Scleroderma

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