Haemorheological changes in patients with systemic lupus erythematosus do not seem to be related to thrombotic events.

Atherothrombotic events are significant factors of mortality and morbidity in patients with systemic lupus erythematosus (SLE). The extent that rheological factors may be involved in these events in these patients has not been established. We measured the following rheological parameters in 86 patients with SLE, of whom 16 had suffered venous and/or arterial thrombotic events, and in 86 healthy controls: fibrinogen (Fbg), plasma viscosity (PV), blood viscosity at 230 s(-1) both at native haematocrit (BVn 230 s(-1) and corrected to 45% (BVc 230 s(-1), erythrocyte aggregation at stasis (AE0) and at 3 s(-1) (AE1), aggregation time (Ta), aggregation index at 10 s (AI10), disaggregation threshold (gammaD), and erythrocyte deformability (ED). In addition glucose, total cholesterol (T-Chol), triglycerides (TG), haematocrit (Hct) and Body Mass Index (BMI) were determined. SLE Disease Activity Index (SLEDAI) was also assessed. The patients showed a significant increase in BMI (P=0.030), TG (P<0.001), PV (P=0.007), AE0 (P=0.005), AE1 (P=0.006), AI10 (P=0.024), gammaD (P=0.001), Fbg (P=0.050); and a significant decrease in Ta (P<0.001), Hct (P<0.001) and BVn 230 s(-1) (P=0.003). When patients with SLEDAI10 were compared, the latter had lower Hct (P=0.041) and lower BVn 230 s(-1) (P=0.017) than those with less SLEDAI. No significant differences were found in any of the parameters analysed on comparing patients who had suffered a thrombotic event with those who had not. Our results suggest that, although patients with SLE have moderate rheological changes, these do not seem to be responsible for the increase in the thrombotic tendency in these patients.

Thrombotic events in systemic lupus erythematosus. Its association with acquired and inherited thrombophilic defects.

Systemic lupus erythematosus (SLE) is characterised by increased venous and arterial thrombotic risk. Although antiphospholipid antibodies (APAs) have been shown to be related with thrombotic tendency in these patients, in more than 40% of them, thrombosis occurs without the presence of such antibodies. We analysed the association of venous and arterial thrombotic events with acquired (anticardiolipin antibodies (ACAs) and lupus anticoagulant (LA)) and inherited (antithrombin (AT), protein C (PC), protein S (PS) deficiencies, factor V Leiden and the prothrombin G20210A mutation), thrombophilic risk factors in 86 SLE patients and 89 healthy controls. Patients showed a higher significant percentage of ACAs titres IgG>41 GPL u/ml and LA than controls (P=0.009; P<0.001, respectively), although no differences in AT, PC, PS deficiencies, factor V Leiden and prothrombin G20210A mutation was observed (P>0.05). When patients with and without thrombosis were compared, those with thrombosis showed a statistically higher percentage of ACAs IgG>41 GPL u/ml and LA (P=0.048; P=0.001, respectively), OR 4.33; 95% CI 1.01-18.50 and OR 11.57; 95% CI 3.28-40.75, respectively. When venous and arterial thrombotic events were considered separately, the presence of LA constituted a risk factor for arterial thrombosis (P=0.010), OR 11.33; 95% CI 1.86-68.89, as well as for venous thrombosis (P=0.005), OR 10.15; 95% CI 2.12-48.64, while ACAs IgG>41 GPL u/ml on their own, were not associated with arterial or venous thrombosis (P=0.142, P=0.233, respectively). In addition inherited thrombophilic risk factors AT, PC, PS deficiencies, factor V Leiden and PT G20210A mutation do not seem to increase thrombotic risk in SLE patients.

Plasma viscosity and related cardiovascular risk factors in a Spanish Mediterranean population.

