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© Copyright Clinical and Experimental Rheumatology 2022., Objectives: To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods: We conducted a multicentre, international, retrospective cohort study. Results: 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusions: The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Cigarette smoke is the main source of indoor chemical and toxic elements. Cadmium (Cd), Thallium (Tl), Lead (Pb) and Antimony (Sb) are important contributors to smoke-related health risks. Data on the association between Rare Earth Elements (REE) Cerium (Ce) and Lanthanum (La) and domestic smoking are scanty. To evaluate the relationship between cigarette smoke, indoor levels of PM2.5 and heavy metals, 73 children were investigated by parental questionnaire and skin prick tests. The houses of residence of 41 "cases" and 32 "controls" (children with and without respiratory symptoms, respectively) were evaluated by 48-hours PM2.5 indoor/outdoor monitoring. PM2.5 mass concentration was determined by gravimetry; the extracted and mineralized fractions of elements (As, Cd, Ce, La, Mn, Pb, Sb, Sr, Tl) were evaluated by ICP-MS. PM2.5 and Ce, La, Cd, and Tl indoor concentrations were higher in smoker dwellings. When corrected for confounding factors, PM2.5, Ce, La, Cd, and Tl were associated with more likely presence of respiratory symptoms in adolescents. We found that: i) indoor smoking is associated with increased levels of PM2.5, Ce, La, Cd, and Tl and ii) the latter with increased presence of respiratory symptoms in children

EBUS-TBNA at Mater Dei Hospital (MDH), which serves a population of about 432000, was audited over a 2 year period. Our aim was to assess results obtained and factors associated with improved diagnostic yield. All patients who underwent EBUS-TBNA at MDH were included and data was collected retrospectively. 71 EBUS TBNA procedures were performed on 70 patients. Sensitivity for malignancy was 93% (40/43) and sensitivity for granulomatous disease was 72% (16/22). The overall diagnostic sensitivity was 84.5% (60/71). Sampling multiple stations as opposed to a single station was associated with a better histology diagnostic yield (90.3% vs 60% p=0.004) as well as a better yield on cytology (83.9% vs 50% p=0.003). It also led to an improved overall combined diagnostic yield (93.5% vs 77.5%) although this narrowly missed statistical significance (p=0.06). Targeting multiple stations also correlated with obtaining an adequate histology sample (96.8% vs 82.5% p=0.05). This effect was even more pronounced for granulomatous disease with improved diagnostic histology yield (100% vs 43.8% p=0.005) diagnostic cytology yield (89% vs 25% p=0.002) and overall combined diagnostic yield (100% vs 62.5% p=0.035) Increasing lesion size was predictive of obtaining an adequate tissue sample (p=0.03) histology diagnostic yield (p=0.05) and overall diagnostic yield (p=0.04) but there was no correlation between lesion size and yield on cytology (p=0.389). Sampling multiple stations and targeting larger lesions led to an improved diagnostic yield; the effect of targeting multiple stations was even more pronounced in granulomatous disease.

The global prevalence of allergic diseases have increased considerably and are a major socio-economic burden. Asthma is a complex disease and understanding asthma phenotypes and endotypes could eventually lead to individualised management, and offer better symptom control and quality of life. In this review, we first summarise the pathogenesis of atopic asthma and delve into the assessment of sensitisation to aeroallergens through skin prick testing and serological testing with total and specific immunoglobulin E testing. We will then analyse the distribution of aeroallergen sensitisation patterns in the United States and Europe and its effect on the population. This review gives a comprehensive overview on atopy and it compares the prevalence and effect of atopy within various regions of both continents using data from large multicentre studies. We will conclude this review by discussing the efficacy of add-on treatments in the most prevalent severe asthma phenotypes and endotypes., peer-reviewed

