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Eosinophilia and thrombophilia in churg strauss syndrome: a clinical and pathogenetic overview
Paul R J Ames1, Maurizio Margaglione, Sara Mackie
1Haematology Department, Airedale General Hospital, Steeton and Immunoclot Ltd, Leeds, United Kingdom. paxmes@aol.com
Insights
Churg Strauss Syndrome (CSS) involves vascular occlusions due to eosinophils. This review details thrombosis prevalence and pathogenesis in CSS, highlighting the role of eosinophils in clot formation.
Area of Science:
- Rheumatology
- Hematology
- Pathology
Background:
- Churg Strauss Syndrome (CSS) is increasingly associated with vascular occlusions.
- The thrombogenic potential of eosinophils in CSS is often underestimated.
- Understanding thrombosis in CSS is crucial for patient management.
Purpose of the Study:
- To evaluate the prevalence of thrombosis in CSS patient series.
- To demonstrate the potential for vascular occlusion in any anatomical location within CSS.
- To describe the underlying mechanisms of thrombosis in CSS.
Main Methods:
- Systematic literature search of PubMed, EMBASE, and Google for CSS series (1951-present).
- Analysis of reported prevalence rates for arterial and venous occlusions.
- Review of pathogenetic mechanisms involving eosinophils and ANCA.
Main Results:
- Prevalence of arterial occlusion in CSS series ranges from 3.1% to 18.7%.
- Prevalence of venous occlusion in CSS series ranges from 5.8% to 30%.
- Specific surveys indicate an 8.1% prevalence of venous thromboembolism in CSS.
Conclusions:
- Eosinophils contribute to thrombosis in CSS by releasing tissue factor and cationic proteins, promoting coagulation and platelet activation.
- Antineutrophil cytoplasmic antibodies (ANCA) may induce a prothrombotic endothelial state.
- Further research into CSS-associated thrombosis is warranted.
Abstract:
During the past decade, there has been an increased description of Churg Strauss syndrome (CSS) characterized by vascular occlusions possibly linked to the thrombogenic potential of the eosinophil that is poorly appreciated. The purpose of this overview is 3-fold: the first to evaluate the available prevalence of thrombosis in Churg Strauss series, the second to demonstrate that any vascular district may be affected, and the third to describe the pathogenesis of thrombosis in CSS. A Pubmed, EMBASE, and Google search of CSS series from 1951 to date revealed a prevalence of arterial occlusion ranging between 3.1% and 18.7% and a prevalence of venous occlusion between 5.8% and 30%, whereas a specific survey for venous thromboembolism in CSS yielded a prevalence of 8.1%. Eosinophils store and release tissue factor as well as other cationic proteins: the former initiates coagulation while the latter inhibits natural anticoagulant activity and activate platelets eventually culminating in excessive thrombin generation and clot formation. In addition, antineutrophil cytoplasmic antibodies may shift the endothelial lining to proadhesive and prothrombotic surface. It is hoped that the review will represent a basis to foster novel research on this topic.
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