{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T14:47:02Z","timestamp":1785422822927,"version":"3.56.0"},"reference-count":36,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2026,6,24]],"date-time":"2026-06-24T00:00:00Z","timestamp":1782259200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100016387","name":"Grifols","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100016387","id-type":"DOI","asserted-by":"crossref"}]},{"name":"CSL Behring"},{"name":"Kamada"},{"name":"Sanofi"},{"name":"pH Pharma"},{"name":"Takeda"},{"id":[{"id":"https:\/\/ror.org\/04hjbmv12","id-type":"ROR","asserted-by":"publisher"}]},{"DOI":"10.13039\/100030969","name":"Spanish Society of Pneumology and Thoracic Surgery","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100030969","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100022302","name":"Catalan Society of Pneumology","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100022302","id-type":"DOI","asserted-by":"crossref"}]},{"id":[{"id":"https:\/\/ror.org\/05dx2pr19","id-type":"ROR","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000272","name":"National Institute for Health and Care Research","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100000272","id-type":"DOI","asserted-by":"crossref"}]},{"id":[{"id":"https:\/\/ror.org\/0187kwz08","id-type":"ROR","asserted-by":"publisher"}]},{"name":"Midlands Patient Safety Research Collaboration"},{"name":"West Midlands Applied Health Research Collaboration"},{"id":[{"id":"https:\/\/ror.org\/059qgvg50","id-type":"ROR","asserted-by":"publisher"}]},{"name":"EME"},{"name":"HTA"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Biomolecules"],"abstract":"<jats:p>The interindividual phenotypic heterogeneity in Alpha-1 Antitrypsin Deficiency (AATD), despite a shared genetic etiology (the Z-allele of SERPINA1), is explained by the interaction of dual pathogenic mechanisms (gain-of-function vs. loss-of-function), additional genetic modifiers, and environmental or metabolic factors. Building on recent evidence suggesting divergent disease trajectories, we investigated whether pulmonary and hepatic impairments represent coupled manifestations or independent clinical dimensions within a large European cohort. Methods: This international multicenter study utilized the European Alpha-1 Research Collaboration (EARCO) registry (n = 1217). Pulmonary and hepatic severities were quantified using concurrent 0.0\u201310.0 composite indices. Independence was evaluated via partial Spearman correlations, multivariable multinomial regression, and geometric mapping across a continuous phenotypic space. Results: Cross-domain correlations between respiratory metrics and liver stiffness were near zero (r = \u22120.03), demonstrating statistical independence. Phenotypic dominance classification isolated distinct profiles; the lung-dominant group exhibited a higher age (57.0 vs. 54.0 years; p &lt; 0.001) and tobacco exposure, while the liver-dominant group registered a higher body mass index (25.8 vs. 24.4 kg\/m2; p &lt; 0.001). Multivariable models identified age (OR 1.03; 95% CI 1.02\u20131.05) and smoking as independent predictors of lung dominance, whereas body mass index was independently associated with liver dominance (OR 1.04; 95% CI 1.01\u20131.07). Geometric mapping revealed advanced disease clusters at orthogonal margins rather than forming a systemic continuum. Conclusions: Hepatic and pulmonary impairments in AATD operate as independent clinical dimensions modulated by distinct metabolic and environmental factors. Risk stratification must transition toward organ-specific prognostic models.<\/jats:p>","DOI":"10.3390\/biom16070940","type":"journal-article","created":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T03:06:34Z","timestamp":1782875194000},"page":"940","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Dissociation of the Hepatic and Pulmonary Axes in Alpha-1 Antitrypsin Deficiency: Independent Trajectories of Organ-Specific Disease"],"prefix":"10.3390","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0552-2484","authenticated-orcid":false,"given":"Juan Luis","family":"Rodr\u00edguez Hermosa","sequence":"first","affiliation":[{"name":"Pulmonology Department, Hospital Cl\u00ednico San Carlos, 28040 Madrid, Spain"},{"name":"Instituto de Investigaci\u00f3n Sanitaria del Hospital Cl\u00ednico San Carlos (IdISSC), 28040 Madrid, Spain"},{"name":"Department of Medicine, School of Medicine, Universidad Antonio Nebrija, 28248 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-3984-3557","authenticated-orcid":false,"given":"Soha","family":"Esmaili","sequence":"additional","affiliation":[{"name":"Instituto de Investigaci\u00f3n Sanitaria del Hospital Cl\u00ednico San Carlos (IdISSC), 28040 Madrid, Spain"},{"name":"Department of Medicine, School of Medicine, Universidad Antonio Nebrija, 28248 Madrid, Spain"},{"name":"Pulmonology Department, Hospital Universitario La Zarzuela and Hospital Quir\u00f3nsalud San Jose, 28023 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-5089-664X","authenticated-orcid":false,"given":"Iman","family":"Esmaili","sequence":"additional","affiliation":[{"name":"ISNS Data Analytics and Research, Vancouver, BC V6Z 1Y6, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Torres-Duran","sequence":"additional","affiliation":[{"name":"NeumoVigo I+i Research Group, Pneumology Department, Hospital \u00c1lvaro Cunqueiro, IIS Galicia Sur, 36211 Vigo, Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, 28029 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanan","family":"Tanash","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine