Bone Mineral Density in Patients with Ankylosing Spondylitis: Incidence and Correlation with Demographic and Clinical Variables

Objective: To evaluate bone mineral density (BMD) in patients with ankylosing spondylitis (AS) and determine its correlation with the demographic and clinical characteristics of AS. Patients and Methods: Demographic, clinical and osteodensitometric data were evaluated in a cross-sectional study that included 136 patients with AS. Spine and hip BMD were measured by means of dual energy X-ray absorptiometry (DXA). Using the modified Schober’s test we assessed spine mobility. We examined the sacroiliac, anteroposterior and lateral dorso-lumbar spine radiographs in order to grade sacroiliitis and assess syndesmophytes. Disease activity was evaluated using C-reactive protein (CRP) levels and erythrocyte sedimentation rate (ESR). Demographic data and BMD measurements were compared with those of 167 age- and sex-matched healthy controls. Results: Patients with AS had a significantly lower BMD at the spine, femoral neck, trochanter and total hip as compared to age-matched controls (all p<0.01). According to the WHO classification, osteoporosis was present in 20.6% of the AS patients at the lumbar spine and in 14.6% at the femoral neck. There were no significant differences in BMD when comparing men and women with AS, except for trochanter BMD that was lower in female patients. No correlations were found between disease activity markers (ESR, CRP) and BMD. Femoral neck BMD was correlated with disease duration, Schober’s test and sacroiliitis grade. Conclusion: Patients with AS have a lower spine and hip BMD as compared to age- and sex-matched controls. Bone loss at the femoral neck is associated with disease duration and more severe AS

Mixed connective tissue disease : state of the art on clinical practice guidelines

© Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.Mixed connective tissue disease (MCTD) is a complex overlap disease with features of different autoimmune connective tissue diseases (CTDs) namely systemic sclerosis, poly/dermatomyositis and systemic lupus erythematous in patients with antibodies targeting the U1 small nuclear ribonucleoprotein particle. In this narrative review, we summarise the results of a systematic literature research which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. Since no specific CPGs on MCTD were found, other CPGs developed for other CTDs were taken into consideration in order to discuss what can be applied to MCTD even if designed for other diseases. Three major objectives were proposed for the future development of CPGs: MCTD diagnosis (diagnostic criteria), MCTD initial and follow-up evaluations, MCTD treatment. Early diagnosis, epidemiological data, assessment of burden of disease and QOL aspects are among the unmet needs identified by patients.This publication was funded by the European Union’s Health Programme (2014-2020)info:eu-repo/semantics/publishedVersio

Effectiveness and safety of tocilizumab in patients with systemic sclerosis : a propensity score matched controlled observational study of the EUSTAR cohort

Objectives Tocilizumab showed trends for improving skin fibrosis and prevented progression of lung fibrosis in systemic sclerosis (SSc) in randomised controlled clinical trials. We aimed to assess safety and effectiveness of tocilizumab in a real-life setting using the European Scleroderma Trial and Research (EUSTAR) database. Methods Patients with SSc fulfilling the American College of Rheumatology (ACR)/EULAR 2013 classification criteria, with baseline and follow-up visits at 12±3 months, receiving tocilizumab or standard of care as the control group, were selected. Propensity score matching was applied. Primary endpoints were the modified Rodnan skin score (mRSS) and FVC at 12±3 months compared between the groups. Secondary endpoints were the percentage of progressive/regressive patients for skin and lung at 12±3 months. Results Ninety-three patients with SSc treated with tocilizumab and 3180 patients with SSc with standard of care fulfilled the inclusion criteria. Comparison between groups did not show significant differences, but favoured tocilizumab across all predefined primary and secondary endpoints: mRSS was lower in the tocilizumab group (difference -1.0, 95% CI -3.7 to 1.8, p=0.48). Similarly, FVC % predicted was higher in the tocilizumab group (difference 1.5 (-6.1 to 9.1), p=0.70). The percentage of progressive/regressive patients favoured tocilizumab over controls. These results were robust regarding the sensitivity analyses. Safety analysis confirmed previously reported adverse event profiles. Conclusion Although this large, observational, controlled, real-life EUSTAR study did not show significant effectiveness of tocilizumab on skin and lung fibrosis, the consistency of direction of all predefined endpoints generates hypothesis for potential effectiveness in a broader SSc population

