EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease.

Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) are considered the same disease, but a common approach for diagnosis and management is still missing. METHODS: In May 2022, EULAR and PReS endorsed a proposal for a joint task force (TF) to develop recommendations for the diagnosis and management of sJIA and AOSD. The TF agreed during a first meeting to address four topics: similarity between sJIA and AOSD, diagnostic biomarkers, therapeutic targets and strategies and complications including macrophage activation syndrome (MAS). Systematic literature reviews were conducted accordingly. RESULTS: The TF based their recommendations on four overarching principles, highlighting notably that sJIA and AOSD are one disease, to be designated by one name, Still's disease.Fourteen specific recommendations were issued. Two therapeutic targets were defined: clinically inactive disease (CID) and remission, that is, CID maintained for at least 6 months. The optimal therapeutic strategy relies on early use of interleukin (IL-1 or IL-6 inhibitors associated to short duration glucocorticoid (GC). MAS treatment should rely on high-dose GCs, IL-1 inhibitors, ciclosporin and interferon-γ inhibitors. A specific concern rose recently with cases of severe lung disease in children with Still's disease, for which T cell directed immunosuppressant are suggested. The recommendations emphasised the key role of expert centres for difficult-to-treat patients. All overarching principles and recommendations were agreed by over 80% of the TF experts with a high level of agreement. CONCLUSION: These recommendations are the first consensus for the diagnosis and management of children and adults with Still's disease.

Pharmacists' involvement in physical activity promotion in community pharmacy: a systematic review.

BACKGROUND: Physical activity (PA) promotion in healthcare systems is one of the priority areas highlighted by the World Health Organization, which recognizes that progress has been slow largely due to a lack of awareness and investment while requiring a system-based approach. Community pharmacies are one of the health structures that are more easily accessible to populations, thus constituting an ideal venue for developing health promotion activities. This research aimed to describe PA-enabling interventions developed in community pharmacies by pharmacists. METHODS: An electronic search was performed in PubMed, Scopus, Web of Science, Cochrane and reference lists of the different papers until June 2023. Studies were eligible if performed in community pharmacies by pharmacists, focused on interventions aimed at increasing PA levels and if at least one PA-specific outcome was measured at two different time points. RESULTS: We identified 7076 publications in the initial search, plus 31 records identified through backward citation tracking from relevant studies. After an initial screening, 236 were selected for full-text analysis. Of the 29 selected papers, 10 presented a low risk of bias for the measurement of PA levels. PA outcomes were generally self-reported outcomes where the change in terms of the percentage of individuals considered active or who increased PA because of the intervention. CONCLUSION: Several interventions to improve PA through community pharmacies were found although with a high level of heterogeneity and with only few with a low risk of bias. More targeted research that aims to capture PA levels and support the training of healthcare professionals is needed.

Outcomes of SARS-CoV-2 infection among children and young people with pre-existing rheumatic and musculoskeletal diseases.

OBJECTIVES: Some adults with rheumatic and musculoskeletal diseases (RMDs) are at increased risk of COVID-19-related death. Excluding post-COVID-19 multisystem inflammatory syndrome of children, children and young people (CYP) are overall less prone to severe COVID-19 and most experience a mild or asymptomatic course. However, it is unknown if CYP with RMDs are more likely to have more severe COVID-19. This analysis aims to describe outcomes among CYP with underlying RMDs with COVID-19. METHODS: Using the European Alliance of Associations for Rheumatology COVID-19 Registry, the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry, and the CARRA-sponsored COVID-19 Global Paediatric Rheumatology Database, we obtained data on CYP with RMDs who reported SARS-CoV-2 infection (presumptive or confirmed). Patient characteristics and illness severity were described, and factors associated with COVID-19 hospitalisation were investigated. RESULTS: 607 CYP with RMDs <19 years old from 25 different countries with SARS-CoV-2 infection were included, the majority with juvenile idiopathic arthritis (JIA; n=378; 62%). Forty-three (7%) patients were hospitalised; three of these patients died. Compared with JIA, diagnosis of systemic lupus erythematosus, mixed connective tissue disease, vasculitis, or other RMD (OR 4.3; 95% CI 1.7 to 11) or autoinflammatory syndrome (OR 3.0; 95% CI 1.1 to 8.6) was associated with hospitalisation, as was obesity (OR 4.0; 95% CI 1.3 to 12). CONCLUSIONS: This is the most significant investigation to date of COVID-19 in CYP with RMDs. It is important to note that the majority of CYP were not hospitalised, although those with severe systemic RMDs and obesity were more likely to be hospitalised.

