Header

UZH-Logo

Maintenance Infos

Defining a unified vascular phenotype in systemic sclerosis


Allanore, Yannick; Distler, Oliver; Matucci-Cerinic, Marco; Denton, Christopher P (2018). Defining a unified vascular phenotype in systemic sclerosis. Arthritis and Rheumatology, 70(2):162-170.

Abstract

Microcirculation impairment and related vasculopathy are hallmarks of systemic sclerosis (SSc). Digital ulceration is second only to Raynaud's phenomenon as a vascular complication occurring in patients with SSc. Digital ulcers are painful and generate disability. Furthermore, patients may develop recurrent digital ulcers, and it is reasonable to question whether the outcomes of such patients might be different from those of patients who are not affected. Recently, several registries have provided relevant information about digital ulcers. Male sex and severe skin disease appear to be the main associated factors observed in several registries. However, limitations of those studies are the differences in the definitions of digital ulcers and organ involvement. Few longitudinal studies are available, and the more robust data from the European League Against Rheumatism Scleroderma Trial and Research cohort suggested worse outcomes in patients with a history of digital ulcers but could not demonstrate that a history of digital ulcers can predict additional vascular complications such as pulmonary arterial hypertension, heart failure, or renal crisis. Nevertheless, the autopsy studies published many years ago and the more recent longitudinal biomarker studies support the concept of generalized vasculopathy and a potential association between various cardiovascular complications. It is expected that with the availability of several structured registries, identification of a vascular profile or vascular phenotype will be addressed using more robust data in the near future.

Abstract

Microcirculation impairment and related vasculopathy are hallmarks of systemic sclerosis (SSc). Digital ulceration is second only to Raynaud's phenomenon as a vascular complication occurring in patients with SSc. Digital ulcers are painful and generate disability. Furthermore, patients may develop recurrent digital ulcers, and it is reasonable to question whether the outcomes of such patients might be different from those of patients who are not affected. Recently, several registries have provided relevant information about digital ulcers. Male sex and severe skin disease appear to be the main associated factors observed in several registries. However, limitations of those studies are the differences in the definitions of digital ulcers and organ involvement. Few longitudinal studies are available, and the more robust data from the European League Against Rheumatism Scleroderma Trial and Research cohort suggested worse outcomes in patients with a history of digital ulcers but could not demonstrate that a history of digital ulcers can predict additional vascular complications such as pulmonary arterial hypertension, heart failure, or renal crisis. Nevertheless, the autopsy studies published many years ago and the more recent longitudinal biomarker studies support the concept of generalized vasculopathy and a potential association between various cardiovascular complications. It is expected that with the availability of several structured registries, identification of a vascular profile or vascular phenotype will be addressed using more robust data in the near future.

Statistics

Citations

Dimensions.ai Metrics
46 citations in Web of Science®
44 citations in Scopus®
Google Scholar™

Altmetrics

Downloads

0 downloads since deposited on 21 Nov 2017
0 downloads since 12 months

Additional indexing

Item Type:Journal Article, refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Rheumatology Clinic and Institute of Physical Medicine
Dewey Decimal Classification:610 Medicine & health
Scopus Subject Areas:Health Sciences > Immunology and Allergy
Health Sciences > Rheumatology
Life Sciences > Immunology
Language:English
Date:2018
Deposited On:21 Nov 2017 16:22
Last Modified:02 Nov 2022 17:00
Publisher:Wiley-Blackwell Publishing, Inc.
ISSN:2326-5191
OA Status:Closed
Free access at:Publisher DOI. An embargo period may apply.
Publisher DOI:https://doi.org/10.1002/art.40377
PubMed ID:29145709