Scleroderma

• Foreword Stephen Katz MD, PhD, Director, NIAMS Foreword A personal view: Luke Evnin, PhD, President, SRF Preface 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 Black Chapter 2 - Therapeutic evolution: a professor's view - Frank A. Wollheim, MD, PhD, FRCP Section II: Epidemiology, Genetics and Classification Chapter 3 - Overview: Epidemiology, Genetics and Classification - Christopher P. Denton, PhD, FRCP Chapter 4 - Epidemiology and Environmental Risk Factors - Jammie K. Barnes, MD, and Maureen D. Mayes, MD, MPH Chapter 5 - Genetic Factors - Yannick Allanore, MD, PhD, and Filemon K. Tan, MD, PhD Chapter 6 - Disease Subsets in Clinical Practice - Robyn Therese Domsic, MD, MPH, and Thomas A. Medsger, Jr., MD Chapter 7 - Evolving Concepts of Diagnosis and Classification - Lorinda Chung, MD, MS, Jaap Fransen, MD, and Frank H.J. Van den Hoogen, MD, PhD Chapter 8 - Disease Classification using Molecular Signatures - Michael L. Whitfield, PhD, and Robert Lafyatis, MD Section III: Scleroderma in Children Chapter 9 - Juvenile Localized Scleroderma - Francesco Zulian, MD Chapter 10 - Juvenile Systemic Scleroderma - Ivan Foeldvari, MD Section IV: Other Fibrosing Skin Conditions Chapter 11 - Scleroderma Mimics -Laura K. Hummers, MD, ScM Chapter 12 - Localized Forms of Scleroderma in Adults - Amy E. Gilliam, MD, and Anita C. Gilliam, MD, PhD Chapter 13 - Nephrogenic Systemic Fibrosis - Peter J. Wermuth, PhD, and Sergio A. Jimenez, MD Section V: Pathogenetic Basis Chapter 14 - Overview: Integrated Pathogenesis - John Varga, MD Chapter 15 - Immunological Mechanisms - Francisco Boin, MD, and Carlo Chizzolini, MD Chapter 16 - Innate Immunity - Keith B. Elkon, MD, and Julia J. Rhiannon, MD, MSW Chapter 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, FRCPath Chapter 19 - Mechanisms of Vascular Disease - Bashar Kalaheh, MD, and Mary Jo Mulligan-Kehoe, PhD Chapter 20 - Vascular Biomarkers - Laura K. Hummers, MD, ScM Chapter 21 - Mechanisms of Fibrosis - John Varga, MD Chapter 22 - Fibrosis: Insights from the stiff skin syndrome - Elizabeth E. Gerber, BS, and Harry C. Dietz, MD Chapter 23 - Biomarkers of Fibrosis - Christian Beyer, MD, Oliver Distler, MD, and Jorg H.W. Distler, MD Chapter 24 - Overview of Animal Models - Christopher P. Denton, PhD, FRCP, and Robert Lafyatis, MD Section VI: Cardiovascular Manifestations and Management Chapter 25 - Overview: Cardiovascular Manifestations and Management - Fredrick M. Wigley, MD Chapter 26 - Raynaud Phenomenon - Arianne Herrick and Fredrick M. Wigley, MD Chapter 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, MD Chapter 30 - Cardiac Involvement -Sanjiv J. Shah, MD, and Andre Kahan, MD, PhD Chapter 31 - Erectile Dysfunction - Edward V. Lally, MD, Arthur L. Burnett, MD, MBA, FACS, and Trinity J. Bivalacqua, MD, PhD Section VII: Pulmonary manifestations and management Chapter 32 - Overview: Pulmonary manifestations and management - Christopher P. Denton, PhD, FRCP Chapter 33 - Clinical Assessment of Lung Disease - Greg J. Keir, MBBS, FRACP, Richard M. Silver, MD, and Athol U. Wells, MD, FRCP Chapter 34 - Computerized Tomography of Interstitial Lung Disease - Athol U. Wells, MD, FRCP, and Jonathan Goldin MD, PhD, FRCR Chapter 35 - Treatment of interstitial lung disease - Dinesh Khanna, MD, MS, and Donald P. Tashkin, MD Chapter 36 - Clinical Assessment of Pulmonary Hypertension - Harrison W. Farber, MD, Michael York, MD, Eric Hachulla, MD, PhD Chapter 37 - Treatment of Pulmonary Hypertension -Todd H. Kolb, MD, PhD, and Paul M. Hassoun, MD Chapter 38 - Complex Challenges of Pulmonary Hypertension - John Gerard Coghlan, MD, FRCP Section VIII: Gastrointestinal manifestations and management Chapter 39- Overview: Gastrointestinal Manifestations and Management - Seth Sweetser, MD, and Michael Camilleri, MD Chapter 40 - Upper Gastrointestinal Tract - John O. Clarke, MD, and Ikuo Hirano, MD Chapter 41 - Small and Large Intestinal Involvement - Philip J. Clements, MD, MPH, Terri R. Getzug, MD, and Dinesh Khanna, MD, MS Section XIX: Skin, musculoskeletal and other complications Chapter 42 - Evaluation and Management of Skin Disease - Noelle Starr Sherber, MD, FAAD Chapter 43 - Skeletal Muscle Involvement - Andrew L. Mammen, MD, PhD Chapter 44 - Tendons, Joints and Bone - Jerome Avouac, MD, PhD, and Yannick Allanore, MD, PhD Chapter 45 - Pregnancy - Virginia Steen, MD, and Eliza F. Chakravarty, MD, MS Chapter 46 - Overlooked Manifestations- Ami A. Shah, MD, MHS Section X: Disease-modifying strategies Chapter 47 -Principles of ManagementD. , MD, MPH Chapter 48 - Immunomodulatory Therapies - Jacob M. van Laar, MD, PhD Chapter 49 - Cell-Based Therapies - Alan G. Tyndall, MD, FRACP, Rheumatology FMH, and Keith M. Sullivan, MD Chapter 50 - Investigative Approaches to Drug Therapy - Voon H. Ong, PhD, MRCP, and Christopher P. Denton, PhD, FRCP Chapter 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, PhD Section XI: Outcome assessment Chapter 54 - Issues in Clinical Trial Design- Suzanne Kafaja, MD, Daniel E. Furst, MD, and Janet E. Pope, MD, MPH, FRCPC Chapter 55 - Measuring Disease Activity- Dinesh Khanna, MD, MS Chapter 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)