Background:Thromboembolic cardiovascular diseases (CVD), affecting both arterial and venous sides, are one of the leading causes of death in patients with Idiopathic Inflammatory Myopathies (IIM), with highest peak of mortality within the first year after diagnosis (1).Objectives:To assess the prevalence of traditional and disease-related risk factors for arterial and venous thrombotic events (TE) in patients with IIM by comparing those reporting TE (cases) with those without history of TE (comparators). To compare clinical characteristics, autoantibody profile inclusive antiphospholipid antibodies (aPL) and serum levels of adhesion molecules (VCAM, ICAM and e-selectin) between cases and comparators as well as between cases reporting arterial vs venous TE.Methods:Using national and international registries, and medical charts, we identified 58 cases and 195 comparators with IIM followed at Karolinska University Hospital between 1993 and 2014. Information on gender, age at the time of diagnosis, IIM subgroup, presence of interstitial lung disease (ILD), myositis specific antibodies (MSAs), was retrospectively collected. Information on traditional risk factors for arterial and venous TE (essential hypertension, diabetes, dyslipidemia, smoking, malignancy) was retrieved for both groups. Serum levels of aPL and adhesion molecules were analyzed in stored sera from the time of diagnosis in both groups, before TE in cases and in 40 age and gender matched heathy controls (HC).Results:One out of 5 IIM patients (22,92%) had suffered from at least one TE, which was observed especially during the first 5 years after diagnosis. Myocardial infarction was the most frequent TE, followed by pulmonary embolism and deep venous thrombosis. In the multivariate analysis, male gender and older age were independent risk factors for TE. Essential hypertension had statistically significant higher prevalence in cases than comparators. Arterial TE was more common in polymyositis, while venous TE occurred more frequently in patients with dermatomyositis, history of malignancy and in those with MSAs. At time of IIM diagnosis, the prevalence of aPL was 6% with no difference between cases and comparators. Significantly higher levels of VCAM and ICAM were obtained in IIM patients compared to HC (Fig.1 and Fig.2). ICAM levels were found significantly higher in comparators than cases (Fig.2). Lower levels of e-selectin were associated with higher odds of developing TE, especially in males and older patients, with no difference between arterial and venous TE (Fig.3).Conclusion:A high risk of arterial and venous TE should be taken into account in patients with IIM, particularly close to time of diagnosis, with extra attention in male patients and older individuals. Preventive measures should be considered especially in patients with concomitant essential hypertension and malignancy. Lower serum levels of e-selectin might predict TE in IIM patients but the mechanism for this risk factor is not known.Reference[1] Dobloug GC, Svensson J, Lundberg IE, Ho...
BackgroundPatients with Idiopathic Inflammatory Myopathies (IIM) have an increased risk of thrombotic event (TE) compared with the general population (1). The role of traditional thrombotic risk factors, IIM disease activity, presence of antiphospholipid antibodies (aPL) and adhesion molecules, in the pathogenesis of TE is unknown.ObjectivesWe aimed to investigate the prevalence of TE (pulmonary embolism (PE), deep venous thrombosis (DVT), myocardial infarction (MI), stroke, transitory ischemic attack (TIA) or peripheral arterial thrombosis (PAT)) in IIM patients. We also compared clinical and serological characteristics of IIM patients with and without TE.Methods286 IIM patients followed between 1993–2014, with and without TE, were identified. Information on traditional risk factors for TE, autoantibody profiles, disease activity and medical treatment was retrieved from registries and medical records. Serum levels of anti-β2-glycoprotein I (anti-β2-GPI) and anticardiolipin antibodies (aCL) as well as serum levels of adhesion molecules i.e. vascular cell adhesion molecule 1 (VCAM), intercellular adhesion molecule 1 (ICAM) and e-selectin were analysed on stored sera. Sera of patients with TE were screened for levels of adhesion molecules at the time of IIM diagnosis and at the time of TE. Levels of adhesion molecules were also analysed in 40 healthy individuals.ResultsSixty-seven (23.4%) of the 286 IIM patients suffered from at least one TE, 33 presented with arterial thrombosis (MI, Stroke, TIA, PAT), 31 with venous thrombosis (DVT, PE), 3 with both. Among traditional risk factors for TE, higher age at diagnosis (p<0.001), male gender (p<0.05) and hypertension (p<0.05) occurred more frequently in patients with TE than in those without. No other significant differences in terms of prevalence of risk factors, disease activity, medical treatment or autoimmune profile were found. Significantly higher levels of VCAM, ICAM and E-selectin (p<0.001, p<0.001 and p<0.05, respectively) were found in patients with IIM compared to controls. However, there was no significant difference in the levels of any of the investigated adhesion molecules between IIM patients with TE and those without. Moreover, no significant difference in the levels of adhesion molecules at the time of IIM diagnosis and at the time of TE was observed.ConclusionsThe main finding of this study is the striking prevalence of thrombotic events in patients with IIM. The fact that 1 out of 5 patients suffered from a TE should alert the clinician to screen for TE when clinically indicated as well as to consider prophylactic treatment in risky situations, including surgery, immobilisation or pregnancy. Male gender, higher age at IIM diagnosis and hypertension should be taken into account when estimating TE risk. High disease activity or aPL positivity were not specific features of IIM patients with TE, nor were elevated serum levels of adhesion molecules. The mechanisms leading to increased risk of TE in patients with IIM need further investigations.ReferencesDa...
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