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Review: Defining a Unified Vascular Phenotype in Systemic Sclerosis
Yannick Allanore1, Oliver Distler2, Marco Matucci-Cerinic3
1Cochin Hospital, INSERM U1016, Paris Descartes University, Paris, France.
Arthritis & Rheumatology (Hoboken, N.J.)
|November 18, 2017
Summary
Systemic sclerosis (SSc) patients with digital ulcers experience pain and disability. While associated factors like male sex and severe skin disease are noted, predicting further vascular complications remains challenging.
Area of Science:
- Rheumatology
- Vascular Medicine
- Dermatology
Background:
- Microcirculation impairment and vasculopathy are key features of systemic sclerosis (SSc).
- Digital ulcers are a common and disabling vascular complication in SSc patients, second only to Raynaud's phenomenon.
- Recurrent digital ulcers raise questions about distinct patient outcomes and potential generalized vasculopathy.
Purpose of the Study:
- To review current understanding of digital ulcers in SSc.
- To identify associated factors and limitations in existing research.
- To explore the potential for a generalized vascular profile in SSc.
Main Methods:
- Analysis of data from SSc registries and cohorts.
- Review of longitudinal studies and autopsy findings.
- Examination of biomarker studies for cardiovascular associations.
Main Results:
- Male sex and severe skin disease are identified as associated factors for digital ulcers in SSc.
- Existing registries show limitations due to varying definitions of digital ulcers and organ involvement.
- Longitudinal data suggests worse outcomes for SSc patients with a history of digital ulcers, but prediction of other vascular complications is not established.
Conclusions:
- A history of digital ulcers in SSc may indicate worse outcomes but does not reliably predict additional vascular complications.
- Autopsy and biomarker studies support a concept of generalized vasculopathy in SSc.
- Future structured registries are expected to enable better identification of vascular profiles or phenotypes in SSc.
