This book provides a practical guide for managing a variety of problems encountered by the clinician in managing patients with systemic sclerosis. Chapters take a problem-orientated approach to help the reader cut through potential barriers that can arise when working with different medical specialities. Management strategies for a broad range of conditions, including pericardial and pleural effusion, sicca syndrome, calcinosis and watermelon stomach, are presented.
Practical Management of Systemic Sclerosis in Clinical Practice describes a range of problems and clinical items encountered by a variety of medical professionals who encounter these patients. It is a valuable resource for rheumatologists, immunologists, specialist nurses and primary care professionals.
Author(s): Marco Matucci-Cerinic, Christopher P. Denton
Publisher: Springer
Year: 2021
Language: English
Pages: 399
City: Cham
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
