Practical Management of Systemic Sclerosis in Clinical Practice

Contents
Chapter 1: Major Scleroderma Emergencies
	Introduction
	Case History 1
	Case History 2
	Summary
	References
Chapter 2: Scleroderma Renal Crisis
	Introduction
		Case #1 Classic Case of SRC
			Evaluation
			Management (Fig. 2.2)
		Case #2 Isolated Systemic Hypertension
	References
Chapter 3: Anemia and Thrombocytopenia
	Outline
		Nutritional Deficit Anemia
		Autoimmune Causes of Isolated Anemia and Thrombocytopenia
		Microangiopathic Anemia and Thrombocytopenia
		Drug-Induced Anemia and Thrombocytopenia
		Bone Marrow Insufficiency
		Pregnancy/Post-Partum/Infancy
	References
Chapter 4: Breathlessness
	References
Chapter 5: Itch
	Case Vignette
	Background
	Differential Diagnosis
	Therapy
	References
Chapter 6: Mood Problems and Depression in Systemic Sclerosis
	Introduction
	Contributory Factors Related to Mood Disorders
	Scleroderma-Related Features and Mood Disorders
	Possible Pathogenic Mechanisms of Mood Disorders in SSc
	Patients’ Perspectives
	How to Provide Better Health Care to SSc Patients with Mood Disorders
	Conclusion
	References
Chapter 7: Raynaud’s Phenomenon
	What Is Raynaud’s Phenomenon?
	The Clinical Features of Raynaud’s Pheneomen
	The Relevance of Raynaud’s Phenomenon in Scleroderma
	The Burden of Raynaud’s Phenomenon in Systemic Sclerosis
	Clinical Vignette
		Case History
		Point for Consideration
	Assessment and Management of Scleroderma-Related Raynaud’s Phenomenon
	Conclusions
	References
Chapter 8: Critical Ischemia
	Case Presentation
	Definitions and Pathogenetic Pathways
	Risk Factors for Developing Critical Ischemia
	Therapeutic Approaches
		Interdisciplinary Team Care
	Detailed Treatment Approach
		Macrovascular Ischemia
		Microvascular Ischemia—The Acute Setting
	Adjunctive Therapies
	Revisiting the Case
	References
Chapter 9: Increase in Pulmonary Artery Pressures
	Patient 1: Routine Follow-Up Patient
	Patient 2: Elevated Pulmonary Pressures with Breathlessness
	References
Chapter 10: Digital Ulcers
	Introduction
	Microvascular and Microvascular Assessment
	Pain Evaluation and its Management
	Disability and Functionality Assessment and its Management
	Complications and Their Management
	Local Treatment: Wound Bed Preparation
	Systemic Treatment: Healing and Prevention
	Conclusion
	References
Chapter 11: Lower Limb Ulcers
	Background
		Clinical Assessment of the Lower Limb Lesions
		How Would you Classify the Limb Ulcers Described Above?
		How Should These Ulcers Be Managed?