Racial differences in systemic sclerosis disease presentation: a European Scleroderma Trials and Research group study

Objectives. Racial factors play a significant role in SSc. We evaluated differences in SSc presentations between white patients (WP), Asian patients (AP) and black patients (BP) and analysed the effects of geographical locations.Methods. SSc characteristics of patients from the EUSTAR cohort were cross-sectionally compared across racial groups using survival and multiple logistic regression analyses.Results. The study included 9162 WP, 341 AP and 181 BP. AP developed the first non-RP feature faster than WP but slower than BP. AP were less frequently anti-centromere (ACA; odds ratio (OR) = 0.4, P &lt; 0.001) and more frequently anti-topoisomerase-I autoantibodies (ATA) positive (OR = 1.2, P = 0.068), while BP were less likely to be ACA and ATA positive than were WP [OR(ACA) = 0.3, P &lt; 0.001; OR(ATA) = 0.5, P = 0.020]. AP had less often (OR = 0.7, P = 0.06) and BP more often (OR = 2.7, P &lt; 0.001) diffuse skin involvement than had WP.AP and BP were more likely to have pulmonary hypertension [OR(AP) = 2.6, P &lt; 0.001; OR(BP) = 2.7, P = 0.03 vs WP] and a reduced forced vital capacity [OR(AP) = 2.5, P &lt; 0.001; OR(BP) = 2.4, P &lt; 0.004] than were WP. AP more often had an impaired diffusing capacity of the lung than had BP and WP [OR(AP vs BP) = 1.9, P = 0.038; OR(AP vs WP) = 2.4, P &lt; 0.001]. After RP onset, AP and BP had a higher hazard to die than had WP [hazard ratio (HR) (AP) = 1.6, P = 0.011; HR(BP) = 2.1, P &lt; 0.001].Conclusion. Compared with WP, and mostly independent of geographical location, AP have a faster and earlier disease onset with high prevalences of ATA, pulmonary hypertension and forced vital capacity impairment and higher mortality. BP had the fastest disease onset, a high prevalence of diffuse skin involvement and nominally the highest mortality

SST Anywhere&mdash;A Portable Solution for Wide Field Low Earth Orbit Surveillance

The low-Earth orbit (LEO) is filled with active satellites, but also with space debris, which need constant observation. The orbiting objects may be affected by collisions or by atmospheric drag, and therefore they can change their orbit or even fall to the ground, a process known as reentry. The low altitude of these objects (below 2000 km, usually even below 1000 km) means that at given time they can be observed from a limited range of locations on the Earths&rsquo; surface, and therefore having multiple, easy to set up observation stations can be extremely useful. This paper presents a portable hardware solution for on-demand wide-field surveillance of the LEO region, the image processing algorithms for detecting the satellite streaks and for joining these streaks into tracklets, and the solution for astrometrical reduction and generating the result file for each tracklet. An automatic validation solution that is able to automatically identify the detected satellites and compute the measurement angular errors is also presented. The acquisition and processing system is built with commercially available items of low and moderate costs and is capable of on-site acquisition and real-time processing of images. The acquired images are processed by background subtraction, analysis of the difference between frames, extraction of elongated objects corresponding to the satellite streaks, and forming trajectories (tracklets) from consecutive detections. The pixel coordinates of the tracklets are converted to angular coordinates using the tools from Astrometry.net, subsequently filtered for improving the accuracy. The results are validated by using daily updated orbital parameters (TLEs), which are used to predict the angular positions that are subsequently matched with the detection results

