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 Jörg 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 André 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 – Noëlle Starr Sherber, MD, FAAD

Chapter 43 – Skeletal Muscle Involvement – Andrew L. Mammen, MD, PhD

Chapter 44 - Tendons, Joints and Bone – Jérôme 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