Abstract
Primary antiphospholipid syndrome (APS) is a disease characterized by the presence of autoantibodies reacting with proteins bound to phospholipids, leading to thrombosis and gestation abnormalities. Prothrombotic states and impaired clot dissolution are believed to contribute to the occurrence of chronic thromboembolic pulmonary hypertension (CTEPH) in APS. Whether preventive anticoagulation therapy in patients with antiphospholipid autoantibodies without a history of thrombosis reduces the risk of thrombosis is currently unclear. The diagnosis and treatment of CTEPH in APS is similar to CTEPH complicated by other predisposing conditions, with surgical treatment being the most effective. However, not every patient with CTEPH is suitable for pulmonary thromboendartarectomy and such individuals may benefit from pharmacotherapy of pulmonary hypertension, given the presence of pulmonary microvascular abnormalities similar to those in idiopathic pulmonary hypertension. Anticoagulation therapy is the mainstay of management because of the high risk of recurrent embolization and local in-situ thrombosis.
Keywords: Antiphospholipid syndrome, pulmonary hypertension, thrombosis, pulmonary embolism.
Current Pharmaceutical Design
Title:Primary Antiphospholipid Syndrome and Pulmonary Hypertension
Volume: 20 Issue: 4
Author(s): Aibek E. Mirrakhimov and Nicholas S. Hill
Affiliation:
Keywords: Antiphospholipid syndrome, pulmonary hypertension, thrombosis, pulmonary embolism.
Abstract: Primary antiphospholipid syndrome (APS) is a disease characterized by the presence of autoantibodies reacting with proteins bound to phospholipids, leading to thrombosis and gestation abnormalities. Prothrombotic states and impaired clot dissolution are believed to contribute to the occurrence of chronic thromboembolic pulmonary hypertension (CTEPH) in APS. Whether preventive anticoagulation therapy in patients with antiphospholipid autoantibodies without a history of thrombosis reduces the risk of thrombosis is currently unclear. The diagnosis and treatment of CTEPH in APS is similar to CTEPH complicated by other predisposing conditions, with surgical treatment being the most effective. However, not every patient with CTEPH is suitable for pulmonary thromboendartarectomy and such individuals may benefit from pharmacotherapy of pulmonary hypertension, given the presence of pulmonary microvascular abnormalities similar to those in idiopathic pulmonary hypertension. Anticoagulation therapy is the mainstay of management because of the high risk of recurrent embolization and local in-situ thrombosis.
Export Options
About this article
Cite this article as:
Mirrakhimov E. Aibek and Hill S. Nicholas, Primary Antiphospholipid Syndrome and Pulmonary Hypertension, Current Pharmaceutical Design 2014; 20 (4) . https://dx.doi.org/10.2174/138161282004140213125951
DOI https://dx.doi.org/10.2174/138161282004140213125951 |
Print ISSN 1381-6128 |
Publisher Name Bentham Science Publisher |
Online ISSN 1873-4286 |
Call for Papers in Thematic Issues
"Tuberculosis Prevention, Diagnosis and Drug Discovery"
The Nobel Prize-winning discoveries of Mycobacterium tuberculosis and streptomycin have enabled an appropriate diagnosis and an effective treatment of tuberculosis (TB). Since then, many newer diagnosis methods and drugs have been saving millions of lives. Despite advances in the past, TB is still a leading cause of infectious disease mortality ...read more
Current Pharmaceutical challenges in the treatment and diagnosis of neurological dysfunctions
Neurological dysfunctions (MND, ALS, MS, PD, AD, HD, ALS, Autism, OCD etc..) present significant challenges in both diagnosis and treatment, often necessitating innovative approaches and therapeutic interventions. This thematic issue aims to explore the current pharmaceutical landscape surrounding neurological disorders, shedding light on the challenges faced by researchers, clinicians, and ...read more
