Feasibility study of stem-cell enriched autologous lipotransfer to treat oro-facial fibrosis in systemic sclerosis (Sys-Stem): Protocol for open-label randomised controlled trial.

Introduction: Oro-facial fibrosis is a common and disabling manifestation of systemic sclerosis (SSc), causing a plethora of functional, aesthetic and social compromise, yet is without effective treatment. Autologous lipotransfer is an established minimally invasive surgical procedure that is postulated to exert anti-fibrotic effects by adipose-derived stem cells, and presents a novel method in the treatment of fibrotic conditions. This study aims to assess the safety and efficacy of autologous lipotransfer for facial involvement in SSc.

N-terminal pro Brain Natriuretic Peptide as predictor of outcome in scleroderma renal crisis.

Objectives: Although in scleroderma renal crisis (SRC) outcome has improved to a great extent with the introduction of ACE inhibitors, there remains significant mortality and morbidity with frequent requirement for renal replacement therapy. Therefore, novel biomarkers to identify patients at high risk of poor outcome would be valuable. The aim of this study was to assess the role of the N terminal fragment of pro Brain Natriuretic Peptide (N-TproBNP) as predictor of outcome in SRC.

Cytokines in the immunopathology of systemic sclerosis.

Cytokines and growth factors are key regulators of immune activation, vascular alteration and excessive production of extracellular matrix which are hallmark events in the pathogenesis of systemic sclerosis (SSc). They modulate cell-cell and cell-matrix interactions. In particular, cytokines play a central role in the immunopathogenesis of SSc on the basis of molecular pathways which are complex and not completely understood.

Management of digital vasculopathy in systemic sclerosis: benefits of multiple courses of endothelin-1 receptor antagonists.

Two patients with scleroderma and recurrent digital ulcers (DUs) were treated with bosentan for 6 months. Treatment was associated with a reduction in the number of new DUs; however, DUs recurred on treatment discontinuation. This case report illustrates the positive response of digital vasculopathy to bosentan and describes the benefits of multiple bosentan treatment courses in these patients.

Chemokine receptor CCR2 expression by systemic sclerosis fibroblasts: evidence for autocrine regulation of myofibroblast differentiation.

Objective: To investigate expression of the chemokine receptor CCR2 on key cell types involved in the pathogenesis of systemic sclerosis (SSc) and to assess the potential for autocrine activation of SSc dermal fibroblasts via CCL2/CCR2.

Methods: Chemokine receptor expression in skin biopsy tissues and explanted dermal fibroblasts from a well-characterized cohort of SSc patients was examined using immunohistochemistry and flow cytometry techniques. Autocrine regulation of the expression of fibrotic markers in CCR2+ SSc fibroblast cell lines was assessed using specific ligand or receptor antagonists.