Antiphospholipid Syndrome in Systemic Autoimmune Diseases

Chapter 1. History, Classification, and Subsets of the Antiphospholipid Syndrome

• Abstract
• 1.1 Introduction
• 1.2 Historical Perspective
• 1.3 Classification of APS
• 1.4 Primary or Isolated APS
• 1.5 APS Associated With Other Diseases
• 1.6 Seronegative APS
• 1.7 Catastrophic APS
• 1.8 International aPL/APS Congresses
• 1.9 APS Action
• References

Chapter 2. Epidemiology of the Antiphospholipid Syndrome

• Abstract
• 2.1 Introduction
• 2.2 APS in the General Population
• 2.3 aPL and Venous Thrombosis
• 2.4 aPL and Arterial Thrombosis
• 2.5 aPL and Pregnancy Morbidity
• 2.6 aPL and SLE
• 2.7 Conclusions
• Acknowledgements
• References

Chapter 3. Mechanisms of Action of the Antiphospholipid Antibodies

• Abstract
• 3.1 Introduction
• 3.2 Antiphospholipid Antibodies
• 3.3 aPL-Mediated Mechanisms of Thrombosis
• 3.4 aPL-Mediated Mechanism of Pregnancy Complications
• 3.5 Receptors for β2GPI/Anti-β2GPI Antibodies
• 3.6 Intracellular Pathways
• 3.7 Two-Hit Hypothesis
• 3.8 Genetics and Epigenetics
• 3.9 Conclusions
• References

Chapter 4. Laboratory Markers With Clinical Significance in the Antiphospholipid Syndrome

• Abstract
• 4.1 Introduction
• 4.2 aPL Detected by Solid-Phase Immunoassays
• 4.3 Lupus Anticoagulant
• 4.4 Annexin A5 Resistance Test: A Mechanistic Test for the Detection of Pathogenic aPL Antibodies
• 4.5 New Technologies for the Detection of aPL
• 4.6 Which aPL Should Be Tested in Patients With Suspicion of Having APS?
• 4.7 Conclusions
• Acknowledgements
• References

Chapter 5. Genetic and Epigenetic Aspects of Antiphospholipid Syndrome: What we knew, what we know

• Abstract
• 5.1 HLA, APS, and aPL
• 5.2 The Role of Non-MHC Genes in APS Susceptibility
• 5.3 Thrombophilic Hereditary Factors
• 5.4 Posttranscription Modifications of Anti-β2GPI Antibodies
• 5.5 Conclusions
• References

Chapter 6. Thrombotic Manifestations of the Antiphospholipid Syndrome

• Abstract
• 6.1 Introduction
• 6.2 Large Vessel Manifestations
• 6.3 Neurologic Manifestations
• 6.4 Pulmonary Manifestations
• 6.5 Cardiac Manifestations
• 6.6 Renal Manifestations
• 6.7 Haematologic Manifestations
• 6.8 Dermatologic Manifestations
• 6.9 Hepatic and Digestive Manifestations
• 6.10 Adrenal Manifestations
• 6.11 Osteoarticular Manifestations
• 6.12 Catastrophic APS
• References

Chapter 7. Obstetric Manifestations of the Antiphospholipid Syndrome

• Abstract
• 7.1 Introduction
• 7.2 Recurrent Early Pregnancy Loss
• 7.3 Foetal Death
• 7.4 Preeclampsia and PI
• 7.5 Risk Stratification
• 7.6 Conclusions
• References

Chapter 8. Thrombocytopenia in the Antiphospholipid Syndrome

• Abstract
• 8.1 History and Definition
• 8.2 Prevalence in Primary and Secondary APS
• 8.3 Pathogenesis
• 8.4 Treatment
• 8.5 Immune Thrombocytopenic Purpura
• 8.6 Heparin-Induced Thrombocytopenia
• 8.7 Thrombotic Microangiopathy
• 8.8 Pseudothrombocytopenia
• 8.9 Disseminated Intravascular Coagulation
• References

Chapter 9. Nonclassification Criteria Manifestations of the Antiphospholipid Syndrome

• Abstract
• 9.1 Introduction
• 9.2 Nonclassification Criteria Manifestations
• References

Chapter 10. Paediatric Antiphospholipid Syndrome

• Abstract
• 10.1 Introduction
• 10.2 Epidemiology
• 10.3 Clinical Manifestations
• 10.4 Differential Diagnosis
• 10.5 Treatment and Outcome
• References

Chapter 11. Antiphospholipid Antibodies and Their Relationship With Infections, Vaccines, and Drugs

• Abstract
• 11.1 Introduction
• 11.2 aPL Associated With Infections
• 11.3 The Infection Origin of APS
• 11.4 aPL and Vaccination
• 11.5 Drug-Induced aPL
• 11.6 Summary
• References

Chapter 12. Antiphospholipid Syndrome Associated With Malignancies

• Abstract
• 12.1 Introduction
• 12.2 Solid and Haematological Malignancies and aPL
• 12.3 Catastrophic APS and Malignancies
• 12.4 Therapeutic Aspects
• 12.5 Conclusion
• References

Chapter 13. Antiphospholipid Antibodies and Atherosclerosis

• Abstract
• 13.1 Introduction
• 13.2 Clinical Evidence for or Against a Relationship Between aPL and Atherosclerosis
• 13.3 Classic aPL: Can They Explain All Autoimmune Mechanisms for Atherosclerosis or are They the Tip of an Iceberg?
• 13.4 Intravascular Autoantibodies: Evidence for Effects on Traditional Cardiovascular Risk Factors
• 13.5 Natural Autoantibodies: Can They Protect Against Atherosclerosis, and are They Related to aPL?
• References

Chapter 14. Global Antiphospholipid Syndrome Score

• Abstract
• 14.1 Introduction
• 14.2 The Global APS Score
• 14.3 External Validations of GAPSS
• 14.4 Conclusion
• References

Chapter 15. Primary Prophylaxis in Patients With Positive Antiphospholipid Antibodies

• Abstract
• 15.1 Introduction
• 15.2 Conclusion
• References

Chapter 16. Treatment of Thrombosis in Antiphospholipid Syndrome

• Abstract
• 16.1 Prevention of Recurrent Thrombosis
• 16.2 Primary Thromboprophylaxis
• 16.3 New Anticoagulant Drugs
• 16.4 Other Therapies
• 16.5 Final Remarks
• References

Chapter 17. Treatment of Catastrophic Antiphospholipid Syndrome

• Abstract
• 17.1 Introduction
• 17.2 Current Approach
• 17.3 New Approaches
• 17.4 Conclusions
• References

Chapter 18. Treatment of Pregnancy Complications in Antiphospholipid Syndrome

• Abstract
• 18.1 Introduction
• 18.2 Obstetric Complications Associated with APS
• 18.3 Mechanisms for Adverse Pregnancy Outcomes
• 18.4 Therapeutic Options for Women with APS
• 18.5 Management Algorithm
• 18.6 Management of Specific Complications
• 18.7 Conclusions
• References

Chapter 19. Prognosis in Antiphospholipid Syndrome

• Abstract
• 19.1 Introduction
• 19.2 Mortality and Morbidity in Patients with Long-Term Follow-Up APS
• 19.3 Obstetric APS
• 19.4 Catastrophic APS
• References