Respiratory

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https://westmid.openrepository.com/handle/20.500.14200/1455

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Sex-Differences in Alpha-1 Antitrypsin Deficiency: Data From the EARCO Registry
(International Journal of Spanish Respiratory Society of Pulmonology and Thoracic Surgery, 2022-12-16) Miravitlles, Marc; Turner, Alice M; Torres-Duran, María; Tanash, Hanan; Rodríguez-García, Carlota; López-Campos, José Luis; Chlumsky, Jan; Guimaraes, Catarina; Rodríguez-Hermosa, Juan Luis; Corsico, Angelo; Martinez-González, Cristina; Hernández-Pérez, José María; Bustamante, Ana; Parr, David G; Casas-Maldonado, Francisco; Hecimovic, Ana; Janssens, Wim; Lara, Beatriz; Barrecheguren, Miriam; González, Cruz; Stolk, Jan; Esquinas, Cristina; Clarenbach, Christian F; Hilal Ersöza,bMaría Torres-Duránc,dAlice M. Turnere,fHanan TanashgCarlota Rodríguez Garcíah,iAngelo Guido Corsicoj,kJosé Luis López-Camposl,dMarc MiravitllesmChristian F. Clarenbacho,pKenneth R. ChapmanqJose Mª Hernández PérezrCatarina GuimarãessEva BartošovskátTimm GreulichuMiriam BarrechegurenmAndreas Rembert Koczullav,wPhilipp Högera,bArturo Olivares Riveraa,bFelix Hertha,bFranziska C. Trudzinskia,b, for the EARCO study investigators ◊ a Department of Pneumology and Critical Care Medicine, Thoraxklinik University of Heidelberg, Heidelberg, Germany b Translational Lung Research Center Heidelberg (TLRC-H), German Center for Lung Research (DZL), Heidelberg, Germany c Pneumology Service Hospital Alvaro Cunqueiro, Vigo, Institute for Health Research Galicia Sur (IISGS), Vigo, Spain d Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, Madrid, Spain e Institute for Applied Health Research, University of Birmingham, Birmingham B15 2TT, United Kingdom f University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom g Department of Respiratory Medicine and Allergology, Skåne University Hospital, Lund University, Malmö, Sweden w Schön Klinik Berchtesgadener, PMU Salzburg, Austria
Sex and gender influence many aspects of chronic obstructive pulmonary disease (COPD). Limited data are available on this topic in alpha-1 antitrypsin deficiency (AATD). We therefore aimed to investigate sex issues in the EARCO registry, a prospective, international, observational cohort study.
Looking for new biomarkers in bronchiectasis (BE): sputum alpha-1 antitrypsin, neutrophil elastase activity and secretory leucocyte protease inhibitor and their role in bronchiectasis airways
(Poster Presentation, 2024) Lara, Beatriz; Traversi L, Pons C, Gallego M, Lara B, Suarez G, Tagliabue A, Ahroud M, Felipe Am Clofent D, Loor K, Alvarez A, Culebras M, Polverino E; Medical and Dental
No Abstract
Solitary pulmonary hyalinising granuloma : a rare cause of pulmonary nodule
(Oxford University Press, 2016-11-26) Adnan Saleem, Muhammad; Bhat, Rahul; Sinha, Bidisa; George Eliot Hospital, Nuneaton; University Hospitals Coventry and Warwickshire; Respiratory Medicine; Medical and Dental; Saleem, Muhammad Adnan; Bhat, Rahul; Sinha, Bidisa
A pulmonary nodule is a common incidental finding on chest imaging, which includes a wide variety of differential diagnosis. Pulmonary hyalinising granuloma is a rare disease aetiology of pulmonary nodule(s). We report a 74-year-old female who was referred to the respiratory clinic with incidental finding of a solitary pulmonary nodule on chest X-ray. CT confirmed the presence of a 1.2 cm solitary pulmonary nodule in the left upper lobe with no lymphadenopathy. The patient underwent wedge resection, and histopathological examination of the lesion confirmed pulmonary hyalinising granuloma. In most previously reported cases, patients had multiple lesions on chest radiography. Solitary pulmonary lesion is an uncommon presentation of this clinical entity and only a few cases have been reported in the literature.