Sinus node disfunction in an adolescent with systemic lupus erythematosus.

Cardiac involvement in systemic lupus erythematosus (SLE) is well documented. The pericardium, myocardium and endocardium, as well as the coronary arteries, the valves and the conduction system can all be affected. While pericarditis is common, arrythmias are less frequently described.We present a 13-year-old male, who had fatigue, anorexia, weight loss, myalgias and arthralgias for four months. On physical examination, we identified bradycardia (heart rate 31-50 bpm), oral and nasal ulcers and polyarthritis. The laboratory results showed hemolytic anemia, hypocomplementemia, antinuclear and anti-dsDNA antibodies, hematuria and non-nephrotic proteinuria. Renal function was normal. Lupus nephritis class II was diagnosed by kidney biopsy. On the transthoracic echocardiogram we identified a minimal pericardial effusion, suggesting pericarditis, and, on the electrocardiogram, we detected sinus arrest with junctional rhythm, denoting sinus node dysfunction. The patient was diagnosed with juvenile SLE with cardiac, renal, musculoskeletal and hematologic involvement. Disease remission and cardiac rhythm control were obtained with steroids and mycophenolate mofetil. Currently, the patient is asymptomatic, with normal sinus rhythm.We described an adolescent with SLE who had sinus node dysfunction upon diagnosis. Other cases have been reported in adults but none in juvenile SLE. All SLE patients should have a thorough cardiac examination to promptly diagnose and treat the innumerous cardiac manifestations of this disease.

Spondyloepiphyseal dysplasia type Stanescu: Expanding the clinical and molecular spectrum of a very rare type II collagenopathy.

Spondyloepiphyseal dysplasia type Stanescu (SED-S) is a very rare type II collagenopathy. We describe an 8-year-old boy who presented with short trunk, C2-C3 vertebral fusion, hand, foot, leg and thigh pain, stiffness and limited joint mobility, and waddling gait. Radiographs showed platyspondyly with anterior wedging and endplate irregularities, broad femoral necks, and large epiphyses and epiphyseal equivalents. Differential diagnosis included progressive pseudorheumatoid dysplasia and SED-S. A skeletal dysplasia custom-designed NGS panel was performed and the heterozygous pathogenic variant c.620G>A; p.(Gly207Glu) in COL2A1 was detected, establishing the diagnosis of SED-S. Vertebral fusions, observed in our patient, have not been previously described in this dysplasia. This variant has not been previously associated with SED-S, but was reported in two other families with spondyloepiphyseal dysplasia. Thus, this case expands the clinical and mutational spectrum of SED-S and demonstrates that SED-S significantly overlaps with other skeletal dysplasias.

Epidemiology of Transthyretin Familial Amyloid Polyneuropathy in Portugal: A Nationwide Study.

BACKGROUND: Transthyretin-associated familial amyloid polyneuropathy (TTR-FAP) is a rare, hereditary, progressive and neurodegenerative disease. We aimed to study -TTR-FAP epidemiology in Portugal. METHODS: National, observational, prospective and retrospective, case identification of adults with TTR-FAP. Countrywide patient multiple identification sources included reference centers registries and centralized medical electronic prescription database. Crude rates were reported per 100,000 adult inhabitants. RESULTS: Over 2010-2016 period, mean incidence rates was 0.87/100,000 (95% CI 0.68-1.10) corresponding to 71 new patients yearly, that has decreased 31% in the last 7 years. The proportion of late-onset cases (age ≥50 years) among incident cases was 28.7%. Estimated crude 2016 prevalence was 22.93/100,000 adult inhabitants (95% CI 21.90-23.99) corresponding to 1,865 TTR-FAP individuals in Portugal (45.8% male; mean age: 52.3 ± 15.4 years). In 2016, the Portuguese region with the highest TTR-FAP prevalence shows a 16% prevalence increase over the last 25 years. CONCLUSIONS: In Portugal, TTR-FAP affects both genders and mainly young adults. TTR-FAP incidence appears to be decreasing while prevalence is increasing. In comparison to previous studies, there is an increased representativeness of late-onset patients. This epidemiological setting poses future and complex challenges for the social and healthcare system, strengthening the relevance of regular epidemiologic surveillance.