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The use of oral methotrexate for refractory eosinophilic asthma in a tertiary asthma referral centre, Glenfield Hospital, Leicester, was evaluated between January 2006 and December 2014. The patients ( n = 61) were carefully phenotyped at baseline with markers of airway inflammation. In addition, a structured oral methotrexate proforma was utilized to evaluate response to therapy and adverse events. Oral steroid withdrawal was attempted 3 months after commencing treatment. Several outcomes were evaluated at 12 months, including both efficacy and adverse effects; 15% ( n = 9/61) responded by achieving a decrease in daily oral corticosteroid dose (mean 8.43 (±8.76) mg), although we were unable to identify factors that predicted a treatment response. There were no other significant changes in any other clinical outcome measures. There was a high rate of adverse events (19/61 (31%)), primarily gastrointestinal/hepatitis. Our findings support the use of biological agents in preference to using oral methotrexate as a steroid sparing agent at the first instance. In the event of failure of these agents, oral methotrexate remains a therapeutic option, which can be considered in highly specialist severe asthma centres.

Cigarette smoke is the main source of indoor chemical and toxic elements. Cadmium (Cd), Thallium (Tl), Lead (Pb) and Antimony (Sb) are important contributors to smoke-related health risks. Data on the association between Rare Earth Elements (REE) Cerium (Ce) and Lanthanum (La) and domestic smoking are scanty. To evaluate the relationship between cigarette smoke, indoor levels of PM

Introduction: Exposure to airborne allergens was associated to increasing exacerbation of respiratory symptoms and airways inflammation. Fractional exhaled nitric oxide (FeNO) is a non-invasive met...

An 18-year-old man presented to the local hospital in Malta, with dyspnoea, cough, mild haemoptysis, chest pain and night sweats. CT revealed a right hilar mass. Pleural tap, bronchoscopy and open lung biopsy were inconclusive. Biopsies obtained at repeat bronchoscopy and endobronchial ultrasound (EBUS) revealed a likely diagnosis of inflammatory myofibroblastic tumour (IMT). The patient subsequently underwent right pneumonectomy, and histology revealed the presence of two further nodules apart from the main tumour. Follow-up with positron emission tomography (PET)/CT showed the development of a right basal paracardial lesion due to recurrence and the presence of lymph node, pleural and skeletal disease. Despite radiotherapy to the recurrent nodule and chemotherapy, there was skeletal disease progression. Treatment with an anaplastic lymphoma kinase inhibitor, ceritinib, resulted in very good metabolic response. This case report highlights the importance of keeping IMT in mind when the diagnosis of lung tumours is difficult, as delayed diagnosis may lead to worsened prognosis.

Introduction. E-learning is a useful tool providing after-hours access to continuous Medical Education and the theoretical component of postgraduate medical trainings. Furthermore, it allows accurate recording of trainee activity. Objective. To observe the pattern of usage by trainees and to assess feedback on e-learning use. Method. A modular object-oriented dynamic learning environment (Moodle) online e-learning management system – including 24 online modules relevant to different medical specialities and 24 quizzes. All 23 (14 Female) medical ‘Commontrunk’ trainees at Mater Dei Hospital, Malta were asked to complete these modules as part of their compulsory academic activities within a 6-week deadline. Data on usage was collected by the learning management system and trainees were asked to fi ll in an online feedback form. Results. All trainees (n=23) completed all modules; however, 36.1% of modules (M=49.8%, F=27.4%) were completed in the last week. Nineteen (n=21) trainees found the e-learning as a useful or very useful tool. 19/21 Students reported presentations as being of good/high quality and 13/21 reported quizzes to be of a good/high quality standard. Selfrating of acquisition of new knowledge was 7.95 (SD 1.75) on a scale of 0–10. The quality of IT work was rated 7.81 (SD 1.86). Forty-three percent (M 36.6%, F 47.0%) of modules were completed from 16:00 hours to 19:59 hours; 16.5% (M=29.3%, F=16.5%); from 20:00 hours to 23:59 hours and 4.5% (M=8.2%, F=2.2%) from 00:00 hours to 03:59 hours. A total of 31.2% of modules were performed during weekends (Sat=13.6%, Sun=17.6%, Average Mon–Fri=13.8%). Average time to complete learning module: F=49.7 minutes, M=76.4 minutes. Conclusion. Overall feedback on e-learning was positive. There was signifi cant after hour and weekend use. Gender diff erences in time of access and total time needed to complete modules were noted. Keywords: Moodle, Online E-Learning, “common trunk” trainees, feedback, medicine (Published: 29 June 2012) DOI: 10.3109/21614083.2012.699440

