. Thereafter, the aim of this review is to summarize the occurrence and the clinical significance of such comorbidities in SSc population and to discuss how rheumatologists can incorporate the management of these conditions in daily clinical practice.


Systemic Sclerosis Scleroderma Cancer Infections Depression Comorbidities 


Authors contribution

Conceived research idea and designed study: AG, GK, and THD; acquired data: EP, AA, and DD; analyzed and interpreted data: EP, AA, DD, and THD; provided supervision or mentorship: AG, GK, and THD. Each author contributed important intellectual content during manuscript drafting or revision and accepts accountability for the overall work by ensuring that questions pertaining to the accuracy or integrity of any portion of the work are appropriately investigated and resolved.

Compliance with ethical standards

Conflict of interest

All authors disclose that they do not have any financial or other relationships, which might lead to a conflict of interest regarding this paper.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Fourth Department of Internal Medicine, Hippokration University Hospital, Medical SchoolAristotle University of ThessalonikiThessalonikiGreece
  2. 2.Cardiology Department, AHEPA University HospitalAristotle University of ThessalonikiThessalonikiGreece
  3. 3.Department of Rheumatology, Faculty of Medicine, Patras University HospitalUniversity of Patras Medical SchoolPatrasGreece
  4. 4.Department of Rheumatology, Dudley Group NHS Foundation TrustRussells Hall HospitalDudleyUK
  5. 5.Arthritis Research UK, Centre for EpidemiologyUniversity of ManchesterManchesterUK

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