INTRODUCTION: Plasma viscosity (PV) constitutes an independent important predictor of initial and recurrent cardiac events and mortality. It has been suggested that there is a geographical variation in PV values related to coronary event rates. Little information exists regarding PV in Spain. Therefore, our objective was to determine PV in a large sample of randomly selected subjects from the Spanish population and to study which demographic or cardiovascular risk factors (CVRF) influence levels of PV in this population. MATERIALS AND METHODS: 1277 subjects (503 males, 774 females) aged 43+/-14 years (range: 20-70) were randomly selected from an Eastern Spanish population. These subjects were free of cardiovascular diseases and other major diseases. PV was measured at 37 degrees C by means of the Fresenius GmbH plasma viscosimeter. In addition, total cholesterol, triglycerides, glucose and fibrinogen were measured. RESULTS: In the crude analysis, no differences in PV were observed regarding gender (males: 1.235+/-0.061 cP; females: 1.236+/-0.059 cP, P=0.952). Women older than 50 years showed higher PV:1.248+/-0.057 cP than those aged less than 50 years: 1.232+/-0.059 cP, P=0.001. No differences in PV by age groups were observed in men (P=0.842). Furthermore, we evaluated the prevalence of the following CVRF: hypercholesterolemia, hypertriglyceridemia, hypertension, diabetes, tobacco smoking, obesity, and high fibrinogen levels. Prevalence of these factors was: 28%, 4.3%, 8%, 2.3%, 33%, 8.8% and 15% respectively. As some of these CVRF were correlated with PV levels, we carried out a multivariate analysis to adjust PV levels for the potential confounding effect of each one of these factors. After multivariate adjustment, PV was positively associated with high fibrinogen (>320 mg/dL) levels in both men and women (P<0.001). In addition, in women, but not in men, PV levels were independently associated with obesity (P<0.001) and hypertriglyceridemia (P=0.01). After the multivariate adjustment, the association between PV and age older than 50 in women did not remain statistically significant, revealing a confusing effect of the CVRF in the crude analysis.

Erythrocyte deformability does not seem to be altered in Behçet's disease.

Behçet's disease (BD) is a chronic systemic vasculitis characterized by recurrent oral and genital ulcers, uveitis, and skin lesions. It is also associated with an increased risk of developing thrombosis, although the prothrombotic mechanisms are not clearly defined. The role played by rheological alterations in the development of thrombotic events in BD is not well defined, existing little information on whether erythrocyte deformability (ED) may be involved in this issue. Therefore we aimed to evaluate ED by ektacytometric techniques in a large group of patients with BD in a non-active phase of the disease at sampling and in a well-matched control group, in order to establish a possible association between alterations in ED and the presence of thrombotic events. The patient group comprised 45 patients with BD (22 male, 23 female aged 42+/-14 years) and the control group comprised 46 healthy volunteers (23 male, 23 female aged 45+/-13 years). Twelve of the 45 patients with BD had a previous documented history of deep vein thrombosis at least six months before entering the study, and the other 33 did not. Erythrocyte elongation indexes (EI) at the three shear stresses tested (EI12, EI30, EI60) were not statistically different between patients and controls (EI12: P=0.453; EI30: P=0.411; EI60: P=0.403). There were no significant differences in these parameters, either, when patients with and without previous thrombotic events were compared (EI12: P=0.272; EI30: P=0.215; EI60: P=0.171). Our results suggest that ED is not compromised in BD and does not seem to be involved in the development of thrombotic events in these patients.

Erythrocyte deformability in anaemic patients with reticulocytosis determined by means of ektacytometry techniques.

It is not clearly established whether reticulocyte deformability is lower than that of the mature erythrocytes, as most of studies published on this matter have evaluated this rheological parameter by means of micropipette techniques, which are unsuitable for routine measurements. Information is scarce as regards the evaluation of reticulocyte deformability by means of ektacytometry techniques, routinely used in clinical laboratories. We aimed to evaluate erythrocyte deformability (ED), with ektacytometry, in samples of 44 anaemic patients with peripheral reticulocytosis (reticulocytes: (260+/-150)x10(3)/microl) and in 60 healthy non-anaemic volunteers with a normal reticulocyte count (reticulocytes: (60+/-20)x10(3)/microl). We also determined other factors that may influence ED, such as erythrocyte indices (MCV, MCH, MCHC), glucose, total cholesterol and triglycerides. ED was evaluated determining the elongation indices (EI) at 12, 30 and 60 Pa, by means of the Rheodyn SSD. At the three shear stresses tested, patients showed statistically lower EI than controls, higher reticulocyte count, lower cholesterol levels and higher MCHC (P<0.001, respectively). A statistically significant negative correlation (P<0.01) was found between the reticulocyte count and the EI at 12, 30 and 60 Pa (r=-0.643, r=-0.678 and r=-0.692, respectively), and between the EI and the MCHC (correlation coefficients: -0.743, -0.741 and -0.738; P<0.01). As the differences in ED could be attributed partly to alterations in erythrocyte indices and plasma lipid levels, a linear regression analysis was performed, showing that EI is independently associated with the reticulocyte count. Our results suggest that reticulocytes are responsible for the decreased ED observed in anaemic patients with peripheral reticulocytosis, when this hemorheological parameter is evaluated by means of ektacytometry techniques.

Thrombophilic risk factors and homocysteine levels in Behçet's disease in eastern Spain and their association with thrombotic events.

