Dataset Information

Assessment of blood clot formation and platelet receptor function ex vivo in patients with primary Sjogren's syndrome.

ABSTRACT:

Objectives

Primary Sjögren's syndrome (pSS) shares clinical features and pathogenetic mechanisms with systemic lupus erythematosus (SLE). SLE is associated with an increased thromboembolic risk; however, it is unclear whether pSS patients are susceptible to thromboembolic diseases. In this study, we examined ex vivo blood clot formation (clot strength, rates of clot formation and lysis) in pSS using thromboelastography (TEG) and platelet aggregation to common agonists using multiple electrode aggregometry (MEA). We also investigated the relationship between TEG/MEA parameters and clinical/laboratory features of pSS.

Design

Case control.

Setting

Secondary care, single centre.

Participants

34 pSS patients, 11 SLE patients and 13 healthy volunteers (all women) entered and completed the study.

Primary and secondary outcome measures

Primary outcomes

TEG and MEA parameters between three subject groups.

Secondary outcomes

The relationships between TEG/MEA and clinical/laboratory parameters analysed using bivariate correlation analysis with corrections for multiple testing.

Results

All TEG and MEA parameters were similar for the three subject groups. After corrections for multiple testing, interleukin (IL)-1? and Macrophage inflammatory proteins (MIP)-1? remain correlated inversely with clot strength (r=-0.686, p=0.024 and r=-0.730, p=0.012, respectively) and overall coagulability (r=-0.640, p=0.048 and r=-0.648, p=0.048). Stepwise regression analysis revealed that several cytokines such as MIP-1?, IL-17a, IL-1? and Interferon (IFN)-? may be key predictors of clot strength and overall coagulability in pSS.

Conclusions

Clot kinetics and platelet receptor function are normal in pSS. Several cytokines correlate with clot strength and overall coagulability in pSS.

SUBMITTER: Collins KS

PROVIDER: S-EPMC3668418 | biostudies-literature | 2013 May

REPOSITORIES: biostudies-literature

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Publications

Assessment of blood clot formation and platelet receptor function ex vivo in patients with primary Sjogren's syndrome.

Collins K S KS Balasubramaniam K K Viswanathan G G Natasari A A Tarn J J Lendrem D D Mitchell S S Zaman A A Ng W F WF

BMJ open 20130528 5

<h4>Objectives</h4>Primary Sjögren's syndrome (pSS) shares clinical features and pathogenetic mechanisms with systemic lupus erythematosus (SLE). SLE is associated with an increased thromboembolic risk; however, it is unclear whether pSS patients are susceptible to thromboembolic diseases. In this study, we examined ex vivo blood clot formation (clot strength, rates of clot formation and lysis) in pSS using thromboelastography (TEG) and platelet aggregation to common agonists using multiple elec ...[more]

PMID: 23793707

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Project description:Thrombosis, like other cardiovascular diseases, has a strong genetic component, with largely unknown determinants. EMILIN2, Elastin Microfibril Interface Located Protein2, was identified as a candidate gene for thrombosis in mouse and human quantitative trait loci studies. EMILIN2 is expressed during cardiovascular development, on cardiac stem cells, and in heart tissue in animal models of heart disease. In humans, the EMILIN2 gene is located on the short arm of Chromosome 18, and patients with partial and complete deletion of this chromosome region have cardiac malformations. To understand the basis for the thrombotic risk associated with EMILIN2, EMILIN2 deficient mice were generated. The findings of this study indicate that EMILIN2 influences platelet aggregation induced by adenosine diphosphate, collagen, and thrombin with both EMILIN2-deficient platelets and EMILIN2-deficient plasma contributing to the impaired aggregation response. Purified EMILIN2 added to platelets accelerated platelet aggregation and reduced clotting time when added to EMILIN2-deficient mouse and human plasma. Carotid occlusion time was 2-fold longer in mice with platelet-specific EMILIN2 deficiency, but stability of the clot was reduced in mice with both global EMILIN2 deficiency and with platelet-specific EMILIN2 deficiency. In vitro clot retraction was markedly decreased in EMILIN2 deficient mice, indicating that platelet outside-in signaling was dependent on EMILIN2. EMILIN1 deficient mice and EMILIN2:EMILIN1 double deficient mice had suppressed platelet aggregation and delayed clot retraction similar to EMILIN2 mice, but EMILIN2 and EMILIN1 had opposing affects on clot retraction, suggesting that EMILIN1 may attenuate the effects of EMILIN2 on platelet aggregation and thrombosis. In conclusion, these studies identify multiple influences of EMILIN2 in pathophysiology and suggest that its role as a prothrombotic risk factor may arise from its effects on platelet aggregation and platelet mediated clot retraction.

Dataset Information

Assessment of blood clot formation and platelet receptor function ex vivo in patients with primary Sjogren's syndrome.

Objectives

Design

Setting

Participants

Primary and secondary outcome measures

Primary outcomes

Secondary outcomes

Results

Conclusions

Publications

Assessment of blood clot formation and platelet receptor function ex vivo in patients with primary Sjogren's syndrome.

Similar Datasets

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