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Foreword Stephen Katz MD, PhD, Director, NIAMSForeword A personal view: Luke Evnin, PhD, President, SRFPreface John Varga, MD, Christopher P. Denton, PhD, FRCP, Fredrick M. Wigley, MD Section I. Introduction Chapter 1 - Historical Perspective - Christopher P. Denton, PhD, FRCP, and Carol BlackChapter 2 - Therapeutic evolution: a professor's view - Frank A. Wollheim, MD, PhD, FRCP Section II: Epidemiology, Genetics and ClassificationChapter 3 - Overview: Epidemiology, Genetics and Classification - Christopher P. Denton, PhD, FRCPChapter 4 - Epidemiology and Environmental Risk Factors - Jammie K. Barnes, MD,and Maureen D. Mayes, MD, MPHChapter 5 - Genetic Factors - Yannick Allanore, MD, PhD, and Filemon K. Tan, MD, PhDChapter 6 - Disease Subsets in Clinical Practice - Robyn Therese Domsic, MD, MPH,and Thomas A. Medsger, Jr., MDChapter 7 - Evolving Concepts of Diagnosis and Classification - Lorinda Chung, MD, MS, Jaap Fransen, MD, and Frank H.J. Van den Hoogen, MD, PhDChapter 8 - Disease Classification using Molecular Signatures - Michael L. Whitfield, PhD, and Robert Lafyatis, MDSection III: Scleroderma in ChildrenChapter 9 - Juvenile Localized Scleroderma - Francesco Zulian, MDChapter 10 - Juvenile Systemic Scleroderma - Ivan Foeldvari, MDSection IV: Other Fibrosing Skin ConditionsChapter 11 - Scleroderma Mimics -Laura K. Hummers, MD, ScMChapter 12 - Localized Forms of Scleroderma in Adults - Amy E. Gilliam, MD, and Anita C. Gilliam, MD, PhDChapter 13 - Nephrogenic Systemic Fibrosis - Peter J. Wermuth, PhD, and Sergio A. Jimenez, MDSection V: Pathogenetic Basis Chapter 14 - Overview: Integrated Pathogenesis - John Varga, MDChapter 15 - Immunological Mechanisms - Francisco Boin, MD, and Carlo Chizzolini, MDChapter 16 - Innate Immunity - Keith B. Elkon, MD, and Julia J. Rhiannon, MD, MSWChapter 17 - Autoantibodies in Pathogenesis - Kimberly Doering, BS, and Antony Rosen, MB ChB, BSc (Hons)Chapter 18 - The Clinical Aspects of Autoantibodies - John D. Pauling, BMBS, MRCP, and Neil McHugh, MB, ChB, MD, FRCP, FRCPathChapter 19 - Mechanisms of Vascular Disease - Bashar Kalaheh, MD, and Mary Jo Mulligan-Kehoe, PhDChapter 20 - Vascular Biomarkers - Laura K. Hummers, MD, ScM Chapter 21 - Mechanisms of Fibrosis - John Varga, MDChapter 22 - Fibrosis: Insights from the stiff skin syndrome - Elizabeth E. Gerber, BS, and Harry C. Dietz, MDChapter 23 - Biomarkers of Fibrosis - Christian Beyer, MD, Oliver Distler, MD, and Joerg H.W. Distler, MDChapter 24 - Overview of Animal Models - Christopher P. Denton, PhD, FRCP, and Robert Lafyatis, MDSection