Correction: Clinical and functional characteristics of individuals with alpha-1 antitrypsin deficiency: EARCO international registry
(BMC, 2023-02-18) Miravitlles, Marc; Turner, Alice M; Torres-Duran, María; Tanash, Hanan; Rodríguez-García, Carlota; López-Campos, José Luis; Chlumsky, Jan; Guimaraes, Catarina; Rodríguez-Hermosa, Juan Luis; Corsico, Angelo; Martinez-González, Cristina; Hernández-Pérez, José María; Bustamante, Ana; Parr, David G; Casas-Maldonado, Francisco; Hecimovic, Ana; Janssens, Wim; Lara, Beatriz; Barrecheguren, Miriam; González, Cruz; Stolk, Jan; Esquinas, Cristina; Clarenbach, Christian F; Respiratory; Medical and Dental; Turner, Alice; PARR, DAVID; Lara, Beatriz
None available
Clinical and functional characteristics of individuals with alpha-1 antitrypsin deficiency: EARCO international registry
(BMC, 2022-12-16) Miravitlles, Marc; Turner, Alice M; Torres-Duran, María; Tanash, Hanan; Rodríguez-García, Carlota; López-Campos, José Luis; Chlumsky, Jan; Guimaraes, Catarina; Rodríguez-Hermosa, Juan Luis; Corsico, Angelo; Martinez-González, Cristina; Hernández-Pérez, José María; Bustamante, Ana; Parr, David G; Casas-Maldonado, Francisco; Hecimovic, Ana; Janssens, Wim; Lara, Beatriz; Barrecheguren, Miriam; González, Cruz; Stolk, Jan; Esquinas, Cristina; Clarenbach, Christian F; Respiratory; Medical and Dental; Turner, Alice; PARR, DAVID; Lara, Beatriz
Background: Alpha-1 antitrypsin deficiency (AATD) is a rare disease that is associated with an increased risk of pulmonary emphysema. The European AATD Research Collaboration (EARCO) international registry was founded with the objective of characterising the individuals with AATD and investigating their natural history. Methods: The EARCO registry is an international, observational and prospective study of individuals with AATD, defined as AAT serum levels < 11 μM and/or proteinase inhibitor genotypes PI*ZZ, PI*SZ and compound heterozygotes or homozygotes of other rare deficient variants. We describe the characteristics of the individuals included from February 2020 to May 2022. Results: A total of 1044 individuals from 15 countries were analysed. The most frequent genotype was PI*ZZ (60.2%), followed by PI*SZ (29.2%). Among PI*ZZ patients, emphysema was the most frequent lung disease (57.2%) followed by COPD (57.2%) and bronchiectasis (22%). Up to 76.4% had concordant values of FEV1(%) and KCO(%). Those with impairment in FEV1(%) alone had more frequently bronchiectasis and asthma and those with impairment in KCO(%) alone had more frequent emphysema and liver disease. Multivariate analysis showed that advanced age, male sex, exacerbations, increased blood platelets and neutrophils, augmentation and lower AAT serum levels were associated with worse FEV1(%). Conclusions: EARCO has recruited > 1000 individuals with AATD from 15 countries in its first 2 years. Baseline cross sectional data provide relevant information about the clinical phenotypes of the disease, the patterns of functional impairment and factors associated with poor lung function. Trial registration www.
A randomised phase 2a study to investigate the effects of blocking interleukin-33 with tozorakimab in patients hospitalised with COVID-19: ACCORD-2.
