Abstract
ObjectivesGiven reports on the increased prevalence of thromboembolic incidents in patients with eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome), we investigated whether fibrin clot properties are unfavorably altered in EGPA.MethodsEx vivo plasma fibrin clot characteristics, including clot permeability, turbidimetry and efficiency of fibrinolysis using two assays, were investigated in 34 consecutive patients with remission in EGPA according to the Birmingham Vasculitis Activity Score version 3 (23 female, 11 male), aged 48 (range, 21–80) years. The control group comprised 34 age- and sex- matched volunteers.ResultsCompared with controls, patients with EGPA were characterized by denser fiber clots (estimated pore size, Ks, 7.30±0.93 vs 10.14±1.07 10−9 cm2), faster fibrin polymerization (lag phase in a turbidimetric curve, 41.8±3.6 vs 47.4±2.9 s), thicker fibrin fibers (maximum absorbance, ΔAbs, 0.87±0.09 vs 0.72±0.07), higher maximum levels of D-dimer released from clots (DDmax 4.10±0.46 vs 3.54±0.35 mg/L), and prolonged clot lysis time (t50%; 9.50±1.45 vs 7.56±0.87 min); all p<0.0001. Scanning electron microscopy images confirmed denser plasma fibrin networks composed of thinner fibers formed in EGPA. Antineutrophil cytoplasmic antibody status and C-reactive protein did not affect clot variables. Multivariate analysis adjusted for fibrinogen showed that Ks was predicted by eosinophil count, peak thrombin generation, factor VIII, and soluble CD40 ligand, whereas eosinophil count, peak thrombin generation and antiplasmin predicted t50%.ConclusionThis study is the first to show that EGPA is associated with prothrombotic plasma fibrin clot phenotype, which may contribute to thromboembolic manifestations reported in this disease.
Highlights
Patients with Eosinophilic granulomatosis with polyangiitis (EGPA) were characterized by denser fiber clots, faster fibrin polymerization, thicker fibrin fibers, higher maximum levels of D-dimer released from clots (DDmax 4.10±0.46 vs 3.54±0.35 mg/L), and prolonged clot lysis time (t50%; 9.50±1.45 vs 7.56±0.87 min); all p
This study is the first to show that EGPA is associated with prothrombotic plasma fibrin clot phenotype, which may contribute to thromboembolic manifestations reported in this disease
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare disease characterized by necrotising vasculitis involving small and medium blood vessels accompanied by eosinophilia in the peripheral blood, eosinophilic infiltration of the tissues and the presence of bronchial asthma [1, 2]
Summary
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare disease characterized by necrotising vasculitis involving small and medium blood vessels accompanied by eosinophilia in the peripheral blood, eosinophilic infiltration of the tissues and the presence of bronchial asthma [1, 2]. Patients with active antineutrophil cytoplasmic antibody-associated vasculitis are at increased risk of developing venous thromboembolic events (VTE) that is about 7 per 100 person-years as compared to 0.15–0.31 per 100 person-years in the general population. The risk probably remains elevated during remission (about 1.0 per 100 person-years)[4]. Several mechanisms such as inflammation, coexisting heart failure and peripheral blood hypereosinophilia have been suggested as a potential cause of thromboembolism in patients with EGPA [5]. It has been reported that eosinophils release tissue factor, the major in vivo initiator of blood coagulation [9], this observation was not confirmed in experiments on isolated cells [10]
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