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.

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