The Portuguese version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR).

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Portuguese language. The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the three Likert assumptions, floor/ceiling effects, internal consistency, Cronbach's alpha, interscale correlations, and construct validity (convergent and discriminant validity). A total of 80 JIA patients (6.3% systemic, 68.8% oligoarticular, 3.7% RF negative polyarthritis, 21.2% other categories) and 30 healthy children were enrolled. The JAMAR components discriminated well healthy subjects from JIA patients. All JAMAR components revealed good psychometric performances. Notably, there was no significant difference between healthy subjects and their affected peers in school-related items. In conclusion, the Portuguese version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research.

Effectiveness and long-term retention of anti-tumour necrosis factor treatment in juvenile and adult patients with juvenile idiopathic arthritis: data from Reuma.pt.

OBJECTIVES: Assess the effectiveness and safety of biologic therapy as well as predictors of response at 1 year of therapy, retention rate in biologic treatment and predictors of drug discontinuation in JIA patients in the Portuguese register of rheumatic diseases. METHODS: We prospectively collected patient and disease characteristics from patients with JIA who started biological therapy. Adverse events were collected during the follow-up period. Predictors of response at 1 year and drug retention rates were assessed at 4 years of treatment for the first biologic agent. RESULTS: A total of 812 JIA patients [65% females, mean age at JIA onset 6.9 years (s.d. 4.7)], 227 received biologic therapy; 205 patients (90.3%) were treated with an anti-TNF as the first biologic. All the parameters used to evaluate disease activity, namely number of active joints, ESR and Childhood HAQ/HAQ, decreased significantly at 6 months and 1 year of treatment. The mean reduction in Juvenile Disease Activity Score 10 (JADAS10) after 1 year of treatment was 10.4 (s.d. 7.4). According to the definition of improvement using the JADAS10 score, 83.3% respond to biologic therapy after 1 year. Fourteen patients discontinued biologic therapies due to adverse events. Retention rates were 92.9% at 1 year, 85.5% at 2 years, 78.4% at 3 years and 68.1% at 4 years of treatment. Among all JIA subtypes, only concomitant therapy with corticosteroids was found to be univariately associated with withdrawal of biologic treatment (P = 0.016). CONCLUSION: Biologic therapies seem effective and safe in patients with JIA. In addition, the retention rates for the first biologic agent are high throughout 4 years.

A lifelong journey: Long-term perspectives on Juvenile Idiopathic Arthritis.

Juvenile Idiopathic Arthritis (JIA) represents a diverse group of chronic inflammatory conditions that begin in childhood or adolescence and continue into adulthood, with varying severity and outcomes. This review discusses the complexities of transitioning JIA patients emphasizing that inadequate transition from pediatric to adult care leads to loss of follow-up, treatment discontinuation, and increased disease activity. Furthermore, challenges in disease classification hinder continuity of care across lifespan. It is also pointed out that predicting long-term outcomes in JIA remains complex due to heterogeneity and evolving phenotypes. Factors such as disease category, joint involvement, and treatment influence disease activity, functional disability, and quality of life. Despite advancements in treatment strategies, a substantial proportion of patients experience long-term disability and joint damage. Finally, it is underscored that optimising long-term outcomes in adults with JIA requires a multifaceted approach encompassing structured transition processes, personalised treatment strategies, and comprehensive management of comorbidities. Further research is needed to refine predictive models, enhance disease monitoring tools, and understand the complex interplay between disease activity, treatment response, and long-term outcomes.

Physical activity promotion in the community pharmacy: An opportunity for an expanded role?

