Section I. Introduction
Historical Perspective of Scleroderma
Section II. Epidemiology, Genetics and Classification
Epidemiology, Environmental and Infectious Risk Factors
Genetic Factors
Disease Subsets in Clinical Practice
Evolving Concepts of Diagnosis and Classification
Overlap Syndromes
Juvenile Localized and Systemic Scleroderma
Morphea (Localized Scleroderma)
Scleroderma Mimics
Systems Biology Approaches to Understanding the Pathogenesis of Systemic Sclerosis
Section III: The Biological Basis of Systemic Sclerosis
Introduction: The Etiopathogenesis of Systemic Sclerosis: An Integrated Overview
Pathology of Systemic Sclerosis
Inflammation and Immunity
Autoantibodies as Markers and Possible Mediators of Scleroderma Pathogenesis
The Clinical Aspects of Autoantibodies
Mechanisms of Vascular Disease
Biomarkers in Systemic Sclerosis
Pathophysiology of Fibrosis in Systemic Sclerosis
Overview of Animal Models
Section IV. Cardiovascular Manifestations and Management
Raynaud's Phenomenon, Digital Ulcers, and Nailfold Capillaroscopy
Renal Crisis and Other Renal Manifestations of Scleroderma
Cardiac Involvement
Section V. Pulmonary Manifestations and Management
Overview of Lung Involvement: Diagnosis, Differential Diagnosis, and Monitoring
Clinical Assessment of Lung Disease
Treatment of Interstitial Lung Disease
Pathogenesis of Pulmonary Arterial Hypertension
Clinical Assessment of Pulmonary Hypertension
The Management of Pulmonary Arterial Hypertension in the Setting of Systemic Sclerosis
Section VI. Gastrointestinal Manifestations and Management
Overview of Gastrointestinal Tract Involvement
Upper Gastrointestinal Tract: Manifestations of Systemic Sclerosis
Small and Large Intestinal Involvement and Nutritional Issues
Section VII. Skin, Musculoskeletal and Other Complications
Calcinosis
Evaluation and Management of Skin Disease
Assessment and Management of Progressive Skin Involvement in Diffuse Scleroderma
Skeletal Muscle Involvement
Tendons, Joints, and Bone
Cancer in Systemic Sclerosis
Overlooked Manifestations
Pregnancy
Section VIII: Management and Outcome Assessment
Introduction to Section VIII: Management and Outcome Assessment
Immunomodulatory, Immunoablative, and Biologic Therapies
Investigative Approaches to Drug Therapy
Drug Development and Regulatory Considerations for SSc Therapies
Physical and Occupational Therapy
Psychosocial Issues and Care for Patients with Systemic Sclerosis
Clinical Trial Design in Systemic Sclerosis
Measuring Disease Activity and Outcomes in Clinical Trials
Innovative Approaches to Clinical Trials in Systemic Sclerosis
Molecular Stratification by Gene Expression as a Paradigm for Precision Medicine in Systemic Sclerosis
Section IX. Case Studies: Approach to Complex Clinical Problems
Managing the Ischemic Finger in Scleroderma
Scleroderma Renal Crisis
Rapid Diffuse Skin Disease with Progressive Joint Contractures
Management of the Scleroderma Patient with Pulmonary Arterial Hypertension Failing Initial Therapy
Pseudo-obstruction with Malabsorption and Malnutrition
Progressive Interstitial Lung Disease Non-Response to Cyclophosphamide
Coping with the Disfigurement of Scleroderma: Facial, Skin, and Hand Changes
Managing Complicated Digital Ulcers .