SST Anywhere—A Portable Solution for Wide Field Low Earth Orbit Surveillance

The low-Earth orbit (LEO) is filled with active satellites, but also with space debris, which need constant observation. The orbiting objects may be affected by collisions or by atmospheric drag, and therefore they can change their orbit or even fall to the ground, a process known as reentry. The low altitude of these objects (below 2000 km, usually even below 1000 km) means that at given time they can be observed from a limited range of locations on the Earths’ surface, and therefore having multiple, easy to set up observation stations can be extremely useful. This paper presents a portable hardware solution for on-demand wide-field surveillance of the LEO region, the image processing algorithms for detecting the satellite streaks and for joining these streaks into tracklets, and the solution for astrometrical reduction and generating the result file for each tracklet. An automatic validation solution that is able to automatically identify the detected satellites and compute the measurement angular errors is also presented. The acquisition and processing system is built with commercially available items of low and moderate costs and is capable of on-site acquisition and real-time processing of images. The acquired images are processed by background subtraction, analysis of the difference between frames, extraction of elongated objects corresponding to the satellite streaks, and forming trajectories (tracklets) from consecutive detections. The pixel coordinates of the tracklets are converted to angular coordinates using the tools from Astrometry.net, subsequently filtered for improving the accuracy. The results are validated by using daily updated orbital parameters (TLEs), which are used to predict the angular positions that are subsequently matched with the detection results

THE PREVALENCE AND FEATURES OF ULTRASOUND PULMONARY ARTERIAL HYPERTENSION IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

Background. Connective tissue diseases (CTDs) are an important cause of pulmonary arterial hypertension (PAH), which leads to worsening of prognosis especially in patients with systemic lupus erythematosus (SLE) and systemic sclerosis. However, studies on the prevalence of PAH in SLE scarce; our aim is to assess the prevalence and characteristics of PAH in a series of SLE inpatients of a tertiary Romanian SLE Center. Methods. The study included 54 consecutive SLE patients with a regular follow-up at the Department of Rheumatology Cluj-Napoca. The patients underwent physical examination and transthoracic echocardiography to evaluate systolic pulmonary arterial pressure (sPAP), left ventricular performance, and the presence of valvular heart disease. Patient characteristic, cumulative organ damage and laboratory were retrieved by medical chart review. Results. Within the cohort (mean age 43.7 ± 12.4 years, 90.8% women, median duration of disease 7 years), 11 (20.3%) patients were diagnosed with PAH, the majority of which (63.6%) were categorized as mild. The mean sPAP value was 45.54 mmHg and was associated with a history thromboembolic events (p=0.0067), antiphospholipid antibodies (aPL) (p=0.039), and cumulative organ damage (p=0.001). No significant associations with disease duration, Raynaud’s phenomenon, pericardial effusion or SLE-associated autoantibodies were found. Left ventricular diastolic dysfunction (LVDD) occurred more frequent in patients with PAH (p=0.008). Conclusion. Patients with SLE have an increased prevalence of PAH, which is generally asymptomatic and of low severity. PAH is associated with cumulative organ damage, LVDD, and antiphospholipid syndrome (likely reflecting secondary PAH to pulmonary embolism), but not with the disease duration and the Raynaud’s phenomenon or SLE specific autoantibodies

Mixed connective tissue disease: state of the art on clinical practice guidelines

Mixed connective tissue disease (MCTD) is a complex overlap disease with features of different autoimmune connective tissue diseases (CTDs) namely systemic sclerosis, poly/dermatomyositis and systemic lupus erythematous in patients with antibodies targeting the U1 small nuclear ribonucleoprotein particle. In this narrative review, we summarise the results of a systematic literature research which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. Since no specific CPGs on MCTD were found, other CPGs developed for other CTDs were taken into consideration in order to discuss what can be applied to MCTD even if designed for other diseases. Three major objectives were proposed for the future development of CPGs: MCTD diagnosis (diagnostic criteria), MCTD initial and follow-up evaluations, MCTD treatment. Early diagnosis, epidemiological data, assessment of burden of disease and QOL aspects are among the unmet needs identified by patients