BACKGROUND: Physical inactivity is a major risk factor for the development of chronic diseases, and it is increasingly prevalent in the Portuguese population. Pharmacists' role in promoting physical activity (PA) is still not well established, although health promotion is foreseen by law in Portugal. Competing tasks and location where the pharmacy is embedded can hinder this promotion in their daily practice. OBJECTIVE: The aim of this study was to identify the main barriers and facilitators of physical activity promotion (PAP) in Portuguese community pharmacies and explore possible pathways for future implementation of physical activity promotion. METHODS: In-depth, semi-structured interviews were conducted with purposively enrolled community pharmacists. Participant recruitment was aligned with data saturation. Data analysis comprised a mixed model of a deductive theme mapping strategy using the Theoretical Domains Framework (TDF) for the behaviour of promoting physical activity and an inductive approach for any other relevant themes and which might influence PA promotion. RESULTS: Data saturation was reached at eleven interviews. Barriers and facilitators for the behaviour of promoting PA were identified from 11 out of the 14 TDF domains. Following an inductive approach, other emerging codes were clustered in additional seven major themes. Highlighted barriers focused on domains #1 - Knowledge, #10 - Memory, Attention and Decision Processes and #13 - Environmental Context and Resources. Community mapping, establishment of remuneration models and the use of digital technologies were suggested as additional potential contributors to scale up PAP. CONCLUSION: Community pharmacists are well placed inside their communities to serve as a focal point for signposting, engagement with other healthcare professionals and community resources and activities organized by the pharmacy itself. Pharmacists should be supported in being knowledgeable, aware, and available when promoting PA in their daily counseling.

The role of community pharmacists and pharmacies in physical activity promotion: an interdisciplinary e-Delphi study.

BACKGROUND: Physical activity has a key role in the prevention and control of noncommunicable diseases. Community pharmacists are an accessible source to provide brief advice to people on how to be more physically active. Nonetheless, there is a limited understanding of stakeholders' perspectives on their role in promoting physical activity, to inform policy and practice. The present study addresses this gap. AIM: To determine consensus from different health professionals on the role of pharmacists and pharmacies in brief physical activity counselling in Portugal. METHOD: This cross-sectional study used a two-round e-Delphi panel. The questionnaire was organised into four domains of physical activity promotion and comprised 37 items. Interdisciplinary experts rated their level of agreement using a 5-point Likert scale. Consensus was set at the outset as 75% or more of participants scoring 4 or 5 (consensus "in") or 1 or 2 (consensus "out"). RESULTS: Forty-two health professionals involved in promoting physical activity in the ambulatory setting in Portugal were selected through purposive quota sampling. Eighteen out of 37 items were consensual in the first round and five more achieved consensus after the second round (62.2%). Physical activity promotion was seen as the role of all healthcare workforce and pharmacies were considered as suitable spaces for service provision, regardless of remuneration. CONCLUSION: This study endorses a set of roles for physical activity promotion in community pharmacy from an interdisciplinary perspective. Consensually established perspectives can inform policy making and practice, streamlining the coordination of pharmacies with the national health service.

Effects of inpatient creatinine testing frequency on acute kidney injury identification and staging: a historical cohort study.

BACKGROUND: Acute kidney injury (AKI) is a multifactorial condition often induced by drugs commonly used in hospitals. Identifying and staging AKI necessitates frequent monitoring of renal function. AIM: To assess the impact of real-world hospital practices regarding serum creatinine (SCr) testing on the identification and staging of AKI, and its implications for adjusting drug doses. METHOD: A historical cohort study utilizing hospital records from all adult patients admitted between 01/06/2018 and 31/12/2020 was conducted. Patients with no SCr assessment during their stay or those with an SCr at admission ≥ 2 mg/dL were excluded. AKI was determined using two criteria, namely AKIN and KDIGO, considering the time intervals between two SCr tests as outlined in the criteria. Additionally, patients with SCr increases exceeding AKI limits, regardless the time interval, were also identified. The estimated glomerular filtration rate (eGFR) and kinetic eGFR (KeGFR) were calculated. RESULTS: During the study period, 17,269 hospitalizations and 62,255 SCr tests were recorded. Among the 17,032 hospitalizations with a length of stay > 48 h, 46.8% experienced periods with no SCr tests performed for more than 48 h. Any stage of AKI was identified in 7.0% of patients and in 9.1% using AKI and KDIGO criteria, respectively. Ignoring time limits in both criteria revealed potential AKI in 1942 patients (11.2%), indicating a potential underdiagnosis of AKI by 37.5% or 19.1%, depending on the criteria used. A total of 76 drugs requiring dose adjustment in patients with eGFR ≤ 50 ml/min were prescribed in 78.5% admissions. These drugs were prescribed in 87.9% of patients potentially underdiagnosed with AKIN and in 88.9% with KDIGO. CONCLUSION: There is a need for changes in the established hospital procedures to ensure more frequent testing of SCr levels. Implementing an advanced scope of practice for clinical pharmacists could support these changes.