(European Respiratory Society, 2023-10-02) Wilkinson, Tom; De Soyza, Anthony; Carroll, Miles; Chalmers, James D; Crooks, Michael G; Griffiths, Gareth; Shankar-Hari, Manu; Ho, Ling-Pei; Horsley, Alex; Kell, Chris; Lara, Beatriz; Mishra, Biswa; Moate, Rachel; Page, Clive; Pandya, Hitesh; Raw, Jason; Reid, Fred; Saralaya, Dinesh; Scott, Ian C; Siddiqui, Salman; Ustianowski, Andy; van Zuydam, Natalie; Woodcock, Ashley; Singh, Dave; Lara, Beatriz; Medicine; Medical and Dental; Lara, Beatriz
Efficacy analyses included 97 patients (tozorakimab+SoC, n=53; SoC, n=44). Median time to clinical response did not differ between the tozorakimab and SoC arms (8.0 and 9.5 days, respectively; HR 0.96, 80% CI 0.70-1.31; one-sided p=0.33). Tozorakimab was well tolerated and the OR for risk of death or respiratory failure with treatment versus SoC was 0.55 (80% CI 0.27-1.12; p=0.26), while the OR was 0.31 (80% CI 0.09-1.06) in patents with high baseline serum IL-33/sST2 complex levels.
Characteristics of individuals with alpha-1 antitrypsin deficiency from Northern and Southern European countries: EARCO international registry
(European Respiratory Society, 2023-03-30) Miravitlles, Marc; Turner, Alice M; Torres-Duran, María; Tanash, Hanan; Rodríguez-García, Carlota; López-Campos, José Luis; Chlumsky, Jan; Guimaraes, Catarina; Rodríguez-Hermosa, Juan Luis; Corsico, Angelo; Martinez-González, Cristina; Hernández-Pérez, José María; Bustamante, Ana; Parr, David G; Casas-Maldonado, Francisco; Hecimovic, Ana; Janssens, Wim; Lara, Beatriz; Barrecheguren, Miriam; González, Cruz; Stolk, Jan; Esquinas, Cristina; Clarenbach, Christian F; Respiratory Medicine; Medical and Dental; Turner, Alice; PARR, DAVID
The EARCO registry contains information on >1000 patients with AATD from 15 countries. The demographic characteristics and the disease characteristics are not significantly different in individuals from Northern or Southern European countries.
Predictive value of CPET in identifying patients with chronic thromboembolic pulmonary hypertension (CTEPH).
(European Respiratory Society, 2023-10-27) Shakespeare, Joanna; Burge, Jason; Moore, Vicky; Shuker, Sarah; PARR, DAVID; University Hospitals Coventry and Warwickshire NHS Trust;; Medicine; Healthcare Scientists; Medical and Dental; PARR, DAVID; Burge, Jason; Shakespeare, Joanna; Shuker, Sarah; Moore, Vicky
Early identification of chronic thromboembolic pulmonary disease/pulmonary hypertension (CTED/CTEPH) is of clinical importance. VQ imaging is reliable for detection of chronic disease but poorly distinguishes CTEPH from CTED
The utility of routine systematic respiratory assessment following initial management of acute pulmonary embolism
(European Respiratory Society, 2023-10-27) Shuker, Sarah; Helm, Emma; Bailiff, Benjamin; PARR, DAVID; bailiff; University Hospitals Coventry and Warwickshire NHS Trust; Medicine; Medical and Dental; PARR, DAVID; Shuker, Sarah; Helm, Emma; Bailiff, Benjamin
The British Lung Foundation recommends specialist review of patients with acute PE but this is not consistent UK practice, nor included in NICE guidance
Characterisation of physiological discordance in patients with idiopathic pulmonary fibrosis.
(European Respiratory Society, 2023-10-27) Shakespeare, Joanna; Choudhury, Absari; Choudhury, Absari; PARR, DAVID; University Hospitals Coventry and Warwickshire NHS Trust;; Medicine; Healthcare Scientists; Medical and Dental; PARR, DAVID; Shakespeare, Joanna; Choudhury, Absari
IPF causes lung restriction and impaired gas diffusion but these measures may be discordant. Understanding of discordant physiology and the implications for interpretation of physiology and clinical management are limited.
