Concordance of gout management with European League against Rheumatism recommendations in hospital practice

Aim: To assess the concordance of gout management with the European League Against Rheumatism (EULAR) gout recommendations in hospital practice. Methods: This was a retrospective review of case notes of patients presenting to rheumatology outpatients between June and December 2009 under the care of 2 consultant rheumatologists. Data collected consisted of demographics, time lag to specialist referral, comorbidities, details about acute and recurrent attacks, lifestyle advice and use of urate lowering therapies. Documented management was assessed for concordance with the EULAR recommendations. Results: Thirty consecutive patients (27 males, 3 females) attending Rheumatology clinic at Mater Dei Hospital were recruited. Mean age at the time of survey was 59.4 ± 10.7 years, while mean age at diagnosis was 51.1 ± 14 years. Documentation of lifestyle advice was recorded for alcohol reduction (83%), weight loss (43%), diet (13%), and exercise (13%). Adequate control of comorbidities was attained in hyperlipidaemia (71%), diabetes mellitus (55%) and hypertension (30%). Advice about smoking cessation was given to 37%. Uric acid levels below target were achieved in 47%. The mean uric acid level at time of survey was 379 ± 146 μmol/l. This was significantly less than that at presentation (p=0.001). Conclusions: Current treatment of gout is poorly concordant with many of the EULAR recommendations. Documentation of lifestyle modifications advice is infrequent except for alcohol reduction. A significant number of patents on allopurinol still have hyperuricaemia implying that more aggressive management is required to improve standard of care. A proforma has been developed to make and help sustain the necessary improvements.peer-reviewe

Audit on follow-up of patients with primary Osteoporosis

Aim: To document the frequency of Dual-energy X- ray absorptiometry (DEXA) scanning and Rheumatology clinic follow-up visits of patients with primary osteoporosis, and compare these with recommended guidelines. Method: Medical notes of all primary osteoporotic patients attending a hospital Rheumatology clinic were reviewed over a period of four months. Data was collected on age, gender, frequency of follow up visits, frequency of DEXA scanning, osteoporosis treatment, any changes in such treatment during the last visit, and comparison of the last two DEXA scan results. Frequency of follow up DEXA scans was compared to Group Health Osteoporosis Screening Diagnosis and Treatment guidelines.1 Results: Eighty-two patients were included, 6 males (7.3%) and 76 females (92.7%). The age range was 35-87 years (mean age was 68.6 years). In total, 42.7% of all the patients were on combined calcium and vitamin D, with added Bisphosphonates, Strontium ranelate, or Denosumab. During their last clinic visit, 61% showed improvement in T score since their previous result, and 64.6% of patients had no change in treatment. In this audit, 29.3% were being followed up on a 13 monthly basis, and 72% had annual bone mineral density scans or more frequently Conclusion: According to the guidelines, none of the patients included in this audit should have had a repeat DEXA scan within less than two years. Patients attending the clinic have too frequent DEXA scans and therefore, too frequent follow up appointments.peer-reviewe

A large pericardial effusion and bilateral pleural effusions as the initial manifestations of Familial Mediterranean Fever

Familial Mediterranean Fever (FMF) is a condition characterized by recurrent febrile poly-serositis. Typical presentations of the disease include episodes of fever, abdominal pain and joint pains. Chest pain is a less common presentation. We report a case of FMF which presented with a large pericardial effusion and bilateral pleural effusions in a lady who had no positive family history and negative genetic testing.peer-reviewe

Evaluation of the impact of a pharmaceutical care service offered to rheumatoid arthritis patients within an ambulatory setting

The objectives of the study were to evaluate the impact of a newly developed pharmaceutical care services directed to rheumatoid arthritis patients attending an out-patient setting. A total of 88 patients participated in the study and were randomly divided into two equal groups, Group A and Group B. The study was carried out over three phases. In phase 1 (time 0), Group A patients were assessed and offered a pharmaceutical care session. Group B patients were assessed but no pharmaceutical care session was delivered. At phase 2 (4-6 months), group A patients were re-assessed (first assessment post pharmaceutical care plan). Group B patients were re-assessed a second time (second baseline assessment) and a pharmaceutical care session was offered to Group B patients. At phase 3 (time 10-11 months) both groups were re-assessed a third time. The Health Assessment Questionnaire and the Short Form-36 were used as outcome measures during each assessment. There were statistically significant differences (p<0.05) in all the 8 domains of the SF-36 between Phase 1 and 3 for both groups. For the Health Assessment Questionnaire, a statistical improvement in the daily activities was identified after the pharmaceutical care intervention for both groups (Phase 2 for Group A and phase 3 for Group B). The newly developed individualised pharmaceutical care service provided by the pharmacist led to an improved quality of life as measured by the health related quality of life questionnaires.peer-reviewe