The Initial Journey of Patients with Metastatic Pancreatic Cancer (PaCTO Project): A Nationwide Survey among Portuguese Specialist Physicians.

Introduction: We aimed to characterize the initial healthcare journey of metastatic pancreatic ductal adenocarcinoma (mPDAC) patients in Portugal, including healthcare provision and factors affecting therapeutic decisions, namely BRCA mutations testing. Methods: This is a descriptive cross-sectional, web-based survey using a convenience sampling approach. Portuguese oncologists and pathologists that routinely work with mPDAC patients from the different geographical regions and settings were invited to participate in the study via email (December 2020). Descriptive statistical analyses were performed, with categorical variables reported as absolute and relative frequencies, and continuous variables with non-normal distribution as median and interquartile range (IQR) (Stata v.15.0). Results: Seventy physicians participated in the study (43 oncologists, 27 pathologists). According to the responses, a median of 28 patients per center (IQR 12-70) was diagnosed with PDAC in the previous year; 22 of them referring (IQR 8-70) to mPDAC. The pointed median time from patients' first hospital admission until disease diagnosis/staging is between 2 and 4 weeks. Endoscopic ultrasound with fine-needle biopsy is available in most hospitals (86%). Around 50% of physicians request BRCA testing; the assessment of additional biomarkers besides BRCA is requested by 40% of professionals. Half of them stated that BRCA testing should be requested earlier-upon histological diagnosis, especially because the median time for results is of 4.0 weeks (IQR 4-8). PARP inhibitors such as olaparib, when available, would be the therapy of choice for most oncologists (71%) if no disease' progression occurs after 4 months. Treatments' selection is usually grounded on clinical criteria (e.g., performance status, liver function). Around 45% of patients use FOLFIRINOX/mFOLFIRINOX as the first-line therapy. Gemcitabine + nab-paclitaxel is used by 35% of patients as the second-line therapy. Conclusions: Physicians in Portugal support the increasing role of patient-tailored treatments in mPDAC, whose selection should be grounded on tumoral subtyping and molecular profiling. Further efforts to develop multidisciplinary teams, standardized clinical practice, and optimize the implementation of new target therapies are needed.

Introdução: Este estudo teve como objetivo caracterizar o percurso inicial dos doentes com adenocarcinoma ductal pancreático metastático (ACDPm) em Portugal, incluindo a prestação de cuidados de saúde e determinação de fatores que afetam as decisões terapêuticas, nomeadamente o teste de mutações BRCA. Métodos: Trata-se de um estudo descritivo transversal (web-based) usando uma abordagem de amostragem por conveniência. Médicos oncologistas e anatomopatologistas portugueses dedicados ao ACDPm e de diferentes regiões geográficas e instituições foram convidados a participar do estudo por email (Dez-2020). Foram realizadas análises estatísticas descritivas, com variáveis categóricas relatadas como frequências absolutas e relativas, e variáveis contínuas com distribuição não-normal como mediana e intervalo interquartil (IIQ) (Stata v.15.0). Resultados: Setenta médicos participaram do estudo (43 oncologistas, 27 patologistas). De acordo com as respostas, uma mediana de 28 doentes por centro (IIQ 12­70) foi diagnosticada com ACDP no ano anterior; 22 deles (IIQ 8­70) referentes a ACDPm. O tempo médio desde a primeira admissão hospitalar dos doentes até o diagnóstico/estadiamento da doença foi entre 2­4 semanas. A ultrassonografia endoscópica com biópsia por agulha fina é realizada pela maioria dos hospitais (86%). Aproximadamente 50% dos médicos referem solicitar o teste BRCA; a avaliação de biomarcadores adicionais além do BRCA é solicitada por 40% dos profissionais. Metade dos médicos assume que o teste BRCA deveria ser solicitado mais precocemente ­ durante o diagnóstico histológico, principalmente porque o tempo médio para obtenção do resultado é de 4,0 semanas (IIQ 4­8). Os inibidores PARP, como o olaparibe, quando disponíveis, seriam a terapia de escolha para a maioria dos oncologistas (71%) caso não haja progressão da doença após quatro meses. A seleção dos tratamentos é usualmente baseada em critérios clínicos (por exemplo, performance status, função hepática). Em cerca de 45% dos doentes é utilizado FOLFIRINOX/mFOLFIRINOX como terapia de primeira linha. Um esquema com Gemcitabina + nab-paclitaxel é usado em 35% dos doentes como terapia de segunda linha. Conclusões: Os médicos em Portugal apoiam o papel crescente do tratamento individualizado no ACDPm, cuja seleção deve ser baseada na subtipagem tumoral e no perfil molecular. São necessários mais esforços para capacitar as equipas multidisciplinares, desenvolver práticas clínicas padronizadas e otimizar a implementação de novas terapias-alvo.

