Evidence map›Paper›PMID 41668877›Full record

ArticleERJ open research2026

Characteristics of bronchiectasis in patients with different genotypes of severe α

Francesca Mandurino Mirizzi, Cristina Aljama, Pierachille Santus, Marco Mantero, Maja Omcikus, María Torres-Duran, Alice M Turner, Hanan Tanash, Carlota Rodríguez-García, Jens-Ulrik Stæhr Jensen and 10 more

Abstract read
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In one paragraph

Article in ERJ open research, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Bronchiectasis in severe αERJ open research · 2026
    Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

20 authors.

Francesca Mandurino MirizziDepartment of Biomedical and Clinical Sciences (DIBIC), Università degli Studi di Milano, Division of Respiratory Diseases, Ospedale L. Sacco, ASST Fatebenefratelli-Sacco, Milan, Italy.
Cristina AljamaDepartment of Pneumology, Hospital Universitari Vall d'Hebron/Vall d'Hebron Institut de Recerca, Health Care Provider of the European Reference Network on Rare Respiratory Diseases (ERN LUNG), Barcelona, Spain.
Pierachille SantusDepartment of Biomedical and Clinical Sciences (DIBIC), Università degli Studi di Milano, Division of Respiratory Diseases, Ospedale L. Sacco, ASST Fatebenefratelli-Sacco, Milan, Italy.
Marco ManteroFondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Respiratory Disease and Adult Cystic Fibrosis Center, University of Milan, Department of Pathophysiology and Transplantation, Milan, Italy.
Maja OmcikusClinic for Pulmonary Diseases, University Clinical Center of Serbia, Belgrade and Medical School University of Belgrade, Belgrade, Serbia.
María Torres-DuranServicio de Neumología, Hospital Álvaro Cunqueiro, NeumoVigo I+i Research Group, IIS Galicia Sur, Vigo, Spain.ORCID https://orcid.org/0000-0001-6710-3766
Alice M TurnerRespiratory Medicine, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.ORCID https://orcid.org/0000-0002-5947-3254
Hanan TanashDepartment of Respiratory Medicine and Allergology, Skåne University Hospital, Lund University, Malmö, Sweden.
Carlota Rodríguez-GarcíaServicio de Neumología, Complejo Hospitalario Clínico-Universitario de Santiago, Santiago de Compostela, Spain.
Jens-Ulrik Stæhr JensenSection of Respiratory Medicine, Department of Medicine, Herlev and Gentofte Hospital, University of Copenhagen, Hellerup, Denmark.ORCID https://orcid.org/0000-0003-4036-0521
Angelo Guido CorsicoDepartment of Internal Medicine and Medical Therapeutics, University of Pavia, Pavia, Italy.
José Luis López-CamposCentro de Investigación Biomédica en Red de Enfermedades Respiratorias, Instituto de Salud Carlos III, Madrid, Spain.ORCID https://orcid.org/0000-0003-1703-1367
Kenneth R ChapmanAsthma and Airway Center, University Health Network, Toronto Western Hospital, and Division of Respiratory Medicine, Department of Medicine, University of Toronto, Toronto, ON, Canada.ORCID https://orcid.org/0000-0002-2498-9859
Christian ClarenbachDivision of Pulmonology, University Hospital Zurich, Zurich, Switzerland.ORCID https://orcid.org/0000-0003-2158-2321
Catarina GuimaraesPulmonology Department, Hospital da Senhora da Oliveira, Unidade Local de Saúde Alto Ave, Guimarães, Portugal.
Eva BartošovskáDepartment of Pneumology, Thomayer Hospital, First Faculty of Medicine, Charles University, Prague, Czech Republic.
José María Hernández-PérezPneumology Department, Hospital Universitario Nuestra Señora de La Candelaria, Santa Cruz de Tenerife, Spain.ORCID https://orcid.org/0000-0002-2920-212X
Marc MiravitllesDepartment of Pneumology, Hospital Universitari Vall d'Hebron/Vall d'Hebron Institut de Recerca, Health Care Provider of the European Reference Network on Rare Respiratory Diseases (ERN LUNG), Barcelona, Spain.ORCID https://orcid.org/0000-0002-9850-9520
Cristina EsquinasDepartment of Public Health, Mental Health and Maternal and Child Health Nursing, Faculty of Nursing, University of Barcelona, Barcelona, Spain.
Miriam BarrechegurenDepartment of Pneumology, Hospital Universitari Vall d'Hebron/Vall d'Hebron Institut de Recerca, Health Care Provider of the European Reference Network on Rare Respiratory Diseases (ERN LUNG), Barcelona, Spain.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Background: α-1 antitrypsin deficiency (AATD) is a rare genetic disorder caused by mutations in the Methods: This cross-sectional study analysed data from the European α-1 Research Collaboration (EARCO) registry, focusing on individuals with bronchiectasis on computed tomography (CT). Participants were stratified by AATD genotypes (Pi*ZZ Results: 349 patients had bronchiectasis on a CT scan, of whom 70.5% had Pi*ZZ, 18.6% had Pi*SZ, 4.3% had Pi*SS and 6.6% had rare variants. Lower lobe involvement was predominant across genotypes, whereas Pi*SS exhibited distinct upper lobe patterns and Pi*SZ showed more frequent middle lobe involvement. People with rare genotypes and Pi*ZZ had worse lung function (FEV Conclusion: Bronchiectasis in AATD is not limited to the Pi*ZZ genotype, with significant phenotypic variability across genotypes. Lower lobe involvement and mild disease predominate; however, severe forms are more frequent in rare genotypes and Pi*ZZ. These findings underscore the importance of systematic screening and genotype-specific management to improve patient outcomes.

Identifiers

PMID41668877
PMCPMC12884379

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