Cost effectiveness of TNF- inhibitors in rheumatoid arthritis

Background. TNF- inhibitors have shown to be effective in reducing disease activity and improving the quality of life. Due to the high costs associated with acquisition of this treatment, this study was undertaken to evaluate the ICER of TNF- antagonists (etanercept, adalimumab, and infliximab) in improving the quality of life. Methods.The HAQ and SF-36 were administered at phases 1, 2, and 3, in order to assess the improvement in the QOL. Suppression of disease activity was assessed through the DAS-28. Results. Statistically significant improvements ( < 0.05) were noted for the SF-36 and HAQ after 3 months and for the DAS-28 after 6 months of TNF- inhibitor therapy.The mean ICER per 10% improvement in the HAQ, DAS-28, and SF-6D were C1976.5, C2086.5, and C2316.4, respectively, following 6 months of TNF- intervention. Most favorable ICERs were reported from a patient who had to undergo surgical intervention whilst on DMARD therapy. Conclusion. Significant improvement was observed in patients’ quality of life, after a short timeframe of 6 months. Such data is useful information in the light of convincing policy makers, in terms of providing access to the medications to individual patients on national health service schemes.peer-reviewe

Health Assessment Questionnaire-Disability Index (HAQ-DI) use in modelling disease progression in diffuse cutaneous systemic sclerosis: an analysis from the EUSTAR database

BACKGROUND: Patients with diffuse cutaneous systemic sclerosis (dcSSc) have a poor prognosis. The importance of monitoring subjective measures of functioning and disability, such as the Health Assessment Questionnaire-Disability Index (HAQ-DI), is important as dcSSc is rated by patients as worse than diabetes or hemodialysis for quality of life impairment. This European Scleroderma Trials and Research (EUSTAR) database analysis was undertaken to examine the importance of impaired functionality in dcSSc prognosis. The primary objectives were to identify predictors of death and HAQ-DI score progression over 1 year. HAQ-DI score, major advanced organ involvement, and death rate were also used to develop a comprehensive model to predict lifetime dcSSc progression. METHODS: This was an observational, longitudinal study in patients with dcSSc registered in EUSTAR. Death and HAQ-DI scores were, respectively, analyzed by Cox regression and linear regression analyses in relation to baseline covariates. A microsimulation Markov model was developed to estimate/predict natural progression of dcSSc over a patient's lifetime. RESULTS: The analysis included dcSSc patients with (N = 690) and without (N = 4132) HAQ-DI score assessments from the EUSTAR database. Baseline HAQ-DI score, corticosteroid treatment, and major advanced organ involvement were predictive of death on multivariable analysis; a 1-point increase in baseline HAQ-DI score multiplied the risk of death by 2.7 (p &lt; &nbsp;0.001) and multiple advanced major organ involvement multiplied the risk of death by 2.8 (p &lt; &nbsp;0.05). Multivariable analysis showed that baseline modified Rodnan Skin Score (mRSS) and baseline HAQ-DI score were associated with HAQ-DI score progression at 1 year (p &lt; &nbsp;0.05), but there was no association between baseline organ involvement and HAQ-DI score progression at 1 year. HAQ-DI score, major advanced organ involvement, and death were successfully used to model long-term disease progression in dcSSc. CONCLUSIONS: HAQ-DI score and major advanced organ involvement were comparable predictors of mortality risk in dcSSc. Baseline mRSS and baseline HAQ-DI score were predictive of HAQ-DI score progression at 1 year, indicating a correlation between these endpoints in monitoring disease progression. It is hoped that this EUSTAR analysis may change physician perception about the importance of the HAQ-DI score in dcSSc

The Athena X-ray Integral Field Unit: a consolidated design for the system requirement review of the preliminary definition phase