The Effects of Benralizumab on Lung Volumes and Airway Resistance in Severe Eosinophilic Asthma: A Real-World Study.

INTRODUCTION: Add-on biological monoclonal antibodies such as benralizumab (anti-IL-5Ra) are recommended by international guidelines to reduce exacerbations in severe eosinophilic asthma (SEA). However, few studies have assessed the impact of these therapies on lung function-related outcomes. Our goal was to evaluate the effectiveness of benralizumab on lung function, including lung volumes and airway resistance, in SEA patients in Portugal. METHODS: This was a real-world, observational, prospective, multicentric study including adult patients diagnosed with SEA (January-June 2023). Spirometry and plethysmography were performed at baseline (T0) and after six months of treatment (T6) with benralizumab to assess: total lung capacity (TLC), residual volume (RV), forced expiratory volume in one second (FEV1), forced vital capacity (FVC), mean forced expiratory flow between 25% and 75% of FVC (mFEF-25/75), intrathoracic gas volume (ITGV), and respiratory airway resistance (Raw). Descriptive statistics (with categorical variables described as frequencies and continuous values as mean and standard deviation (SD)) and paired t-test and Cohen's d effect size were calculated (analyses performed in StataCorp v.15.1; StataCorp LLC, TX, USA). RESULTS: Overall, 30 SEA patients were evaluated, mostly women (n=18, 60.0%), with atopy (n=22, 73.3%), a mean age of 58.4 years (SD 11.7), and assisted by pulmonology (n=19, 63.3%) or immunology-allergology (n=11, 36.7%) services. Mean eosinophilia at baseline was 1103.57 cells/mcL (SD 604.88; minimum-maximum 460-2400); after the use of benralizumab, the count dropped to zero. After six months of treatment, a significant increase (p<0.0001) in FVC (15.3%), FEV1 (22.6%), and mFEF-25/75 (17.7%) were observed from baseline (Cohen's d between 0.78 and 1.11). ITGV, RV, RV/TLC, and Raw significantly decreased (p<0.0001) during the study period (-17.3%, -29.7%, -8.9%, and -100.6%, respectively) (Cohen's d between -0.79 and -1.06). No differences in TLC were obtained (p=0.173). No differences between sexes were observed for any measure. Patients with more significant eosinophilia (>900 cells/mcL count; n=15) presented better responses in FEV1 (p=0.001) and mFEF-25/75 (p=0.007). CONCLUSIONS: A notable eosinophil depletion with add-on benralizumab led to significant improvements in SEA patients' respiratory function (static lung volumes and airway resistance) in real-world settings after six months. The significant deflating effect of benralizumab on patients' hyperinflated lungs led to enhanced expiratory flow (increased FEV1 and mFEF-25/75) and air trapping (decreased RV/TLC), suggesting this antibody improves bronchial obstruction, lung hyperinflation, and airway resistance. Further studies in a larger population are required to confirm these findings.

Eosinophilic Granulomatosis with Polyangiitis - description of a pediatric patient with severe disease.

