About this Research Topic
Autoimmune disease is often associated with an increased risk of thrombosis by a variety of mechanisms. As diagnostic techniques have advanced, the role of antibodies that target proteins involved in coagulation for many of these disorders has become more clear. Despite major breakthroughs in our understanding of the pathophysiology of antibody-mediated thrombosis, current treatments remain somewhat broad in the pathways they target, often leading to off-target effects that can be devastating. Similarly, each autoimmune thrombotic disorder manifests in a variety of clinical presentations, and the role of specific antibody targets as they relate to disease severity is an area of active research in the field.
In this review series, we will explore the current state of knowledge of antibody-mediated autoimmune thrombotic disorders. We will review the role of anti-PF4 antibodies in heparin-induced thrombocytopenia as they relate to diagnostic techniques, including recent advances that promise to significantly shorten the time to diagnosis. The effects of pro-thrombotic antibodies in antiphospholipid syndrome will also be explored, with particular attention to what is known about the relationship between antigenic targets and clinical phenotype. We will summarize recent findings regarding the mechanistic effects of anti-ADAMTS13 antibodies in immune thrombotic thrombocytopenic purpura and their pathophysiologic implications. The consequences of anti-platelet and anti-megakaryocyte antibodies in immune thrombocytopenic purpura, which often manifests in a pro-thrombotic disorder in adults, will be discussed. We will review what is known about the antibodies and antigens involved in autoimmune complement-mediated thrombotic microangiopathy. Current treatments for antibody-mediated autoimmune thrombotic disorders and future directions to improve patient care based on the current state of knowledge will be explored.
Identify what is known about the pathophysiology of the disease, with particular attention to recent advances in identifying specific antigens and antibodies involved. Summarize the diagnostic implications regarding such antibodies in terms of how their effects are identified in vitro; the pathway(s) they affect; and what differs about the clinical consequences of such antibodies within a disease state when they are either absent or detectable, and if known, how quantification of antibody-mediated effects guides management, if at all. Describe the current management of antibody-mediated autoimmune thrombotic disorders and speculate as to how more advances will lead to better diagnostic and therapeutic interventions for the disease.
• Identify what is currently known about pathophysiology of the disease
• Summarize diagnostic implications of antibodies if detected
• Describe current management
• Speculate how further knowledge will lead to better diagnostic and therapeutic interventions
In this review series, we will explore the current state of knowledge of antibody-mediated autoimmune thrombotic disorders. We will review the role of anti-PF4 antibodies in heparin-induced thrombocytopenia as they relate to diagnostic techniques, including recent advances that promise to significantly shorten the time to diagnosis. The effects of pro-thrombotic antibodies in antiphospholipid syndrome will also be explored, with particular attention to what is known about the relationship between antigenic targets and clinical phenotype. We will summarize recent findings regarding the mechanistic effects of anti-ADAMTS13 antibodies in immune thrombotic thrombocytopenic purpura and their pathophysiologic implications. The consequences of anti-platelet and anti-megakaryocyte antibodies in immune thrombocytopenic purpura, which often manifests in a pro-thrombotic disorder in adults, will be discussed. We will review what is known about the antibodies and antigens involved in autoimmune complement-mediated thrombotic microangiopathy. Current treatments for antibody-mediated autoimmune thrombotic disorders and future directions to improve patient care based on the current state of knowledge will be explored.
Identify what is known about the pathophysiology of the disease, with particular attention to recent advances in identifying specific antigens and antibodies involved. Summarize the diagnostic implications regarding such antibodies in terms of how their effects are identified in vitro; the pathway(s) they affect; and what differs about the clinical consequences of such antibodies within a disease state when they are either absent or detectable, and if known, how quantification of antibody-mediated effects guides management, if at all. Describe the current management of antibody-mediated autoimmune thrombotic disorders and speculate as to how more advances will lead to better diagnostic and therapeutic interventions for the disease.
• Identify what is currently known about pathophysiology of the disease
• Summarize diagnostic implications of antibodies if detected
• Describe current management
• Speculate how further knowledge will lead to better diagnostic and therapeutic interventions
Keywords: Autoimmune disease; thrombosis; antibody mechanisms; immunomodulatory treatments; coagulopathy
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