Behçet's disease (BD) is a chronic inflammatory disorder in which thrombosis occurs in about 30% of patients. The prothrombotic mechanisms are unknown. Thrombophilic defects and hyperhomocysteinaemia may be involved in the pathogenesis of thrombotic events, although results have been controversial. Moreover, no information is available on this issue for eastern Spain. We studied the prevalence of inherited and acquired thrombophilic risk factors in 79 patients with BD (43 men, 36 women) who had (n = 23) or did not have (n = 56) thrombosis, and in 84 healthy control subjects (42 men, 42 women). Risk factors examined were antithrombin, protein C and protein S levels, factor V Leiden, the prothrombin G20210A mutation, the methylenetetrahydrofolate reductase C677T polymorphism, and acquired thrombophilic risk factors, including anticardiolipin antibodies, lupus anticoagulant, and serum homocysteine levels. There were no differences between patients and controls in any of the parameters studied. When we studied BD patients with and without thrombotic events, the only thrombophilic defect that differed was the prothrombin G20210A mutation: Three out of 23 patients with thrombosis were carriers, compared with none of 56 patients without thrombosis (p = 0.022). Two of the three carriers developed catastrophic or recurrent thrombotic episodes; one was a homozygous carrier of the G20210A prothrombin mutation and the other was doubly heterozygous for the G20210A prothrombin mutation and factor V Leiden. A meta-analysis demonstrated an association of factor V Leiden and prothrombin mutation with thrombosis in BD. When studies from Turkey were excluded from the meta-analysis, only the prothrombin G20210A mutation was associated with thrombosis.

[Behçet disease: study of 74 patients]. / Enfermedad de Behçet: estudio de 74 pacientes.

BACKGROUND AND OBJECTIVE: Behçet disease (BD) has a low prevalence in the Spanish population. Only a few reports have been published on the clinical features of BD in our country. The aim of this study is to determine the type and frequency of these features of BD in a population of patients in the Community of Valencia. PATIENTS AND METHOD: We retrospectively studied clinical data from patients with BD diagnosed between 1990 and 2005 in La Fe, General and Doctor Peset Universitary Hospitals. All Patients fulfilled the International Study Group Criteria for the diagnosis of BD. Statistical analysis was carried out using the chi2 test. RESULTS: Seventy four patients (40 male and 34 female) were studied. The most frequent manifestations were oral (98.5%) and genital aphthae (82.4%), followed by cutaneous lesions (64.2%), ocular lesions (42.5%), fever (39.4%) and vascular manifestations (28.4%). Venous manifestations were more frequent than arterial events. Gastrointestinal lesions occurred more frequently in females compared with males (p = 0.002). Vascular and ocular manifestations were more severe in males than in females. With respect to cardiovascular risk factors, 32.4% of patients were smokers, 20.3% were hyperlipidemic, 19% hypertensive, 13.5% obese and 9.5%diabetic. Cardiovascular risk factors were not related to thrombotic events or posterior uveitis in these patients (p > 0.05). CONCLUSIONS: BD in patients in the community of Valencia is characterized by a variety of clinical manifestations similar to other geographical areas. Gastrointestinal manifestations occur more frequently in female patients, and venous thrombotic manifestations were more frequent than arterial events. Cardiovascular risk factors do not seem to play a role in the development of thrombotic events and posterior uveitis in these patients.

Red blood cell deformability in iron deficiency anaemia.

In patients with iron deficiency anaemia (IDA) it has been suggested that the shortened erythrocyte lifespan may be in part due to decreased erythrocyte deformability. In order to know whether erythrocyte deformability is decreased in IDA patients, we have determined the erythrocyte Elongation Index (EI) by means of ektacytometric techniques (Rheodyn SSD, Myrenne Gmbh, Germany), in 50 IDA patients and 100 well age and sex matched healthy controls. At the three shear stresses tested, 12, 30 and 60 Pa, IDA patients show statistically lower EI than controls (37.4+/-6.7 vs 48.6+/-2.9; 45.0+/-6.0 vs 54.5+/-2.8; 48.7+/-5.8 vs 57.0+/-2.9 mPa.s, respectively; p<0.001). A statistically significant correlation was found between EI at 12, 30, and 60 Pa and the hematimetric indices (MCV, MCH and MCHC), suggesting that the alteration in surface/volume ratio (shape) which characterizes this kind of microcytic hypocromic anaemia, accounts in part for the decreased EI. Rheodyn SSD, as an ektacytometric technique, is very sensitive to alterations in red blood cell geometry, for what seems to be a useful tool for detecting diminished erythrocyte deformability in IDA patients.

Influence of fibrinogen levels on erythrocyte aggregation determined with the Myrenne aggregometer and the Sefam erythro-aggregometer.