INTRODUCTION: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare vasculitis of small and medium sized blood vessels. CASE DESCRIPTION: Thirteen-year-old male, with history of rhinitis and asthma, who presented to the emergency room with one week of asthenia, arthralgias and myalgias and two days of fever. A diffuse petechial rash, palpable purpura and polyarthritis were detected on examination. Leukocytosis (34990/µL) with eosinophilia (66%) and elevated C-reactive protein were identified. The patient was admitted and ceftriaxone and doxycycline were started. The clinical status deteriorated in the following days. The patient developed myopericarditis, bilateral pulmonary infiltrates and pleural effusion, requiring mechanical ventilation and aminergic support. Non-clonal eosinophils were detected on the bone marrow aspiration and the skin biopsy showed leukocytoclastic vasculitis with eosinophils. Antineutrophil cytoplasmic antibodies and genetic analysis for hypereosinophilic syndrome mutations were negative. After treatment with methylprednisolone for three days a fast clinical, laboratory and radiological improvement occurred. The patient started azatiophrine and reduced steroids progressively. No relapses occurred since diagnosis five years ago. DISCUSSION: Clinical suspicion and early treatment of EGPA are crucial to improve prognosis.

Experience and Learning from the COVID-19 Pandemic in Portugal: Perceptions of Community Pharmacy Professionals.

Background: During the COVID-19 pandemic, community pharmacy (CP) professionals were among those who experienced the greatest risk of contracting SARS-CoV-2, which forced major adaptations. Objectives: The objectives of the study were to describe the changes implemented in CP professionals during the pandemic, understand the perception of professionals about their experience, and explore changes to remain. Methods: An observational and cross-sectional study was conducted via an online questionnaire (June-September 2020). The target population was CP professionals working in Portugal for >2 years and serving the public during the pandemic. Results: Of a total of 353 participants, 84% were female (mean age of 37.6 years), and 81% were pharmacists (mean professional experience of 12.9 years). In the management and organizational dimensions, the most mentioned changes were adaptation to legislative changes (90%), fluctuations in the treasury (82%), and reduction of working hours (46%). Only 2% resorted to simplified layoff. In the back office, there was a need to adapt stock management (93%) and purchase personal protective equipment (99%). In the front office, there was a change in service policies - wicket or conditional opening (92%), routes of the arrival of user requests (91%), and home delivery (82%). Physical changes occurred in 100% of pharmacies. The most frequently implemented procedures were the use of protection systems and PPE, articulation with hospital pharmacies for dispensing in proximity (75%), and training in this area (55%). Regarding interpersonal climate, improvements in the connection between team members are evident: increase in mutual help (57%), solidarity (54%), and group cohesion (50%); in the relationship with clients, the majority indicated the replacement of the usual user by third parties (71%), and changes in communication channels (increase in use of technological means 68%). Conclusions: Results illustrate the profound impact of the pandemic on CP professionals, both professionally and personally. It also highlights the importance of their roles in proximity and community support.

Introdução: Durante a pandemia de COVID-19, os profissionais de farmácia comunitária (FC) estiveram entre os que apresentaram maior risco de contrair SARS-CoV-2, o que obrigou a grandes adaptações. Objetivos: Descrever as alterações implementadas nas FC durante a pandemia, compreender a percepção dos profissionais sobre as suas vivências e explorar as mudanças a serem mantidas. Metodologia: estudo observacional e transversal (junho-setembro de 2020). A população alvo foram os profissionais de FC a trabalhar em Portugal há >2 anos e atender o público durante a pandemia. Resultados: 353 participantes, 84% do sexo feminino (idade média - 37,6 anos) e 81% eram farmacêuticos (média de experiência profissional de 12,9 anos). Nas dimensões "gestão e organização", as mudanças mais referidas foram a adaptação a alterações legislativas (90%), flutuações de tesouraria (82%) e redução do horário de trabalho (46%). Apenas 2% recorreram ao lay-off simplificado. No back office: necessidade de adequação do stock (93%) e aquisição de equipamentos de proteção individual (99%). No front office: alteração das políticas de atendimento ­ atendimento ao postigo ou abertura condicional (92%), vias de chegada dos pedidos dos utentes (91%) e entrega ao domicílio (82%). Alterações físicas ocorreram em 100% das farmácias. Os procedimentos implementados com maior frequência foram a utilização de sistemas de proteção e EPI, a articulação com farmácias hospitalares para dispensa de medicamentos de proximidade (75%) e formação nesta área (55%). Em relação ao clima interpessoal, foram evidentes as melhorias na ligação entre os membros da equipa: aumento da inter-ajuda (57%), solidariedade (54%) e coesão do grupo (50%); no relacionamento com os utentes, a maioria referiu a substituição do utente habitual por terceiros (71%) e alterações nos canais de comunicação (aumento da utilização de meios tecnológicos 68%). Conclusões: Os resultados ilustram o profundo impacto da pandemia nos profissionais de FC, tanto a nível profissional como pessoal. Também de destacar a importância do papel da FC como espaço de saúde de proximidade e apoio à comunidade.