Emerging and re-emerging diseases
Faced with a possible endemic situation of COVID-19, the world has experienced two important phenomena, the emergence of new infectious diseases and/or the resurgence of previously eradicated infectious diseases. Furthermore, the geographic distribution of such diseases has also undergone changes. This context, in turn, may have a strong relationship with ...read more
Melanoma and Non-Melanoma Skin Cancer Treatment: Standard of Care and Recent Advances
In this thematic issue, we aim to provide a standard of care of the diagnosis and treatment of melanoma and non-melanoma skin cancer. The editor will invite authors from different countries who will write review articles of melanoma and non-melanoma skin cancers. The Diagnosis, Staging, Surgical Treatment, Non-Surgical Treatment all ...read more
- Author Guidelines
- Graphical Abstracts
- Fabricating and Stating False Information
- Research Misconduct
- Post Publication Discussions and Corrections
- Publishing Ethics and Rectitude
- Increase Visibility of Your Article
- Archiving Policies
- Peer Review Workflow
- Order Your Article Before Print
- Promote Your Article
- Manuscript Transfer Facility
- Editorial Policies
- Allegations from Whistleblowers
- Announcements
Related Articles
-
Acute Coronary Syndromes in Patients with Atrial Fibrillation and Heart Failure. Could Novel Oral Anticoagulants be the Solution of the Optimal Antithrombotic Therapy Puzzle?
Cardiovascular & Hematological Agents in Medicinal Chemistry Apoptogenic Signal Transduction in Arteriosclerosis-Associated Cells – Opportunities for Future Therapy?
Vascular Disease Prevention (Discontinued) Metalloproteinase-Mediated Shedding of Heparin-Binding Egf-Like Growth Factor and Its Pathophysiological Roles
Protein & Peptide Letters Renin-Angiotensin-Aldosterone System Antagonists and the Prevention of Type 2 Diabetes Mellitus
Current Pharmaceutical Design The Role of Nitric Oxide on Endothelial Function
Current Vascular Pharmacology Salutary Properties of YC-1 in the Cardiovascular and Hematological Systems
Current Medicinal Chemistry - Cardiovascular & Hematological Agents Nocturnal Hypertension and Chronic Kidney Disease
Current Hypertension Reviews Transforming Growth Factor β Signaling Perturbation in the Loeys-Dietz Syndrome
Current Medicinal Chemistry Antithrombotic Treatment Management in Patients with Intracerebral Hemorrhage: Reversal and Restart
Current Pharmaceutical Design Vascular Reactions of Iodinated X-Ray Contrast Media: Mechanisms and Possible Therapeutic Interventions
Anti-Inflammatory & Anti-Allergy Agents in Medicinal Chemistry Detecting the Association Between Residual Thrombus and Post-thrombotic Classification of Chronic Venous Disease with Range Regression
Reviews on Recent Clinical Trials An Overview on Cardiovascular Protective Effects of Catechins
Cardiovascular & Hematological Disorders-Drug Targets The Role of Diabetes Mellitus in Sexual and Reproductive Health: An Overview of Pathogenesis, Evaluation, and Management
Current Diabetes Reviews Novel Biomarkers Assessing the Calcium Deposition in Coronary Artery Disease
Current Medicinal Chemistry Comparison of Changes in Cognitive Functions of Post-stroke Patients with the Computer-based Cognitive Intervention (PABLO System) and Conventional Cognitive Intervention (Paper-pencil Method)
Current Psychiatry Research and Reviews Transcranial Doppler Ultrasonography: Current Status
Current Medical Imaging Update on the Medical Treatment of Allergic Rhinitis
Inflammation & Allergy - Drug Targets (Discontinued) The Synthesis of Coenzyme Q10
Current Organic Chemistry Choice of Antihypertensive Treatment in Subjects with Pre-Diabetes. Is There a Dream After the Navigator
Current Vascular Pharmacology Sulfur Containing Scaffolds in Drugs: Synthesis and Application in Medicinal Chemistry
Current Topics in Medicinal Chemistry