Blood flow in the hands of a predefined homogeneous systemic sclerosis population: the presence of digital ulcers and the improvement with bosentan

Abstract

https://www.clinicaltrialsregister.eu/ (EudraCT number 2010-023908-27).

Treatment of digital ulcers in systemic sclerosis: Case series study of thirteen patients and discussion on outcome

“…9 Endothelin receptor antagonists are approved for the prevention of DU recurrence with improved hand functionality and better functional and vital prognosis based on the results of the RAPIDS-1 and RAPIDS-2 trials. 2,4,6,12,13 Other studies have shown that bosentan treatment increased the fingertip blood flow in patients with low perfusion in its initial stages, helping in the process of remodeling the capillary microcirculation. 2,5 Short-acting antiplatelet and anticoagulant therapy should be considered, although there is no strong evidence of its efficacy.…”

“…2,4,6,12,13 Other studies have shown that bosentan treatment increased the fingertip blood flow in patients with low perfusion in its initial stages, helping in the process of remodeling the capillary microcirculation. 2,5 Short-acting antiplatelet and anticoagulant therapy should be considered, although there is no strong evidence of its efficacy. 4 There is strong theoretical support for the use of antiplatelet agents in SSc and a consensus on the practical approach for prescription, being recommended in all patients with chronic digital ischemia.…”

“…They occur in 30% of patients with limited SSc and 58% in diffuse forms. [1][2][3] Its etiology is multifactorial; however, two triggers have been described as responsible for the development of DU: one is related to the structure of the vascular wall, which leads to a proliferation of the intima and an increased production of vasoconstrictors; the other is related to the existence of a variable degree of intraluminal thrombosis. 1,[4][5][6] Among the mechanisms considered to be responsible for the development of DU, endothelial injury results from a decrease in the caliber of the intravascular lumen caused by the secretion of collagen and other components of the extracellular matrix, which in turn leads to fibroblast activation, hindering blood flow and causing chronic ischemia of tissues.…”

“…Also, treatment with endothelin receptor antagonists (bosentan) may have some efficacy in preventing new DUs as well as in improving the functionality of the hand. 2 The objective of our review is to describe a population of patients with SSc and DU, their treatment, complications and clinical results, implementing the protocol of our Center.…”

Skin ulcers in systemic sclerosis – etiopathogenesis, prophylaxis and treatment

“…In 40% of cases they are observed within the first year, and in 70% within 5 years from the moment of SSc clinical presentation other than Raynaud's phenomenon [2]. They appear in 30% of patients with limited SSc (lSSc) and in almost 60% of patients suffering from diffuse SSc (dSSc) [3][4][5]. Digital ulcers appear more often in male patients and smokers, in whom Raynaud's phenomenon appeared shortly before SSc diagnosis, Scl-70 antibodies are confirmed, acute-phase proteins concentration is elevated, or who suffer from pulmonary hypertension [6].…”