How do hospital characteristics and ties relate to the uptake of second-generation biosimilars? A longitudinal analysis of Portuguese NHS hospitals, 2015-2021.

BACKGROUND: There is limited evidence on within-country discrepancies in biosimilar uptake. This study analyzes differences in timing and diffusion of biosimilar uptake across Portuguese NHS hospitals and explores possible determinants. RESEARCH DESIGN AND METHODS: We analyzed publicly accessible consumption data of originator biologic and biosimilar drugs for adalimumab, etanercept, infliximab, rituximab, and trastuzumab, by hospital and month for the years 2015-2021 (N = 9,467). We modeled the time to biosimilar adoption using survival regression models and the share of biosimilar consumption using generalized estimated equations with random hospital effects. RESULTS: Academic hospitals were characterized by a quicker uptake of adalimumab and infliximab biosimilars but lower shares for other drugs. A higher total consumption of biologics was related to a lower share of biosimilar uptake. A stronger participation in randomized controlled trials was linked to higher biosimilar shares and quicker uptake, except for rituximab. If all NHS hospitals had biosimilar shares equal to the highest ones, potential annual savings could reach 13.9 million euros. CONCLUSION: The findings suggest a need for capacity-building on biosimilar prescribing, including for doctors of academic hospitals and those working in settings where high biosimilar use would be expected.

Correction: The Meaning of Lymphadenopathies During Adjuvant Durvalumab After Chemoradiotherapy for Lung Cancer: Thinking Beyond Disease Progression.

[This corrects the article DOI: 10.7759/cureus.26729.].

Influence of the timing of biological treatment initiation on Juvenile Idiopathic Arthritis long-term outcomes.

BACKGROUND: Juvenile idiopathic arthritis (JIA) treatment is aimed at inducing remission to prevent joint destruction and disability. However, it is unclear what is the long-term impact on health-related outcomes of the timing of biological disease-modifying antirheumatic drug (bDMARD) initiation in JIA. Our aim was to evaluate the long-term impact of the time between JIA onset and the initiation of a bDMARD in achieving clinical remission, on physical disability and health-related quality of life (HRQoL). METHODS: Adult JIA patients registered in the Rheumatic Diseases Portuguese Register (Reuma.pt) and ever treated with bDMARD were included. Data regarding socio-demographic, JIA-related characteristics, disease activity, physical disability (HAQ-DI), HRQoL (SF-36), and treatments were collected at the last visit. Patients were divided into 3 groups (≤ 2 years, 2-5 years, or > 5 years), according to the time from disease onset to bDMARD initiation. Regression models were obtained considering remission on/off medication, HAQ-DI, SF-36, and joint surgeries as outcomes and time from disease onset to bDMARD start as an independent variable. RESULTS: Three hundred sixty-one adult JIA patients were evaluated, with a median disease duration of 20.3 years (IQR 12.1; 30.2). 40.4% had active disease, 35.1% were in remission on medication, and 24.4% were in drug-free remission; 71% reported some degree of physical disability. Starting a bDMARD > 5 years after disease onset decreased the chance of achieving remission off medication (OR 0.24; 95% CI 0.06, 0.92; p = 0.038). Patients who started a bDMARD after 5 years of disease onset had a higher HAQ and worse scores in the physical component, vitality, and social function domains of SF-36, and more joint surgeries when compared to an earlier start. CONCLUSION: Later initiation of bDMARDs in JIA is associated with a greater physical disability, worse HRQoL, and lower chance of drug-free remission in adulthood.