Fibrinogen is one of the plasmatic proteins which has a major influence on erythrocyte aggregation. The level of fibrinogen at which erythrocyte aggregation does not further increase is not well established. Therefore we aim to determine erythrocyte aggregation with two devices: Myrenne aggregometer (M0 and M1) and Sefam erythro-aggregometer (Ta, AI10 and gammaD) in relation with fibrinogen levels, in patients with several diseases with fibrinogen levels ranging between 200-1100 mg/dl. With the Myrenne aggregometer a plateau can be observed for fibrinogen levels higher than 400 mg/dl, while with the Sefam erythro-aggregometer, aggregation increases proportionally with fibrinogen levels higher than 400 mg/dl. In addition, for fibrinogen values higher than 400 mg/dl, only statististically significant correlations could be observed between fibrinogen and erythrocyte aggregation parameters with the Sefam erythro-aggregometer: r(Ta)=-0.463; r(AI10)=0.624, r(gammaD)=0.817, p<0.01, but not with the Myrenne: r(M0)=0.01, r(M1)=0.02, ns. Although the Sefam erythro-aggregometer is a better tool for determining erythrocyte aggregation both at low and high fibrinogen levels, the Myrenne aggregometer may be useful for assessing the erythrocyte aggregation as an indicator of cardiovascular risk, as in such circumstances fibrinogen levels do not usually exceed values higher than 400 mg/dl.

Enfermedad de Behçet: estudio de 74 pacientes / Behçet disease: study of 74 patients

Fundamento y objetivo: La enfermedad de Behçet (EB) es una entidad clínica poco prevalente en España. Son escasos los artículos publicados sobre datos epidemiológicos y manifestaciones clínicas en nuestro país. El objetivo del presente estudio ha sido conocer las características de las manifestaciones clínicas de la EB en la Comunidad Valenciana. Pacientes y método: Se recogieron datos de los pacientes diagnosticados entre 1990 y 2005 de EB en los Hospitales Universitarios La Fe, General y Doctor Peset de Valencia. Todos los pacientes cumplían los criterios diagnósticos del Grupo de Estudio Internacional para el diagnóstico de la EB. Las diferencias entre sexos se analizaron mediante el test de la *2. Resultados: Formaron el grupo de estudio 74 pacientes (40 varones y 34 mujeres). Las manifestaciones clínicas más frecuentes fueron las aftas orales (98,5%) y genitales (82,4%), seguidas de las cutáneas (64,2%), oculares (42,5%), fiebre (39,4%) y vasculares (28,4%), con predominio de las trombosis venosas sobre las arteriales. Sólo las manifestaciones gastrointestinales fueron más frecuentes en el sexo femenino (p = 0,002). Las alteraciones vasculares y oculares fueron más graves en los varones. En cuanto a la prevalencia de los factores de riesgo cardiovascular, el 32,4% de los pacientes eran fumadores, un 20,3% presentaba hiperlipemia; un 19%, hipertensión; un 13,5%, obesidad, y un 9,5%, diabetes, aunque no se observó asociación entre éstos y los episodios trombóticos ni la uveítis posterior (p > 0,05). Conclusiones: Los resultados obtenidos fueron similares a los de otras áreas geográficas. Destacan la mayor frecuencia de manifestaciones digestivas en mujeres y el predominio de los episodios trombóticos venosos sobre los arteriales. Los factores de riesgo cardiovascular no parecen desempeñar un papel en el desarrollo de episodios trombóticos ni uveítis posterior en estos pacientes

Background and objective: Behçet disease (BD) has a low prevalence in the Spanish population. Only a few reports have been published on the clinical features of BD in our country. The aim of this study was to determine the type and frequency of these features of BD in a population of patients in the Community of Valencia. Patients and method: We retrospectively studied clinical data from patients with BD diagnosed between 1990 and 2005 in La Fe, General and Doctor Peset Universitary Hospitals. All patients fulfilled the International Study Group criteria for the diagnosis of BD. Statistical analysis was carried out using the *2 test. Results: Seventy four patients (40 male and 34 female) were studied. The most frequent manifestations were oral (98.5%) and genital aphthae (82.4%), followed by cutaneous lesions (64.2%), ocular lesions (42.5%), fever (39.4%) and vascular manifestations (28.4%). Venous manifestations were more frequent than arterial events. Gastrointestinal lesions occurred more frequently in females compared with males (p = 0.002). Vascular and ocular manifestations were more severe in males than in females. With respect to cardiovascular risk factors, 32.4% of patients were smokers, 20.3% were hyperlipidemic, 19% hypertensive, 13.5% obese and 9.5% diabetic. Cardiovascular risk factors were not related to thrombotic events or posterior uveitis in these patients (p > 0.05). Conclusions: BD in patients in the Community of Valencia is characterized by a variety of clinical manifestations similar to other geographical areas. Gastrointestinal manifestations occur more frequently in female patients, and venous thrombotic manifestations were more frequent than arterial events. Cardiovascular risk factors do not seem to play a role in the development of thrombotic events and posterior uveitis in these patients