The Athena X-ray Integral Unit (X-IFU) is the high resolution X-ray spectrometer, studied since 2015 for flying in the mid-30s on the Athena space X-ray Observatory, a versatile observatory designed to address the Hot and Energetic Universe science theme, selected in November 2013 by the Survey Science Committee. Based on a large format array of Transition Edge Sensors (TES), it aims to provide spatially resolved X-ray spectroscopy, with a spectral resolution of 2.5 eV (up to 7 keV) over an hexagonal field of view of 5 arc minutes (equivalent diameter). The X-IFU entered its System Requirement Review (SRR) in June 2022, at about the same time when ESA called for an overall X-IFU redesign (including the X-IFU cryostat and the cooling chain), due to an unanticipated cost overrun of Athena. In this paper, after illustrating the breakthrough capabilities of the X-IFU, we describe the instrument as presented at its SRR, browsing through all the subsystems and associated requirements. We then show the instrument budgets, with a particular emphasis on the anticipated budgets of some of its key performance parameters. Finally we briefly discuss on the ongoing key technology demonstration activities, the calibration and the activities foreseen in the X-IFU Instrument Science Center, and touch on communication and outreach activities, the consortium organisation, and finally on the life cycle assessment of X-IFU aiming at minimising the environmental footprint, associated with the development of the instrument. Thanks to the studies conducted so far on X-IFU, it is expected that along the design-to-cost exercise requested by ESA, the X-IFU will maintain flagship capabilities in spatially resolved high resolution X-ray spectroscopy, enabling most of the original X-IFU related scientific objectives of the Athena mission to be retained. (abridged).Comment: 48 pages, 29 figures, Accepted for publication in Experimental Astronomy with minor editin

The Athena X-ray Integral Field Unit: a consolidated design for the system requirement review of the preliminary definition phase

The Athena X-ray Integral Unit (X-IFU) is the high resolution X-ray spectrometer studied since 2015 for flying in the mid-30s on the Athena space X-ray Observatory. Athena is a versatile observatory designed to address the Hot and Energetic Universe science theme, as selected in November 2013 by the Survey Science Committee. Based on a large format array of Transition Edge Sensors (TES), X-IFU aims to provide spatially resolved X-ray spectroscopy, with a spectral resolution of 2.5 eV (up to 7 keV) over a hexagonal field of view of 5 arc minutes (equivalent diameter). The X-IFU entered its System Requirement Review (SRR) in June 2022, at about the same time when ESA called for an overall X-IFU redesign (including the X-IFU cryostat and the cooling chain), due to an unanticipated cost overrun of Athena. In this paper, after illustrating the breakthrough capabilities of the X-IFU, we describe the instrument as presented at its SRR (i.e. in the course of its preliminary definition phase, so-called B1), browsing through all the subsystems and associated requirements. We then show the instrument budgets, with a particular emphasis on the anticipated budgets of some of its key performance parameters, such as the instrument efficiency, spectral resolution, energy scale knowledge, count rate capability, non X-ray background and target of opportunity efficiency. Finally, we briefly discuss the ongoing key technology demonstration activities, the calibration and the activities foreseen in the X-IFU Instrument Science Center, touch on communication and outreach activities, the consortium organisation and the life cycle assessment of X-IFU aiming at minimising the environmental footprint, associated with the development of the instrument. Thanks to the studies conducted so far on X-IFU, it is expected that along the design-to-cost exercise requested by ESA, the X-IFU will maintain flagship capabilities in spatially resolved high resolution X-ray spectroscopy, enabling most of the original X-IFU related scientific objectives of the Athena mission to be retained. The X-IFU will be provided by an international consortium led by France, The Netherlands and Italy, with ESA member state contributions from Belgium, Czech Republic, Finland, Germany, Poland, Spain, Switzerland, with additional contributions from the United States and Japan.The French contribution to X-IFU is funded by CNES, CNRS and CEA. This work has been also supported by ASI (Italian Space Agency) through the Contract 2019-27-HH.0, and by the ESA (European Space Agency) Core Technology Program (CTP) Contract No. 4000114932/15/NL/BW and the AREMBES - ESA CTP No.4000116655/16/NL/BW. This publication is part of grant RTI2018-096686-B-C21 funded by MCIN/AEI/10.13039/501100011033 and by “ERDF A way of making Europe”. This publication is part of grant RTI2018-096686-B-C21 and PID2020-115325GB-C31 funded by MCIN/AEI/10.13039/501100011033

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