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Chronic Obstructive Pulmonary Disease (COPD) constitutes a major public health problem, and it is anticipated that its prevalence will continue to increase in the future. Its progressive nature requires a continuous and well-coordinated care approach. The follow-up for COPD should primarily focus on disease screening and control, which includes monitoring of pulmonary function, prevention of exacerbations, identification of aggravating factors and comorbidities, as well as ensuring treatment adequacy and adherence. However, existing clinical practice guidelines and consensus documents offer limited recommendations for the follow-up. In this context, we undertake a review of COPD treatment and the continuity of care recommendations endorsed by several scientific societies. Moreover, we underscore the importance of the involvement of nursing and community pharmacy in this process, as well as the utilization of quality indicators in the provision of care for the disease., Depto. de Medicina, Fac. de Medicina, TRUE, pub, Descuento UCM

María Torres-Durán,1 José Luis López-Campos,2,3 Myriam Calle Rubio,4 Carmen Montero-Martínez,5 Ana Priegue Carrera,6 Rosanel Amaro Rodríguez,7 Miriam Barrecheguren,8 María Ángeles Barrio Guirado,9 Francisco Javier Callejas-González,10 Francisco Casas-Maldonado,11 Layla Diab-Cáceres,12 Pilar García-Meseguer,9 José María Hernández-Pérez,13 Lourdes Lázaro-Asegurado,14 Cristina Martínez-González,15 Carlos Martínez Rivera,16 Francisco Javier Michel,17 José-Bruno Montoro-Ronsano,18 Raquel Sánchez,19 Marta Ortiz-Pica,20 Isabel Parra,21 José Pablo Quintero García,22 María del Rosario Ruiz-Serrano-de la Espada,23 Begoña Tortajada-Goitia,24 Marc Miravitlles8 1Pneumology Department, Hospital Álvaro Cunqueiro, NeumoVigo I+i Research Group, IIS Galicia Sur, Vigo, Spain; 2Instituto de Salud Carlos III, Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Madrid, Spain; 3Medical and Surgery Unit for Respiratory Diseases, Instituto de Biomedicina de Sevilla (IBiS), Hospital Universitario Virgen del Rocío/Universidad de Sevilla, Seville, Spain; 4Pneumology Department, Research Institute of Hospital Clínico San Carlos (IdISSC), Department of Medicine, Faculty of Medicine, University Complutense of Madrid, Madrid, Spain; 5Pneumology Department, Hospital Universitario de A Coruña, A Coruña, Spain; 6Nursing Unit, Hospital Álvaro Cunqueiro, Pontevedra, Spain; 7Pneumology Department, Hospital Clínic, Barcelona, Spain; 8Pneumology Department, Hospital Universitari Vall d’Hebron, Vall d’Hebron Institut de Recerca (VHIR), Vall d’Hebron Barcelona Hospital Campus, Barcelona, Spain; 9Nursung Unit, Hospital Universitari Vall d’Hebron, Barcelona, Spain; 10Pneumology Department, Complejo Hospitalario Universitario de Albacete, Albacete, Spain; 11Pneumology Department, Hospital Universitario Clínico San Cecilio, Granada, Spain; 12Pneumology Department, Hospital Universitario 12 de Octubre, Madrid, Spain; 13Pneumology Department

Purpose: Administration of exogenous alpha-1 antitrypsin (AAT) is the only specific therapy for the management of pulmonary morbidity in patients with AAT deficiency. It requires weekly or biweekly intravenous infusions, which may impact patient independence and quality of life. Self-administration of AAT therapy is an alternative to reduce the burden for patients who require AAT therapy. We presented herein experts’ recommendations for the implementation of a program for the self-administration of AAT. Methods: This project was conducted using a modified nominal group technique and was undertaken in two online meetings involving the participation of 25 experts: specialists in pulmonology (n=17), nurses (n=5) and hospital pharmacists (n=3). Results: The following issues were discussed, and several recommendations were agreed upon on the following topics: a) patient profile and clinical evaluation, establishing selection criteria that should include clinical as well as social criteria; b) role of health care professionals, suggested roles for specialists in pulmonology, nurses, and hospital pharmacists; c) training by the nurse, including recommendations before initiating the training and the content of the training sessions; and d) logistic issues and follow-up, adherence, and patient support. Conclusion: We expect this proposal to increase awareness of this therapeutic alternative and facilitate the implementation of self-administration programs, thus contributing to optimizing the patient experience with AAT therapy. Further research on the outcomes of these programs, especially from the patient perspective, will also help to improve their design and implementation.

