Management of Endothelial Dysfunction in Systemic Sclerosis: Current and Developing Strategies.

Systemic Sclerosis (SSc) is an autoimmune disease marked by dysregulation of the immune system, tissue fibrosis and dysfunction of the vasculature. Vascular damage, remodeling and inadequate endothelial repair are hallmarks of the disease. Since early stages of SSc, damage and apoptosis of endothelial cells (ECs) can lead to perivascular inflammation, oxidative stress and tissue hypoxia, resulting in multiple clinical manifestations. Raynaud's phenomenon, edematous puffy hands, digital ulcers, pulmonary artery hypertension, erectile dysfunction, scleroderma renal crisis and heart involvement severely affect quality of life and survival. Understanding pathogenic aspects and biomarkers that reflect endothelial damage in SSc is essential to guide therapeutic interventions. Treatment approaches described for SSc-associated vasculopathy include pharmacological options to improve blood flow and tissue perfusion and, more recently, cellular therapy to enhance endothelial repair, promote angiogenesis and heal injuries. This mini-review examines the current knowledge on cellular and molecular aspects of SSc vasculopathy, as well as established and developing therapeutic approaches for improving the vascular compartment.

Chronic Liver Disease Questionnaire as a Tool to Evaluate the Quality of Life in Liver Transplant Candidates.

INTRODUCTION:: The advanced stage of liver disease causes impairments in the quality of life due to the physiological symptoms, besides the social and emotional stress. The aim of this study was to evaluate the quality of life of candidates for liver transplantation in specialized center in the interior of the state of São Paulo, Brazil. METHODS:: An observational study was carried out, with a quantitative approach. The sample was of convenience with the participation of 50 candidates for liver transplantation. Demographic characterization data, clinical data, and the Chronic Liver Disease Questionnaire were used to evaluate the quality of life. RESULTS:: The majority of the participants were male (68%), married (76%), with an average age of 55.7 years and average income of 2 to 3 minimum wages (42%). The main cause of liver disease was alcoholism (34%), mean time on the waiting list was 247.8 days, and mean model for end-stage liver disease was 20.5 points. The mean quality of life score was 2.9 points (standard deviation = 1.0) and the analysis of the 6 domains showed greater impairment of fatigue (2.2), activity (2.7), and concern (2.9) and better evaluation of systemic symptoms (3.4) and emotion (3.3). CONCLUSION:: Quality of life is impaired in most of the participants, indicating the need for greater attention and evaluation in the physical, mental, and social scope of this clientele.