Utility of CPET for the investigation of persistent breathlessness in post PE patients
(European Respiratory Society, 2023-10-27) Shakespeare, Joanna; Moore, Vicky; Shuker, Sarah; Burge, Jason; PARR, DAVID; University Hospitals Coventry and Warwickshire NHS Trust;; Medicine; Clinical Diagnostic Services; Healthcare Scientists; Medical and Dental; PARR, DAVID; Shakespeare, Joanna; Burge, Jason; Moore, Vicky; Shuker, Sarah
Breathlessness persists in about 50% patients following acute PE despite anticoagulation. Chronic thromboembolic pulmonary hypertension (CTEPH) occurs in only a small proportion of cases, but early diagnosis is critically important.
Impact of COVID-19 on patients with PiZZ alpha1-antitrypsin deficiency: the IMCA1 study revisited.
(European Respiratory Society, 2023-10-27) Chorostowska-Wynimko, Joanna; Corsico, Angelo; Esquinas, Cristina; Mcelvaney, Gerard; Sark, Annelot; Sucena, Maria; Tanash, Hanan; Turner, Alice; Miravitlles, Marc; PARR, DAVID; Medicine; Medical and Dental; PARR, DAVID
IMCA1 concluded that a poor outcome in patients with an AAT level <60mg/dl related to non-respiratory comorbidities more than to markers of respiratory disease1, but deaths only occurred in PiZZ patients.
Clinical utility of alpha-1 proteinase inhibitor in the management of adult patients with severe alpha-1 antitrypsin deficiency: a review of the current literature.
(Taylor and Francis Group, 2017-07-14) PARR, DAVID; Lara, Beatriz; Medicine; Medical and Dental; Lara, Beatriz; PARR, DAVID
Alpha-1 antitrypsin (AAT) functions primarily to inhibit neutrophil elastase, and its deficiency predisposes individuals to the development of chronic obstructive pulmonary disease (COPD). The putative protective serum concentration is generally considered to be above a threshold of 11 μM/L, and therapeutic augmentation of AAT above this value is believed to retard the progression of emphysema. Several AAT preparations, all derived from human donor plasma, have been commercialized since approval by the US Food and Drug Administration (FDA) in 1987. Biochemical efficacy has been demonstrated by augmentation of pulmonary antiprotease activity, but demonstration of clinical efficacy in randomized, placebo-controlled trials has been hampered by the practical difficulties of performing conventional studies in a rare disease with a relatively long natural history. Computed tomography has been applied to measure lung density as a more specific and sensitive surrogate outcome measure of emphysema than physiologic indices, such as forced expiratory volume in 1 second, and studies consistently show a therapeutic reduction in the rate of lung density decline. However, convincing evidence of benefit using traditional clinical measures remains elusive. Intravenous administration of AAT at a dose of 60 mg/kg/week is the commonest regime in use and has well-documented safety and tolerability. International and national guidelines on the management of AAT deficiency recommend intravenous augmentation therapy to supplement optimized usual COPD treatment in patients with severe deficiency and evidence of lung function impairment.
Baricitinib in patients admitted to hospital with COVID-19 (RECOVERY): a randomised, controlled, open-label, platform trial and updated meta-analysis.
(Elsevier, 2022-07) Lara, Beatriz; Medicine; Medical and Dental; Lara, Beatriz
Background: We aimed to evaluate the use of baricitinib, a Janus kinase (JAK) 1-2 inhibitor, for the treatment of patients admitted to hospital with COVID-19.
The prevalence of bronchiectasis in patients with alpha-1 antitrypsin deficiency: initial report of EARCO.
(BMC, 2023-08-12) Stockley, Robert A; Pye, Anita; De Soyza, Joshua; Turner, Alice M; Miravitlles, Marc; PARR, DAVID; Medicine; Medical and Dental; PARR, DAVID; Lara, Beatriz
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.

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