New Horizons: Autoimmune-Related Thrombosis

Dear Colleagues,

The development of certain transient or chronic autoantibodies has long been recognized among the various factors causing thrombosis in clinical conditions like Lupus Anticoagulant (LA), Anti-Phospholipid Syndrome (APS), or Heparin-Induced Thrombocytopenia and Thrombosis (HIT/HITT). These clinical contexts are nowadays extensively investigated and well-documented in the literature. The primary autoantigens identified are prothrombin and β2-GlycoProtein 1 (β2GP1), while more rarely, other phospholipid binding proteins that induce LA/APS, complexed with or without phospholipids, include Platelet Factor 4 (PF4) complexed with heparin, which can cause HIT/HITT, even as autoantibodies remain asymptomatic in many heparin-treated patients. Aside from these two main complications, a wide variety of autoantibodies, occurring rarely to very rarely, have been reported or are suspected to cause thrombosis in isolated cases, and in very diverse clinical situations. These pathologies are associated with variable triggers, like the intake of specific drugs or infectious diseases, mainly viral, or diseases altering human biological structures like malignancy. The implicated autoantigens are not always well identified, although they have been characterized in other thrombotic diseases like ADAMTS-13 in Thrombotic Thrombocytopenic Purpura (TTP); coagulation protein S in chicken pox; prothrombin or PF4 in some adenoviral infections; factor V in second-line antibiotic therapy; factor XIII, thrombin, protein Z, interleukin-8 in non-identified causative contexts or in some tumoral diseases; and PF4 in Vaccine-Induced Thrombotic Thrombocytopenia (VITT). Eventually, the autoantigen is only suspected through its cofactor, for example, phosphatidyl-ethanolamine in thrombotic diseases associated with acquired protein C deficiency. This list is not exhaustive and new targeted autoantigens are continuously identified parallel to technological developments and investigations that enable their characterization. This Special Issue focuses on the autoantibodies associated with thrombosis and welcomes reviews, clinical studies, or case reports in this field. It aims to synthesize the present understanding of these rare clinical complications, and provide a review on the leading causes of these autoantibodies, how they can induce pathology, and how affected patients can be managed. In addition, laboratory tools designed for detecting these autoantibodies, monitoring their pathophysiological consequences, and observing their evolution or association with the disease course will be presented. We invite experts and clinicians working on these topics to share their work in this Special Issue and to contribute to enhancing the knowledge and management of these rare pathological complications, helping mitigate their clinical burden, especially in the context of their life-threatening manifestations.

Dr. Jean Amiral
Dr. Osamu Kumano
Guest Editors

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• chronic or transient autoantibodies
• autoimmune thrombosis
• lupus anticoagulant (LA)
• thrombotic thrombocytopenic Purpura (TTP)
• anti-phospholipid syndrome (APS)
• heparin induced thrombocytopenia/and thrombosis (HIT/HITT)
• vaccine induced thrombotic thrombocytopenia (VITT)