Intro: RESPIRA (EU Funded - Italia-Malta 07-13) indicated that living in Malta is a risk factor for asthma. Aim: To determine the chemical profile of PM2.5 in Malta, and compare with reference values. Methods: Using FAI pumps at 10l/min/48hr, samples were collected on Teflon and Quartz filters from 6 schools and 46 homes in Malta. Total ICP-MS measurement (Residual+extracted), X ray Fluorescence, and thermo-optical methods(TO) were used for analysis by CNR in Rome. Aeroqual IQM60 counters were used for total PM2.5 mass. All data in ng/m3, Mean, (1st to 3rd quartile) of all 48 hour measurements., N/A

Objective. The optimal time-frame for pulmonary rehabilitation (PR) in patients diagnosed with chronic obstructive pulmonary disease (COPD) is still debated. A 12 week programme was designed looking at whether the benefits were reached at or before a 12 week period of PR for COPD patients. Method. Seventy-five patients (59 males, 16 females) aged 40 75 years were referred from the local general hospital in Malta. Baseline assessments were carried out on all patients 2 weeks before initiation of the programme. Sixty patients were eligible to start a twice-weekly, 12 week multidisciplinary programme delivered after the screening process. The Six-Minute Walk Test (6MWT), dyspnoea score using the Borg scale, spirometry testing, plethysmography, COPD Assessment Tool (CAT) score, St George's Respiratory Questionnaire (SGRQ) and Hospital and Anxiety scale score were monitored at 4 weekly intervals throughout the 12 weeks of PR for these COPD patients. Results. The 6MWT distance increased by a mean total of 132.45 m (p < 0.001) by 12 weeks, with the highest change recorded in the first 4 weeks for the milder COPD patients. Lung function test improvements were marginal. Borg scale readings at rest and following exertion decreased significantly from weeks 0 to 4 but remained fairly constant thereafter. The Body mass index, airway Obstruction, Dyspnoea, and Exercise capacity (BODE) index, SGRQ and CAT score values decreased significantly throughout the weeks irrespective of the initial Medical Research Council score. Anxiety scoring decreased significantly by 12 weeks, while the depression rating improved by 8 weeks. Conclusion. These findings show that 12 weeks of PR in this cohort of COPD patients resulted in clinically significant changes in functional outcome measures which are supported by statistically significant changes in health-related quality of life measures. In milder COPD cases, by 4 weeks of PR gains in exercise tolerance had already resulted. The more severe group required more time to obtain improvements. Therefore, hospitals could organize shorter PR programmes for larger numbers of patients with milder COPD., peer-reviewed

Following a provisional diagnosis of asthma of several years' duration by his general practitioner, a 43-year-old otherwise healthy man who was a non-smoker was referred to a pulmonologist with worsening productive cough and exertional breathlessness. A thoracic CT scan revealed dilated airways (tracheal diameter 35 mm, left bronchial diameter 20 mm, right bronchial diameter 18 mm). Inflamed and easily collapsible airways were seen on bronchoscopy. The patient remained stable and was followed up with regular spirometry. A follow-up CT scan 7 years later showed tracheobronchomegaly (tracheal diameter 42 mm, left bronchial diameter 25 mm, right bronchial diameter 23 mm) with large cystic spaces consistent with Mounier-Kuhn syndrome. Repeat bronchoscopy showed a massively dilated trachea and generalised collapse on expiration with a dilated thin-walled bronchial tree. He was deemed ineligible for lung transplantation due to the extent of airway involvement making it difficult to anastomose donor lung to native tissue.