Arterial and venous involvement in Behçet’s syndrome: a narrative review

Behçet syndrome (BS) is a unique type of vasculitis that affects veins and arteries of all sizes, leading to recurrent vascular events, mostly venous thrombosis. The prevalence of venous thromboembolism in BS patients ranges between 15 and 40%. Thrombosis is usually an early manifestation leading to diagnosis of BS in up to 40% of patients. BS is per se a model of inflammation-induced thrombosis. The primary autoimmune response activates lymphocytes that in turn produce a cytokine cascade that activates neutrophils, which modify the secondary structure of fibrinogen making it less susceptible to plasmin-induced lysis. This leads to endothelial dysfunction, platelet activation and overexpression of tissue factor leading to inflammatory thrombi, usually attached to the wall. The pathogenesis of thrombosis is especially relevant to direct the specific treatment, that is based on immunosuppression rather than anticoagulation. Superficial vein thrombosis (SVT) and deep vein thrombosis (DVT) are the most common form of thrombosis in BS, but thrombosis in atypical sites (cava vein, suprahepatic veins, intracardiac thrombus) and arterial involvement can also occur. We assessed the latest update of the European League Against Rheumatism recommendations for the management of BS. Vascular Behçet treatment is usually based of immunosuppressants, and the role of anticoagulation remains controversial. The use of interventional and surgical procedures should be carefully evaluated, due to the risk of triggering a vascular pathergy phenomenon.

     

Correlation between the Measurement of Transverse Diameter in the Proximal Neck on Computed Tomography and on Aortography before Endovascular Treatment of Infrarenal Aortic Aneurysm

The aim of this study was to determine the correlation between the measurement of transverse diameter of the proximal neck on computed tomographic angiography (CTA) and graduated catheter aortography in patients who are candidates for endovascular graft placement in order to replace, if both measurements are equivalent, aortography for CTA alone. Preoperative dual-slice CTA and graduated catheter aortography were performed in 35 consecutive patients with infrarenal aortic aneurysm within 10 days. Transverse proximal neck diameters were measured on a true axial section on CTA reconstructions and on aortographic images, always 6 mm distal from the most inferior main renal artery. Mean, median, and standard deviation were obtained and the measurements correlated for each patient using Pearson’s correlation and linear regression analysis. A significant difference in proximal neck transverse diameter measurements was found between graduated catheter aortography and CTA in all cases. CTA values were a mean of 1.74 mm higher than aortography values. Pearson’s correlation indicates a strong correlation between both techniques, and a regression equation determines the predictive value of aortography on the basis of CTA values. Estimation of the transverse diameter of the proximal neck on aortography on the basis of that obtained on CTA allows us to affirm that CTA could be used as the sole method for the preoperative selection of appropriate endograft size in patients with infrarenal aortic aneurysm.     

Two novel mutations in the SLC40A1 and HFE genes implicated in iron overload in a Spanish man

The most common form of hemochromatosis is caused by mutations in the HFE gene. Rare forms of the disease are caused by mutations in other genes. We present a patient with hyperferritinemia and iron overload, and facial flushing. Magnetic resonance imaging was performed to measure hepatic iron overload, and a molecular study of the genes involved in iron metabolism was undertaken. The iron overload was similar to that observed in HFE hemochromatosis, and the patient was double heterozygous for two novel mutations, c.-20G>A and c.718A>G (p.K240E), in the HFE and ferroportin (FPN1 or SLC40A1) genes, respectively. Hyperferritinemia and facial flushing improved after phlebotomy. Two of the patient's children were also studied, and the daughter was heterozygous for the mutation in the SLC40A1 gene, although she did not have hyperferritinemia. The patient presented a mild iron overload phenotype probably because of the two novel mutations in the HFE and SLC40A1 genes.     

Monitoring anti-Xa levels in patients with cancer-associated venous thromboembolism treated with bemiparin

To analyze the relationship between therapeutic (weight-adjusted) dose of bemiparin and anti-Xa activity in patients with venous thromboembolism (VTE) and