Antiphospholipid Syndrome (APS) is an autoimmune disease characterized by thrombotic pathology presenting to a wide range of disciplines from orthopedicians to obstetricians. It is driven by the activity of antiphospholipid antibodies (aPL), which were initially described in the 1980s by the pioneering work of Hughes. The subsequent 4 decades have resulted in several innovations in APS characterization, diagnostic assays and treatment modalities. Recent work on the analysis of APS clinical phenotypes has resulted in an increased understanding of the putative link between disease pathophysiology and clinical outcomes. Laboratory criteria aPL include anticardiolipin (aCL), anti-ß2-glycoprotein I (anti-ß2GPI) and lupus anticoagulant (LA) due to their key role in driving APS pathophysiology. However, several ongoing developments in testing platforms as well as identification of novel antigenic targets may further improve APS diagnosis. While anticoagulant therapy remains the focus of therapeutic approaches for APS, intense basic science and clinical research is focusing on several promising immunomodulatory and biologic agents for the disease.
While significant improvements have been made with regards to diagnosis and management of APS patients over the last 40 years, it is clear that there is yet work to be done. The central role that criteria aPL play in APS disease is supported by extensive research data but there are several drawbacks to these tests in terms of sensitivity, specificity and logistical difficulties. Implementation of automated testing platforms has led to improved intra and inter-assay variation and the development of novel antigenic targets for non-criteria assays have provided promising candidates for improved diagnosis of APS. While APS patients can be reliably grouped into various clinical phenotypes based on presence of thrombosis, obstetric disease, microvascular disease, secondary autoimmune disease and high-risk type aPL; there is limited data on the pathophysiological mechanisms defined by antibody type and signaling pathways that link with a particular phenotype. Immunomodulatory therapies, while typical for most autoimmune diseases, have not been widely used in APS due to the focus on anticoagulation for typical thrombotic and obstetric presentations of the disease. However, promising data in this regard may drive the push to more targeted immunomodulatory APS therapies in the coming years.
The scope of this Research Topic is to gather a collection of basic science and translational research studies that focus on recent advances in APS disease characterization, pathophysiology, diagnosis and therapeutic approaches. This topic seeks to i) Identify novel concepts in the pathophysiological mechanisms that define APS disease ii) characterize clinical phenotypes of APS and any underlying pathophysiological mechanisms; iii) identify new testing paradigms for current criteria aPL assays and non-criteria assays utilizing novel antigenic targets and iv) Identify novel therapeutic approaches for APS disease management.
In this Research Topic, we welcome the submission of Original Research, Reviews, Mini-Reviews, Perspective, Opinion, Clinical Trial, Case Reports, Methods and Protocols focusing on, but not limited to, the following areas:
1. Pathogenic and pathophysiological mechanisms of APS
2. APS phenotypes: thrombosis, microvascular disease, obstetric disease
3. Association of APS and infectious agents
4. The link between APS and other conditions (SLE, RA etc.)
5. Novel biomarkers for APS diagnosis and risk assessment
5. Evaluation of aPL as a diagnostic tool
6. Comparison of test methods for aPL
7. Novel testing methods/antigenic targets for aPL
8. Monoclonal antibodies as APS therapeutic agents
9. Treatment modalities based on APS molecular targets
10. Targeted treatment and prognosis for distinct APS clinical disease profiles
11. Clinical tools for risk and damage assessment in APS
12. The multidisciplinary approach to patients with antiphospholipid syndrome
13. Diagnostic challenges in systemic lupus erythematosus and antiphospholipid syndrome
14. Lessons from the national antiphospholipid syndrome cohort: criteria and non-criteria manifestations
Antiphospholipid Syndrome (APS) is an autoimmune disease characterized by thrombotic pathology presenting to a wide range of disciplines from orthopedicians to obstetricians. It is driven by the activity of antiphospholipid antibodies (aPL), which were initially described in the 1980s by the pioneering work of Hughes. The subsequent 4 decades have resulted in several innovations in APS characterization, diagnostic assays and treatment modalities. Recent work on the analysis of APS clinical phenotypes has resulted in an increased understanding of the putative link between disease pathophysiology and clinical outcomes. Laboratory criteria aPL include anticardiolipin (aCL), anti-ß2-glycoprotein I (anti-ß2GPI) and lupus anticoagulant (LA) due to their key role in driving APS pathophysiology. However, several ongoing developments in testing platforms as well as identification of novel antigenic targets may further improve APS diagnosis. While anticoagulant therapy remains the focus of therapeutic approaches for APS, intense basic science and clinical research is focusing on several promising immunomodulatory and biologic agents for the disease.
While significant improvements have been made with regards to diagnosis and management of APS patients over the last 40 years, it is clear that there is yet work to be done. The central role that criteria aPL play in APS disease is supported by extensive research data but there are several drawbacks to these tests in terms of sensitivity, specificity and logistical difficulties. Implementation of automated testing platforms has led to improved intra and inter-assay variation and the development of novel antigenic targets for non-criteria assays have provided promising candidates for improved diagnosis of APS. While APS patients can be reliably grouped into various clinical phenotypes based on presence of thrombosis, obstetric disease, microvascular disease, secondary autoimmune disease and high-risk type aPL; there is limited data on the pathophysiological mechanisms defined by antibody type and signaling pathways that link with a particular phenotype. Immunomodulatory therapies, while typical for most autoimmune diseases, have not been widely used in APS due to the focus on anticoagulation for typical thrombotic and obstetric presentations of the disease. However, promising data in this regard may drive the push to more targeted immunomodulatory APS therapies in the coming years.
The scope of this Research Topic is to gather a collection of basic science and translational research studies that focus on recent advances in APS disease characterization, pathophysiology, diagnosis and therapeutic approaches. This topic seeks to i) Identify novel concepts in the pathophysiological mechanisms that define APS disease ii) characterize clinical phenotypes of APS and any underlying pathophysiological mechanisms; iii) identify new testing paradigms for current criteria aPL assays and non-criteria assays utilizing novel antigenic targets and iv) Identify novel therapeutic approaches for APS disease management.
In this Research Topic, we welcome the submission of Original Research, Reviews, Mini-Reviews, Perspective, Opinion, Clinical Trial, Case Reports, Methods and Protocols focusing on, but not limited to, the following areas:
1. Pathogenic and pathophysiological mechanisms of APS
2. APS phenotypes: thrombosis, microvascular disease, obstetric disease
3. Association of APS and infectious agents
4. The link between APS and other conditions (SLE, RA etc.)
5. Novel biomarkers for APS diagnosis and risk assessment
5. Evaluation of aPL as a diagnostic tool
6. Comparison of test methods for aPL
7. Novel testing methods/antigenic targets for aPL
8. Monoclonal antibodies as APS therapeutic agents
9. Treatment modalities based on APS molecular targets
10. Targeted treatment and prognosis for distinct APS clinical disease profiles
11. Clinical tools for risk and damage assessment in APS
12. The multidisciplinary approach to patients with antiphospholipid syndrome
13. Diagnostic challenges in systemic lupus erythematosus and antiphospholipid syndrome
14. Lessons from the national antiphospholipid syndrome cohort: criteria and non-criteria manifestations