and Allergology, Sk\u00e5ne University Hospital, Lund University, 214 28 Malm\u00f6, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5947-3254","authenticated-orcid":false,"given":"Alice M.","family":"Turner","sequence":"additional","affiliation":[{"name":"Respiratory Medicine, University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2GW, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlota","family":"Rodr\u00edguez-Garc\u00eda","sequence":"additional","affiliation":[{"name":"Pneumology Department, Complejo Hospitalario Universitario de Ferrol, 15405 El Ferrol, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miriam","family":"Barrecheguren","sequence":"additional","affiliation":[{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, 28029 Madrid, Spain"},{"name":"Department of Pneumology, Universitary Hospital Vall d\u2019Hebron\/Vall d\u2019Hebron Institut de Recerca (VHIR), Vall d\u2019Hebron Barcelona Hospital Campus, and European Reference Network on Rare Respiratory Diseases (ERN LUNG), 08035 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4036-0521","authenticated-orcid":false,"given":"Jens-Ulrik","family":"St\u00e6hr Jensen","sequence":"additional","affiliation":[{"name":"Section of Respiratory Medicine, Department of Medicine, Herlev and Gentofte Hospital, University of Copenhagen, 2830 Gentofte, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Bunel","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, Allergology, and Transplantation, AP-HP, Bichat Hospital, Paris Cit\u00e9 University, Inserm, Centre de Recherche de l\u2019Inflammation, F-75018 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8716-4694","authenticated-orcid":false,"given":"Angelo Guido","family":"Corsico","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Therapeutics, University of Pavia, 27100, Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenneth R.","family":"Chapman","sequence":"additional","affiliation":[{"name":"Division of Respiratory Medicine, Department of Medicine, University of Toronto, Toronto, ON M5T 2S8, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Fran\u00e7ois","family":"Mornex","sequence":"additional","affiliation":[{"name":"Hospices Civils de Lyon, OrphaLung, Respifil and CHU de Saint Etienne, F-69000 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eva","family":"Barto\u0161ovsk\u00e1-Klinkov\u00e1","sequence":"additional","affiliation":[{"name":"Department of Pneumology, Thomayer Hospital, First Faculty of Medicine, Charles University, CZ-121 08 Prague, Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0090-8206","authenticated-orcid":false,"given":"Beatriz","family":"Lara","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, University Hospitals Coventry and Warwickshire NHS Trust, Coventry CV2 2DX, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jos\u00e9 Luis","family":"L\u00f3pez-Campos","sequence":"additional","affiliation":[{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, 28029 Madrid, Spain"},{"name":"Unidad M\u00e9dico-Quir\u00fargica de Enfermedades Respiratorias, Instituto de Biomedicina de Sevilla (IBiS), Hospital Universitario Virgen del Roc\u00edo, Universidad de Sevilla, 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2158-2321","authenticated-orcid":false,"given":"Christian F.","family":"Clarenbach","sequence":"additional","affiliation":[{"name":"Pulmonary Clinic, University Hospital Zurich, University of Zurich, 8091 Zurich, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-6299-9943","authenticated-orcid":false,"given":"Emily F. A.","family":"van \u2019t Wout","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, Leiden University Medical Centre, 2333 ZA Leiden, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariano","family":"Fernandez-Acquier","sequence":"additional","affiliation":[{"name":"Hospital Zonal Especializado de Agudos y Cr\u00f3nicos Dr. Antonio Cetr\u00e1ngolo, Vicente L\u00f3pez, Buenos Aires 1802, Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3890-2742","authenticated-orcid":false,"given":"Myriam","family":"Calle Rubio","sequence":"additional","affiliation":[{"name":"Pulmonology Department, Hospital Cl\u00ednico San Carlos, 28040 Madrid, Spain"},{"name":"Instituto de Investigaci\u00f3n Sanitaria del Hospital Cl\u00ednico San Carlos (IdISSC), 28040 Madrid, Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, 28029 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,6,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2225","DOI":"10.1016\/S0140-6736(05)66781-5","article-title":"Alpha1-antitrypsin deficiency","volume":"365","author":"Stoller","year":"2005","journal-title":"Lancet"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1016\/j.cld.2022.03.004","article-title":"Alpha-1 antitrypsin deficiency liver disease","volume":"26","author":"Suri","year":"2022","journal-title":"Clin. 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Liver Transplant."}],"container-title":["Biomolecules"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2218-273X\/16\/7\/940\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,3]],"date-time":"2026-07-03T04:40:15Z","timestamp":1783053615000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2218-273X\/16\/7\/940"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6,24]]},"references-count":36,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2026,7]]}},"alternative-id":["biom16070940"],"URL":"https:\/\/doi.org\/10.3390\/biom16070940","relation":{},"ISSN":["2218-273X"],"issn-type":[{"value":"2218-273X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6,24]]}}}