VI: Cardiovascular Manifestations and ManagementChapter 25 - Overview: Cardiovascular Manifestations and Management - Fredrick M. Wigley, MDChapter 26 - Raynaud Phenomenon - Arianne Herrick and Fredrick M. Wigley, MDChapter 27 - Nailfold Capillaroscopy - Maurizio Cutolo, MD and Vanessa Smith, MD, PhD 28SystemicVascular Disease , MD, PhD, MD, FRCP Chapter 29 - Scleroderma Renal Crisis - Christopher P. Denton, PhD, FRCP, and Virginia Steen, MDChapter 30 - Cardiac Involvement -Sanjiv J. Shah, MD, and Andre Kahan, MD, PhDChapter 31 - Erectile Dysfunction - Edward V. Lally, MD, Arthur L. Burnett, MD, MBA, FACS, and Trinity J. Bivalacqua, MD, PhDSection VII: Pulmonary manifestations and management Chapter 32 - Overview: Pulmonary manifestations and management - Christopher P. Denton, PhD, FRCPChapter 33 - Clinical Assessment of Lung Disease - Greg J. Keir, MBBS, FRACP, Richard M. Silver, MD, and Athol U. Wells, MD, FRCPChapter 34 - Computerized Tomography of Interstitial Lung Disease - Athol U. Wells, MD, FRCP, and Jonathan Goldin MD, PhD, FRCRChapter 35 - Treatment of interstitial lung disease - Dinesh Khanna, MD, MS, and Donald P. Tashkin, MDChapter 36 - Clinical Assessment of Pulmonary Hypertension - Harrison W. Farber, MD, Michael York, MD, Eric Hachulla, MD, PhDChapter 37 - Treatment of Pulmonary Hypertension -Todd H. Kolb, MD, PhD, and Paul M. Hassoun, MDChapter 38 - Complex Challenges of Pulmonary Hypertension - John GerardCoghlan, MD, FRCPSection VIII: Gastrointestinal manifestations and managementChapter 39- Overview: Gastrointestinal Manifestations and Management - Seth Sweetser, MD, and Michael Camilleri, MDChapter 40 - Upper Gastrointestinal Tract - John O. Clarke, MD, and Ikuo Hirano, MDChapter 41 - Small and Large Intestinal Involvement - Philip J. Clements, MD, MPH, Terri R. Getzug, MD, and Dinesh Khanna, MD, MSSection XIX: Skin, musculoskeletal and other complications Chapter 42 - Evaluation and Management of Skin Disease - Noelle Starr Sherber, MD, FAADChapter 43 - Skeletal Muscle Involvement - Andrew L. Mammen, MD, PhDChapter 44 - Tendons, Joints and Bone - Jerome Avouac, MD, PhD, and Yannick Allanore, MD, PhDChapter 45 - Pregnancy - Virginia Steen, MD, and Eliza F. Chakravarty, MD, MSChapter 46 - Overlooked Manifestations- Ami A. Shah, MD, MHSSection X: Disease-modifying strategiesChapter 47 -Principles of ManagementD. , MD, MPH Chapter 48 - Immunomodulatory Therapies - Jacob M. van Laar, MD, PhDChapter 49 - Cell-Based Therapies - Alan G. Tyndall, MD, FRACP, Rheumatology FMH, and Keith M. Sullivan, MDChapter 50 - Investigative Approaches to Drug Therapy - Voon H. Ong, PhD, MRCP, and Christopher P. Denton, PhD, FRCPChapter 51 - Complementary and alternate medicine approaches - Judy A. Fulop, MD, MS, FABNO, and John Varga, MD Chapter 52 - Physical and occupational therapy- Luc Mouthon,MD, PhD, and Janet L. Poole, PhD, OTR/L Chapter 53 - Evaluation and Management of Psychosocial Issues - El-Baalbaki Ghassan, PhD, Lisa Jewett, BA, MSc, Jennifer A. Haythornthwaite, PhD, and Brett D. Thombs, PhDSection XI: Outcome assessmentChapter 54 - Issues in Clinical Trial Design- Suzanne Kafaja, MD, Daniel E. Furst, MD, and Janet E. Pope, MD, MPH, FRCPCChapter 55 - Measuring Disease Activity- Dinesh Khanna, MD, MSChapter 56 - Patient Reported Outcomes - Monique Hinchcliff, MD, and David Cella, PhD


From the reviews:"This is a very comprehensive summary of the various aspects of scleroderma, one of the most complex and challenging autoimmune disorders to diagnose and treat. ... a valuable resource not only for practicing rheumatologists, but also for any physicians who treat scleroderma patients, including gastroenterologists, nephrologists, primary care doctors, and residents. ... good number of illustrations and graphs make the subject more interesting and easier to comprehend. ... The authors have done an incredible job summarizing all the recent research and advances in this area." (Saba Beg, Doody's Book Reviews, January, 2014)"Scleroderma: From Pathogenesis to Comprehensive Management emphasises the multidisciplinary approach to comprehensive care of the scleroderma patient. The book serves as a valuable management guide and scientific review for both the 'sclerodermaologist'/clinician and the budding researcher seeking in-depth and authoritative discussions on all aspects of scleroderma. The book is recommended to any clinician who participates in the care of our patients with this complex, yet fascinating syndrome." (R. Saggar, European Respiratory Review, Vol. 21 (126), December, 2012)

"This book integrates the latest developments in scleroderma in terms of understanding its pathophysiology and management. The purpose is to provide an ultimate source that integrates historical management, current pathophysiology, and the possible future of better therapy. ... The book is written for rheumatologists." (Hyon Ju Park, Doody's Review Service, March, 2012)

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