Alpha-1 antitrypsin deficiency (AATD) is characterised by low serum levels of alpha-1 antitrypsin (AAT) and predisposes to the development of pulmonary emphysema and liver disease [1]. This deficiency is considered a rare condition and its prevalence ranges from one in 2400 to one in 6000 individuals in Western European countries [2]. Due to its low prevalence, large observational prospective registries are needed to collect information about the clinical characteristics and prognosis of the individuals affected.

Background: Although bronchiectasis has been recognised as a feature of some patients with Alpha1-Antitrypsin deficiency the prevalence and characteristics are not widely known. We wished to determine the prevalence of bronchiectasis and patient characteristics. The first cohort of patients recruited to the EARCO (European Alpha1 Research Collaboration) International Registry data base by the end of 2021 was analysed for radiological evidence of both emphysema and bronchiectasis as well as baseline demographic features. Results: Of the first 505 patients with the PiZZ genotype entered into the data base 418 (82.8%) had a reported CT scan. There were 77 (18.4%) with a normal scan and 38 (9.1%) with bronchiectasis alone. These 2 groups were predominantly female never smokers and had lung function in the normal range. The remaining 303 (72.5%) ZZ patients all had emphysema on the scan and 113 (27%) had additional evidence of bronchiectasis. Conclusions: The data indicates the bronchiectasis alone is a feature of 9.1% of patients with the PiZZ genotype of Alpha1-antitrypsin deficiency but although emphysema is the dominant lung pathology bronchiectasis is also present in 27% of emphysema cases and may require a different treatment strategy.

Marc Miravitlles,1,2 Fernando González-Torralba,3 Cristina Represas-Represas,4 Xavier Pomares,2,5 Eduardo Márquez-Martín,2,6 Cruz González,7 Carlos Amado,8 Carles Forné,9,10 Soledad Alonso,11 Bernardino Alcázar,12 Miriam Barrecheguren,1 Juan María Jurado Mirete,13 Elsa Naval14 1Pneumology Department, Hospital Universitari Vall d’Hebron, Vall d’Hebron Institut de Recerca (VHIR), Vall d’Hebron Barcelona Hospital Campus, Barcelona, Spain; 2CIBER de Enfermedades Respiratorias (CIBERES), ISCIII, Madrid, Spain; 3Pneumology Department, Hospital Universitario del Tajo, Aranjuez, Spain; 4Pneumology Department, Hospital Álvaro Cunqueiro, Vigo, Spain; 5Pneumology Department, Corporació Sanità ria Parc Taulí, Sabadell, Spain; 6Medical-Surgical Unit for Respiratory Diseases, Hospital Universitario Virgen del Rocío, Sevilla, Spain; 7Pneumology Department, Hospital Clínico de Valencia, Valencia, Spain; 8Pneumology Department, Hospital Universitario Marqués de Valdecilla, Santander, Spain; 9Heorfy Consulting, Lleida, Spain; 10Basic Medical Sciences Department, University of Lleida, Lleida, Spain; 11Pneumology Department, Hospital Universitario de Torrejón, Torrejón de Ardoz, Spain; 12Pneumology Department, Hospital Universitario Virgen de las Nieves, Granada, Spain; 13Scientific Department, GOC Health Consulting, Barcelona, Spain; 14Pneumology Department, Hospital Universitario de La Ribera, Alzira, SpainCorrespondence: Marc Miravitlles, Pneumology Department, Hospital Universitari Vall d’Hebron, P. Vall d’Hebron 119-129, Barcelona, 08035, Spain, Tel/Fax +34 932746083, Email marcm@separ.esIntroduction: Identifying the variables that guide decision-making in relation to the use of inhaled corticosteroids (ICS) can contribute to the appropriate use of these drugs. The objective of this study was to identify the clinical variables that physicians consider most relevant for prescribing or withdrawing ICS in COPD.Methods: A cross-sectional survey was conducted i

