Notes

Automatic revolutionary nephroureterectomy in a affected individual using situs inversus totalis.
70); advanced treatment line was also associated with greater risk for failure (HR 1.64). In a univariable analysis stratified by the treatment line, tofacitinib had similar or better drug survival than other agents in the first and second lines. In the third line and beyond, tocilizumab had a higher EFS compared to tofacitinib (HR 0.57).

Drug survival with tofacitinib is related to treatment line. Early introduction is associated with similar or better survival than other agents, whereas tocilizumab was superior in the third line or later.
Drug survival with tofacitinib is related to treatment line. Early introduction is associated with similar or better survival than other agents, whereas tocilizumab was superior in the third line or later.
To investigate the diagnostic performance of dual-energy computed tomography (DECT) in detection bone marrow oedema (BME) in patients with sacroiliitis associated with axial spondyloarthritis (axial SpA).

Patients with axial SpA according to the ASAS criteria underwent DECT and 1.5-T magnetic resonance imaging (MRI). DECT was post-processed for generating virtual non-calcium (VNCa) images. The presence of abnormal bone marrow attenuation was scored on DECT VNCa images and MRI using a four-point classification system 0-1 = absent or non-significant oedema, 2 = oedema present in a third of the articular surface, 3 = oedema present in 2/3 of the articular surface, 4 = diffuse oedema throughout the articular surface. Diagnostic accuracy values for BME were calculated for DECT images (quantitative assessment) by using receiver operating characteristic (ROC) curves analysis, applying MRI as gold standard.

Eighty sacroiliac joints from 40 axial SpA patients were included for study analysis, and 36 sacroiliac joints (45%) were classified as having BME at MRI and compared to DECT. Sensitivity, specificity, and positive likelihood ratio (LR+) in the identification of BME at DECT were 90.0%, 92.8%, and 12.6 respectively. selleckchem Negative LR was 0.11, positive predictive value 93.1%, and negative predictive value 89.7%. The area under the curve (AUC) was 0.953 in the differentiation of the presence of BME. A cut-off value of -1.6 HU (Youden's index = 0.828) yielded a sensitivity of 90.0% and specificity of 92.8%, with an LR+ of 12.6, in the detection of BME in the sacroiliac joints.

DECT VNCa images had good diagnostic performance in the evaluation of the extent of BME in patients with sacroiliitis associated with axial SpA.
DECT VNCa images had good diagnostic performance in the evaluation of the extent of BME in patients with sacroiliitis associated with axial SpA.
To investigate the prognostic significance of concomitant autoimmune diseases (ADs) in myeloproliferative neoplasms (MPNs).

435 subjects with a diagnosis of MPNs were included in this observational single institution longitudinal study. Of them, 34 patients presented an overt AD at diagnosis of MPN. Clinical presenting features, progression-free and overall survival were compared between MPN subgroups in relation to co-existence of AD at diagnosis of MPN.

Compared to cases without ADs, the subjects with ADs were significantly younger, had lower haemoglobin and haematocrit levels and more frequently presented with splenomegaly. The clinical and biological features associated to progression-free and overall survival were age, presence of splenomegaly, histotype (MF vs. PV vs. ET), anaemia, high platelet count and presence of any AD at diagnosis of MPN. The age-adjusted hazard ratio (HR) of progression for the presence of AD at diagnosis of MPN was 2.76. Overall survival was not significantly associated to AD at diagnosis, but the HR of progression for the presence of AD at diagnosis of MPN was 2.18.

A possible common genetic predisposition, the inflammatory bone marrow microenvironment and the activation of theJAK/STAT pathway could be considered as responsible for the observed association between MPNs and ADs.
A possible common genetic predisposition, the inflammatory bone marrow microenvironment and the activation of theJAK/STAT pathway could be considered as responsible for the observed association between MPNs and ADs.
The aim of this study is to explore the link between the severity of the joint and entheses involvement in psoriatic arthritis (PsA) using musculoskeletal ultrasound (US).

PsA patients from two centres in the Psoriatic Arthritis International Database (PsArt-ID) (n=126) underwent an ultrasound assessment of 46 joints and 12 large entheses. The correlation between joint and enthesitis scores on the US was analysed, in addition to the clinical indices versus the US.

Grey-scale (GS) synovitis score for the joints was moderately correlated with the total enthesitis score (r=0.410, p<0.001). The Global Outcome Measure in Rheumatology in Clinical Trials-European League Against Rheumatism Synovitis Score (GLOESS) score was also found in correlation with the total enthesitis score (r=0.400, p<0.001). The link between the US and clinical examination findings only showed a poor correlation between swollen joint counts (SJC) and joint-US scores (r=0.298, p=0.001 for GLOESS). Assessment of the entheses on US showed a poor-moderate correlation between the entheseal damage scores and tender joint counts (TJC) (r=0.217, p=0.018) and SJC (r=0.326, p<0.001). In terms of the clinical examination and activity parameters, none of the clinical parameters and acute phase reactants were correlated to Leeds Enthesitis Index.

Our study showed a link between the severity of the sonographic findings in the joints and the entheses. Imaging using US to assess enthesitis in clinical trials may improve our understanding on the role of enthesitis in disease pathogenesis.
Our study showed a link between the severity of the sonographic findings in the joints and the entheses. Imaging using US to assess enthesitis in clinical trials may improve our understanding on the role of enthesitis in disease pathogenesis.
Predicting response to anti-tumour necrosis factor alpha (anti-TNFα) drugs at baseline remains an elusive goal in rheumatoid arthritis (RA) management. The purpose of this study was to determine if baseline genetic variants of PTPRC, AFF3, myD228, CHUK, MTHFR1, MTHFR2, CD226 and a number of KIR and HLA alleles could predict response to anti-TNF-α in rheumatoid arthritis patients.

Peripheral blood samples were collected from 238 RA patients treated with anti-TNFα drugs. Genotyping was performed using biochip array technology by Randox Laboratories Ltd. and sequence specific polymerase chain reaction. Linear regression analysis was performed to investigate the role of these genotypes in predicting response to treatment, as defined by European League Against Rheumatism (EULAR) response classification and absolute change in disease activity score (DAS28).

Of 238 RA patients analysed, 50.4% received adalimumab, 29.7% received etanercept, 14.8% received infliximab, 3.4% certoluzimab and 1.7% golimumab. The MTHFR1 variant rs1801133 was significantly associated with the EULAR response, p=0.
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