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Review
. 2018 Jul-Aug;36 Suppl 113(4):175-182.
doi: 10.55563/clinexprheumatol/7ulgbj. Epub 2018 Sep 20.

Implications of endothelial shear stress on systemic sclerosis vasculopathy and treatment

Tracy M Frech  1 , Daniel R Machin  2 , Maureen A Murtaugh  2 , Gregory J Stoddard  2 , Samuel I Bloom  3 , Jessica V Phibbs  4 , Anthony J Donato  5
Affiliations
Review

Implications of endothelial shear stress on systemic sclerosis vasculopathy and treatment

Tracy M Frech et al. Clin Exp Rheumatol. 2018 Jul-Aug.

Abstract

There are no Federal Drug Administration approved drugs for the treatment of systemic sclerosis vascular digital ulcers (DU) in the United States, which are thought to be an end-stage result of prolonged ischaemia due to severe, prolonged Raynaud's phenomenon. Most therapeutics for vasodilation used in SSc work different pathways to target the smooth muscle to induce vessel relaxation. Longitudinal studies of vascular function allow insight into the effects of medications used for Raynaud's phenomenon in the SSc patient population. In this review, we discuss vascular tone, the function of the endothelium in SSc, and provide the rationale for longitudinal studies of vascular function and therapeutics that target the endothelial shear stress in addition to vasodilation for treatment and prevention of DU. This review provides the rationale for vasodilatory medication use for treatment of SSc-related DU and justifies access to non-FDA approved medications for this indication.

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Conflict of interest statement

Competing interests: none declared.

Figures

Fig. 1.
Fig. 1.
Therapeutics targeting vasodilatation in systemic sclerosis.
See this image and copyright information in PMC

References

    1. RUBIO-RIVAS M, ROYO C, SIMEON CP, CORBELLA X, FONOLLOSA V: Mortality and survival in systemic sclerosis: systematic review and meta-analysis. Semin Arthritis Rheum 2014; 44: 208–19. - PubMed
    1. KOWAL-BIELECKA O, LANDEWÉ R, AVOUAC J et al.: EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis 2009; 68: 620–8. - PubMed
    1. MULLER-LADNER U, DISTLER O, IBBAMANNESCHI L, NEUMANN E, GAY S: Mechanisms of vascular damage in systemic sclerosis. Autoimmunity 2009; 42: 587–95. - PubMed
    1. BARON M, POPE J, ROBINSON D et al.: Calcinosis is associated with digital ischaemia in systemic sclerosis-a longitudinal study. Rheumatology (Oxford). 2016; 55: 2148–55. - PMC - PubMed
    1. SULIMAN YABC, JOHNSON SR, PRAINO E et al.: Defining skin ulcers in systemic sclerosis: systematic literature review and proposed World Scleroderma Foundation (WSF) definition. J Scleroderma Relat Disord 2017; 2: 115–20. - PMC - PubMed

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