Scleroderma - modern aspects of pathogenesis, diagnosis and therapy
2nd Edition
Published on 26. February 2014
127 pages
978-3-8374-6256-2 (ISBN)
Description
Systemic sclerosis is a chronic autoimmune disease with a complex pathophysiology and potential involvement of multiple organs. The second edition of this textbook gives a practically oriented overview on epidemiology, pathophysiology, classification, disease manifestations and treatment options. Particular attention is paid to novel developments in the classification criteria, molecular targets for therapy and recently approved treatments for organ manifestations. It gives a comprehensive overview for both the experienced clinician and younger colleagues.
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Content
- Intro
- Authors
- Contents
- 1. Classification, epidemiology and diagnosis
- 1.1. Classification
- 1.2. Epidemiology
- 1.3. Diagnosis of systemic sclerosis
- 1.4. Diagnosis of internal organ involvement
- 1.5. References
- 2. The pathogenic process(es) in scleroderma
- 2.1. Introduction
- 2.2. Early SSc disease manifestations vasculopathy or immune activation?
- 2.2.1. Vasculopathy: endothelial cell injury and dysregulation angiogenesis and vasculogenesis
- 2.2.2. Immune activation, inflammation and autoimmunity in SSc
- 2.3. Connective tissue fibrosis in SSc
- 2.3.1. The extracellular matrix
- 2.3.2. Cellular determinants of connective tissue fibrosis
- 2.3.3. Mediators and cell signal transduction pathways in connective tissue fibrosis
- 2.4. Concluding remarks
- 2.5. References
- 3. Interstitial lung disease in SSc
- 3.1. Importance of interstitial lung disease
- 3.2. Prevalence of interstitial lung disease
- 3.3. Identification of clinically significant lung involvement
- 3.4. Pathogenesis
- 3.5. Histological and CT patterns of lung involvement
- 3.6. Who should be treated?
- 3.7. BAL is of limited value
- 3.8. Therapeutic data
- 3.9. References
- 4. Pulmonary arterial hypertension in systemic sclerosis
- 4.1. Epidemiology
- 4.2. Risk Factors
- 4.3. Clinical Presentation
- 4.4. Biomarkers
- 4.5. Diagnosis and Screening
- 4.6. Treatment
- 4.7. References
- 5. The GI tract in SSc
- 5.1. Symptoms and pathophysiology
- 5.2. Diagnostic approaches
- 5.3. Therapy
- 5.4. References
- 6. Kidney manifestations in systemic sclerosis
- 6.1. Scleroderma renal crisis
- 6.2. Clinical presentation
- 6.3. Risk factors
- 6.4. Diagnosis
- 6.5. Treatment
- 6.6. Prognosis
- 6.7. Other forms of renal disease in systemic sclerosis
- 6.8. References
- 7. Peripheral vasculopathy including Raynaud's in SSc
- 7.1. Definition of Raynaud's phenomenon
- 7.1.1. Epidemiology of secondary Raynaud's phenomenon
- 7.1.2. Assessment of Raynaud's phenomenon
- 7.1.3. Pathophysiology of primary and SSc-associated RP
- 7.2. Digital ulcers (DU)
- 7.2.1. Pathophysiology of digital ulcers
- 7.3. Therapy of Raynaud's phenomenon and of digital ulcers
- 7.3.1. Medical treatment
- 7.3.2. Is there any role of immunosuppression in the treatment of RP and digital ulcers?
- 7.3.3. Additional therapies of digital ulcers
- 7.4. References
- 8. The heart in systemic sclerosis
- 8.1. Prognostic implication of cardiac involvement
- 8.2. Pathophysiology
- 8.3. Assessment of heart involvement
- 8.4. Therapy
- 8.5. References
- 9. Gender related issues in systemic sclerosis
- 9.1. SSc related complications specific to women
- 9.1.1. Female sexuality and genital tract symptoms
- 9.1.2. Fertility of women with SSc
- 9.1.3. Pregnancy in SSc
- 9.1.3.1. Effects of pregnancy on the baby
- 9.1.3.2. Effects of pregnancy on SSc
- 9.1.3.3. Management of delivery
- 9.2. SSc related complications specific to men
- 9.2.1. Erectile dysfunction in the general population
- 9.2.2. Mechanism of erection
- 9.2.3. Prevalence of erectile dysfunction in SSc
- 9.2.4. Causes of erectile dysfunction in SSc
- 9.2.5. Treatment of erectile dysfunction in SSc
- 9.3. References
- 10. DMARDS in systemic sclerosis: Do they exist?
- 10.1. Vascular approach
- 10.2. Immunosuppressive approach
- 10.3. Antifibrotic approach
- 10.4. References
- 11. Future therapies: Novel therapeutic regimens and molecular targets
- 11.1. High dose immunosuppressive therapy and autologous hematopoietic stem cell transplantation
- 11.2. B cell depletion
- 11.3. Interleukin-6 receptor
- 11.4. Neutralising antibodies against TGF 1
- 11.5. Tyrosine kinase inhibitors
- 11.6. Peroxisome proliferatoractivated receptoragonists
- 11.7. Antagonists of the 5-HT receptor 2B
- 11.8. Inhibitors of morphogenic pathways
- 11.9. References
- Index
