Open Access Medical Books



Edited by Timothy Radstake .

218 pages .
Open Access .
ISBN 978-953-307-869-4 .

Scleroderma, or systemic sclerosis (Ssc), means “tight skin” and is caused by the fibrosis of skin and disappearance of subcutaneous fat. There is much more involved, however. Although the fibrosis of the skin is a disabling and often painful sign of SSc, the involvement of the kidneys (see chapter on renal failure), lungs, and heart mostly lead to severe co-morbidity and premature death. The exact cause of SSc is unknown but there is accumulating evidence that the immune systems drives the three hallmarks that are observed in SSc patients: endothelial cell dysfunction, presence of autoantibodies, and fibroblast activation. In order to update the readers in the multiple facets of Ssc, this book covers two issues. In the first eight chapters, multiple key authors will provide better insights into the role of endothelial damage (chapter 1), platelets (chapter 2), and the observations that came forth from proteomic studies so far (chapter 3). T lymphocytes (chapter 4) and IL-17; a cytokine more recently shown to be increased in the circulation, and T cells isolated from SSc patients (chapter 5) are then discussed. In chapter 6, Broen and colleagues dive deeper into the potential role of the telomere and its dysfunction in SSc. Chapter 7 and 8 will deal with fibroblasts and thrombin as a potential therapeutic target of this clinical syndrome.
Besides the immunopathology of this disease, various clinical issues will be dealt with in the next issues. As such, novel issues with regard to diagnosis and pathogenesis will be discussed (chapter 9), as well as the potential role of capillary nail fold lesions in the diagnosis and follow-up (chapter 10). Scleroderma renal crisis is a feared complication of SSc and will be further discussed in chapter 11. Finally, the hurdles and pitfalls with regard pregnancy in a patient with SSc is widely discussed.
Altogether, although significant advances have occurred in our understanding SSc, the exact cause and most optimal way of treatment remains a medical conundrum.
This book is meant to provide an up-to-date in SSc pathogenesis and clinical features. We believe that this book will be able to provide both researchers and clinicians dealing with SSc with the most modern information that will aid them in their work. However, the research into its cause and most efficient treatment is rapidly expanding, hopefully resulting in highly needed updates in the not to distant future.

Dr Timothy Radstake, MD, PhD
Department of Rheumatology, Radboud University Medical Center, Nijmegen,
The Netherlands
The Scleroderma Center, Boston University School of Medicine, Massachusetts,


Part 1 of the Textbook : The Immune System in Systemic Sclerosis .

 1 Pathogenesis of the Endothelial Damage and Related Factors 3 Paola Cipriani, Vasiliki Liakouli, Alessandra Marrelli, Roberto Perricone and Roberto Giacomelli

 2 Blood Platelets and Systemic Sclerosis 29 Sébastien Lepreux, Anne Solanilla, Julien Villeneuve, Joël Constans, Alexis Desmoulière and Jean Ripoche

 3 Using Proteomic Analysis for Studying the Skin Fibroblast Protein Profile in Systemic Sclerosis 53 P. Coral-Alvarado, G. Quintana, C. Cardozo, J. Iriarte, Y. Sanchez, S. Bravo, J. Castano, M.F. Garces, L. Cepeda, A. Iglesias-Gamarra and J.E. Caminos

 4 Apoptosis of T Lymphocytes in Systemic Sclerosis 69 M. Szymanek, G. Chodorowska, A. Pietrzak and D. Krasowska

 5 Cytokines in Systemic Sclerosis: Focus on IL-17 93 Julie Baraut, Dominique Farge, Elena Ivan-Grigore, Franck Verrecchia and Laurence Michel

 6 Biological Ageing Research in Systemic Sclerosis: Time to Grow up? 103 J.C.A. Broen, L. McGlynn, T.R.D.J. Radstake and P.G. Shiels

 7 Fibrocytes in Scleroderma Lung Fibrosis 117 Ronald Reilkoff, Aditi Mathur and Erica Herzog

 8 Inhibition of Thrombin as a Novel Strategy in the Treatment of Scleroderma-Associated Interstitial Lung Disease 131 Galina S. Bogatkevich, Kristin B. Highland, Tanjina Akter, Paul J. Nietert, Ilia Atanelishvili, Joanne van Ryn and Richard M. Silver

Part 2 of the Textbook : Clinical Features of Systemic Sclerosis .

 9 Emerging Issues in the Immunopathogenesis, Diagnosis and Clinical Management of Primary Biliary Cirrhosis Associated with Systemic Sclerosis 151 Dimitrios P. Bogdanos, Cristina Rigamonti, Daniel Smyk, Maria G. Mytilinaiou, Eirini I. Rigopoulou and Andrew K. Burroughs

 10 Capillary Dimension Measured by Computer Based Digitalized Image in Patients with Systemic Sclerosis 167 Hyun-Sook Kim

 11 Scleroderma Renal Crisis 177 Lola Chabtini, Marwan Mounayar, Jamil Azzi, Vanesa Bijol, Sheldon Bastacky, Helmut G Rennke and Ibrahim Batal

 12 Pregnancy and Scleroderma 197 Charlotte Gorgiard, Alice Bérezné and Luc Mouthon .

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Published by: Unknown - Friday, June 14, 2013


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