Miguel Angel Martinez-García,1,2 David Rigau,3 Miriam Barrecheguren,2,4 Alberto García-Ortega,1 Alexa Nuñez,2,4 Grace Oscullo Yepez,1 Marc Miravitlles2,4 1Pneumology Department, Hospital Universitario y Politécnico La Fe, Valencia, Spain; 2CIBER de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, Madrid, Spain; 3Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona, Spain; 4Pneumology Department, Hospital Universitari Vall d´Hebron, Vall d’Hebron Institut de Recerca (VHIR), Vall d’Hebron Barcelona Hospital Campus, Barcelona, SpainCorrespondence: Marc Miravitlles, University Hospital Vall d’Hebron, Department of Pneumology, Vall d’Hebron Barcelona Hospital Campus, Pg Vall d’Hebron 119-129, Barcelona, 08036, Spain, Email marcm@separ.esBackground: Chronic bronchial infection is frequent in chronic obstructive pulmonary disease (COPD), but the impact of the isolation of pathogenic bacteria, and in particular Pseudomonas aeruginosa (PA) in respiratory samples on the prognosis of COPD is unclear.Methods: We conducted a systematic review of prognostic studies including patients with isolation of PA in sputum in stable state or during exacerbations of COPD. The main outcomes were all-cause mortality, respiratory mortality, and number and severity of future exacerbations. Data were expressed as hazard ratio (HR) (95% confidence interval [CI]) whenever possible.Results: Of 2773 studies, eight were finally included (23,228 individuals). The mean age ranged from 65.5 to 73 years. Six studies reported data for all-cause mortality. The adjusted risk of death was almost double in patients with PA isolation (HR 1.95, 95% CI, 1.34 to 2.84; quality of evidence moderate). Patients with PA isolation showed a three times higher adjusted risk of readmission at 30 days after discharge (OR 3.60, 95% CI, 3.60 to 12.03, 1 study; quality of evidence very low), and more than double adjusted risk of death and hospitalization at two years (HR 2.80, 9

Screening of liver disease in alpha-1 antitrypsin deficiency (AATD) is usually carried out with liver enzymes, with low sensitivity. We conducted a multicenter cross-sectional study aiming to describe the utility of transient elastography for the identification of liver disease in patients with AATD. A total of 148 AATD patients were included. Among these, 54.7% were Pi*ZZ and 45.3% were heterozygous for the Z allele. Between 4.9% and 16.5% of patients had abnormal liver enzymes, without differences among genotypes. Liver stiffness measurement (LSM) was significantly higher in Pi*ZZ individuals than in heterozygous Z (5.6 vs. 4.6 kPa; p = 0.001). In total, in 8 (5%) individuals LSM was >7.5 kPa, considered significant liver fibrosis, and ≥10 kPa in 3 (1.9%) all being Pi*ZZ. Elevated liver enzymes were more frequently observed in patients with LSM > 7.5 kPa, but in 5 out of 8 of these patients all liver enzymes were within normal range. In patients with AATD, the presence of abnormal liver enzymes is frequent; however, most of these patients do not present significant liver fibrosis. Transient elastography can help to identify patients with liver fibrosis even with normal liver enzymes and should be performed in all Z-allele carriers to screen for liver disease., Grifols, Depto. de Medicina, Fac. de Medicina, TRUE, pub

The aim of this study was to elucidate whether long-term LT survivors with normal spirometry achieve normal exercise capacity, and to identify predictive factors of exercise capacity. This was a cross-sectional multicentre study, where bilateral LT recipients who survived at least 10 years after LT, with normal spirometry, no diagnosis of CLAD and modified Medical Research Council dyspnoea degree ≤2 underwent cardiopulmonary exercise testing (CPET). 28 LT recipients were included with a mean± age of 48.7±13.6 years. Oxygen uptake (V' ) had a mean± value of 21.49±6.68 mL·kg −1 ·min −1 (75.24±15.6%) and the anaerobic threshold was reached at 48.6±10.1% of the V' predicted. The mean± heart rate reserve at peak exercise was 17.56±13.6%. The oxygen pulse increased during exercise and was within normal values at 90.5±19.4%. The respiratory exchange ratio exceeded 1.19 at maximum exercise. The median (25-75 th percentile) EuroQol-5D score was 1 (0.95-1), indicating a good quality of life. The median (25-75 th percentile) International Physical Activity Questionnaire score was 5497 (4007-9832) MET-min·week −1 with 89% of patients reporting more than 1500 MET-min·week −1. In the multivariate regression models, age, sex and diffusing capacity of the lung for carbon monoxide remained significantly associated with V' (mL·kg −1 ·min −1); haemoglobin and forced expiratory volume in 1 s were significantly associated with maximum work rate (watts), after adjusting for confounders. We report for the first time near-normal peak V' values during CPET and normal exercise capacity in long-term LT recipients without CLAD. This is a multicentre study reporting, for the first time, near-normal peak V ' values during cardiopulmonary exercise testing and normal exercise capacity in long-term lung transplant recipients without CLAD