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

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

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

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,

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