Highlights from Congresses
We are delighted to present to you the highlights from the most updated research results in the scleroderma field presented at international congresses: a selection from oral and poster presentation sessions.
Selexipag treatment in patients with systemic sclerosis–associated pulmonary arterial hypertension in clinical practice, a case series
Abstract Objective: To describe the efficacy and safety in all patients with systemic sclerosis–associated pulmonary arterial hypertension who started selexipag between 09-2016 and 06-2018 in two pulmonary arterial hypertension expert centers. Methods: All patients with systemic sclerosis–associated pulmonary arterial hypertension diagnosed by right heart catheterization and treated with selexipag were included. Every 12 weeks, treatment effect [...]
Characteristics of patients with systemic sclerosis suffering from a lower limb amputation: Results of a French collaborative study
Abstract Objective: Systemic sclerosis mainly affects the microvascular network. However, macrovascular manifestations have been reported. We aimed to investigate the characteristics of systemic sclerosis patients with an amputation of a lower limb segment. Methods: We designed a retrospective, case–control, multicentric study on systemic sclerosis patients with amputation of a lower limb segment secondary to critical [...]
Improving adherence to pulmonary hypertension screening in patients with systemic sclerosis: Overcoming the provider-level barriers
Abstract Objective: Pulmonary arterial hypertension is a life-limiting complication in patients with systemic sclerosis. Current recommendations suggest a minimum screening of an annual transthoracic echocardiogram and pulmonary function test. We hypothesize that modifiable provider-level hurdles contribute to inadequate screening and can be alleviated. Methods and results: We performed a longitudinal study over 23 months of all [...]
Ulnar artery vasculopathy: a common but nonspecific feature of systemic sclerosis
Abstract IntroductionSystemic sclerosis (SSc) is characterized by microvascular abnormalities and fibrosis. Several studies have reported that large vessel disease is also common in SSc. The aim of this case series was to investigate whether ulnar artery involvement in patients with SSc is a disease-specific phenomenon, as compared to other connective tissue diseases (CTD). MethodsA total [...]
Use of intravenous epoprostenol as a treatment for the digital vasculopathy associated with the scleroderma spectrum of diseases
Abstract IntroductionIntravenous prostanoid therapy is recommended for severe systemic sclerosis-related digital vasculopathy. The evidence supporting this recommendation is limited. The aim of this study was to evaluate the safety and efficacy of treating scleroderma spectrum digital vasculopathy with intravenous epoprostenol. MethodsPatients with a diagnosis of systemic sclerosis who had received intravenous epoprostenol for scleroderma spectrum [...]
A randomized, single-blinded cross-over trial of ischemic preconditioning in Raynaud’s phenomenon
A randomized, single-blinded cross-over trial of ischemic preconditioning in Raynaud’s phenomenon Abstract IntroductionIschemic preconditioning (IPC) is protective against future ischemia, with brief cycles of ischemia and reperfusion leading to the release of circulating endogenous compounds from ischemic cells. IPC may increase vasodilatory substances and improve Raynaud’s phenomenon (RP). We hypothesized that IPC would be more [...]
Certainties and uncertainties concerning the contribution of pericytes to the pathogenesis of systemic sclerosis
Certainties and uncertainties concerning the contribution of pericytes to the pathogenesis of systemic sclerosis Abstract The role of pericytes in systemic sclerosis (SSc) is unclear because of the difficulty in phenotyping them. They are mainly distributed in the pre-capillary, capillary and post-capillary abluminal side of non-muscular micro-vessels, express platelet-derived growth factor receptors (PDGFRs), and preside [...]
Paul Klee’s illness – a series of historical and clinical vignettes
Paul Klee’s illness – a series of historical and clinical vignettes Post author correction Article Type: BRIEF REPORT Article Subject: Psychology and Social Phenomena DOI:10.5301/jsrd.5000248 Authors Richard M. Silver Abstract Paul Klee (1879-1940) is undoubtedly the most notable historical figure to have suffered from systemic sclerosis (scleroderma). In this, the first in a series of [...]
Differential expression of hepatocyte growth factor in patients with systemic sclerosis-associated pulmonary arterial hypertension
Differential expression of hepatocyte growth factor in patients with systemic sclerosis-associated pulmonary arterial hypertension Post author correction Article Type: BRIEF REPORT Article Subject: Epidemiology and Diagnostic Methods DOI:10.5301/jsrd.5000245 Authors Yon K. Sung, Roham T. Zamanian, Catriona A. Wagner, William Robinson, Virginia Steen, Lorinda Chung Abstract IntroductionNon-invasive biomarkers are needed to identify pulmonary arterial hypertension (PAH) [...]
