TY - CHAP A1 - Dimitrios P. Bogdanos A2 - Cristina Rigamonti A3 - Daniel Smyk A4 - Maria G. Mytilinaiou A5 - Eirini I. Rigopoulou A6 - Andrew K. Burroughs ED1 - Timothy Radstake Y1 - 2012-02-03 PY - 2012 T1 - Emerging Issues in the Immunopathogenesis, Diagnosis and Clinical Management of Primary Biliary Cirrhosis Associated with Systemic Sclerosis N2 - Systemic sclerosis (SSc), or often referred to as Scleroderma (tight skin), is characterized by an exaggerated formation of collagen fibers in the skin, which leads to fibrosis. Accumulating evidence now points toward three pathological hallmarks that are implicated in Ssc, the order of which has yet to be determined: endothelial dysfunction, autoantibody formation, and activation of fibroblasts. This current book provides up-to-date information on the pathogenesis and clinical features of this severe syndrome. It is our hope that this book will aid both clinicians and researchers in dealing with patients with this clinical syndrome. In addition, we hope to shed more light on this rare and severely disabling syndrome, ultimately leading to better research and successful therapeutic targeting. BT - Systemic Sclerosis SP - Ch. 9 UR - https://doi.org/10.5772/27203 DO - 10.5772/27203 SN - PB - IntechOpen CY - Rijeka Y2 - 2020-09-22 ER -