			Comments to the Questions Regarding Clinical Case
	References
Chapter 12: Interpretation of PFTs and Decline in PFTs
	Patient Examples
	References
Chapter 13: Arrhythmias
	Definitions
	Clinical Cases
	Discussion
	Diagnostic Work-Up
	Treatment
	References
Chapter 14: Pericardial and Pleural Complications in Systemic Sclerosis
	Background
	Clinical Case
	References
Chapter 15: Constipation, Bloating and Abdominal Pain
	Introduction
	Clinical Case
	Constipation
		Pathophysiology
		Diagnosis
		Investigations
		Treatment
	Bloating and Abdominal Pain
		Pathophysiology
		Diagnosis
		Investigations
		Treatment
	References
Chapter 16: Gastroesophageal Reflux
	Clinical Vignette
	Epidemiology
	Pathogenesis
	Clinical Presentation
	Diagnostic Evaluation
	Treatment
	Incomplete Response to Proton Pump Inhibition in SSc Patients
	Exacerbating Factors
	Summary
	References
Chapter 17: Management Diarrhea in Systemic Sclerosis
	Introduction
	Pathogenesis of Lower GIT Dysfunction in SSc
	Assessment of Lower GIT Dysfunction in SSc
	Clinical Cases: Focus on Diarrhea in SSc
		Case 1: JK
			Presentation
			Intervention
		Case 2: SC
			Presentation
			Intervention
	Summary and Recommendations
	References
Chapter 18: Gastric Antral Vascular Ectasia (GAVE)
	Introduction
	Epidemiology
	Pathogenesis
	Clinical Aspects
	Diagnosis
	Management
		Medical Management
		Endoscopic Management
		Surgical Treatments
	Prognosis
	Conclusion
	References
Chapter 19: Management of Orofacial Complications
	Introduction
	Limitation of Mouth Opening
		Description
		Reported Symptoms
		Clinical Signs
		Management
	Xerostomia
		Description
		Reported Symptoms
		Clinical Signs
		Management
	Periodontal Diseases
		Description
		Reported Symptoms
		Clinical Signs
		Management
	Clinical Case
	Conclusion
	References
Chapter 20: Practical Approach to Malnutrition and Weight Loss in SSc
	Prevalence of Malnutrition in Systemic Sclerosis
	Gastrointestinal Involvement and Malnutrition in Systemic Sclerosis
	SIBO and Malnutrition
	Clinical Features of SSc Patients at Risk of Malnutrition
	Clinical Management of Malnutrition in SSc
	References
Chapter 21: Calcinosis
	Introduction
	Case History
	Epidemiology (Including Associates) of Calcinosis
	Clinical Features
	Imaging of Calcinosis
	Management
		General Measures
		Surgical Debridement
		Specific Therapies Aimed at Reducing Calcinosis
	Conclusions
	References
Chapter 22: Pregnancy and Scleroderma
	Case 1
	Case 2
	References
Chapter 23: Sjogren’s Syndrome (Ss) in Progressive Systemic Sclerosis (SSc)
	Introduction
	A Vignette Case of Co-Existence of pSs with SSc
	Common Denominators in Pathogenesis of pSs and SSc
		The Role of Epithelial Cell in pSs and SSc
		Epigenetic Alterations in pSs and SSc
		Activation of Interferon-I Pathway
	Clinical Manifestations of pSs
	Sjogren Syndrome in Scleroderma
	Common Problems in the DD?
	Diagnosis and Treatment
	Conclusion
	References
Chapter 24: Male Sexual Dysfunction in Systemic Sclerosis
	Case
	Background
	Management
	References
Chapter 25: How to Assess a Patient for HSCT
	References
Chapter 26: Very Early Systemic Sclerosis: VEDOSS Type Case
	Background
	Clinical Case 1
	Clinical Case 2
	Discussion
	References
Chapter 27: Primary Biliary Cholangitis
	Epidemiology of PBC
	Diagnosis
	Natural History
	Clinical Manifestations
	Extrahepatic Autoimmune Conditions Associated with PBC
	Risk Stratification
	Treatment
	General Measures
	Conclusions
	References
Chapter 28: Arthritis in Systemic Sclerosis
	Introduction
	Prognostic Factors in Scleroderma Associated Inflammatory Arthritis
	Types of Arthritis
	Diagnosis of Arthritis in SSc
		Who Gets Inflammatory Arthritis in SSc
		Other MSK Features in SSc
	Treatment
		Non-pharmacological
		Pharmacological Treatment
			Analgesics and NSAIDs
		Low Dose Corticosteroids
		Disease Modifying Anti-Rheumatic Drugs (DMARDs)
		Cannabinoids
	Proposed Outcome Measurements in SSc Clinical Trials for Inflammatory Arthritis
	Future Directions
	References
Chapter 29: Systemic Sclerosis-Related Myopathy
	Case
	Discussion
	References
Chapter 30: Clinical Management of Tenosynovitis and Tendon Friction Rubs in Systemic Sclerosis
	Introduction
		Tenosynovitis
		Tendon Friction Rubs (TFRs)
	Clinical Management
		Non-pharmacologic Management
		Pharmacologic Treatment
	References
Chapter 31: Management Challenges in the Early Systemic Sclerosis Population: Navigating Side Effects of Therapeutics and Approach to Diarrhea
	Clinical Vignettes
	Discussion
	References
		Suggested Reading
Index