MUC5B rs35705950 minor allele associates with older age and better survival in idiopathic pulmonary fibrosis.

Autor: van der Vis JJ; St Antonius ILD Center of Excellence, Department of Pulmonology, St. Antonius Hospital, Nieuwegein, the Netherlands.; St Antonius ILD Center of Excellence, Department of Clinical Chemistry, St. Antonius Hospital, Nieuwegein, the Netherlands.; European Reference Network (ERN) ILD core Network center., Prasse A; European Reference Network (ERN) ILD core Network center.; Division of Pulmonology, Hannover Medical School & DZL BREATH, Hannover, Germany.; Fraunhofer Institute ITEM, Hannover, Germany., Renzoni EA; Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK.; Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK., Stock CJW; Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK.; Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK., Caliskan C; European Reference Network (ERN) ILD core Network center.; Division of Pulmonology, Hannover Medical School & DZL BREATH, Hannover, Germany., Maher TM; National Heart and Lung Institute, Imperial College London, London, UK.; Keck Medicine of University of Southern California, Los Angeles, California, USA., Bonella F; European Reference Network (ERN) ILD core Network center.; Center for Interstitial and Rare Lung Diseases, Pneumology Department, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany., Borie R; European Reference Network (ERN) ILD core Network center.; Laboratoire d'excellence INFLAMEX, Inserm U1152, Paris, France.; Service de Pneumologie A, Hôpital Bichat, Paris, France., Crestani B; European Reference Network (ERN) ILD core Network center.; Laboratoire d'excellence INFLAMEX, Inserm U1152, Paris, France.; Service de Pneumologie A, Hôpital Bichat, Paris, France., Petrek M; University Hospital Olomouc - Experimental Medicine, Olomouc, Czech Republic.; Faculty of Medicine and Dentistry Palacky University - Pathophysiology, Molecular and Translational Medicine, Olomouc, Czech Republic., Wuyts WA; European Reference Network (ERN) ILD core Network center.; Unit for Interstitial Lung Diseases, Department of Respiratory Medicine, University Hospitals, Leuven, Belgium., Wind AE; St Antonius ILD Center of Excellence, Department of Pulmonology, St. Antonius Hospital, Nieuwegein, the Netherlands.; European Reference Network (ERN) ILD core Network center., Molyneaux PL; Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK.; National Heart and Lung Institute, Imperial College London, London, UK., Grutters JC; St Antonius ILD Center of Excellence, Department of Pulmonology, St. Antonius Hospital, Nieuwegein, the Netherlands.; European Reference Network (ERN) ILD core Network center.; Division of Heart and Lungs, University Medical Center Utrecht, Utrecht, the Netherlands., van Moorsel CHM; St Antonius ILD Center of Excellence, Department of Pulmonology, St. Antonius Hospital, Nieuwegein, the Netherlands.; European Reference Network (ERN) ILD core Network center.; Division of Heart and Lungs, University Medical Center Utrecht, Utrecht, the Netherlands.
Jazyk: angličtina
Zdroj: Respirology (Carlton, Vic.) [Respirology] 2023 May; Vol. 28 (5), pp. 455-464. Date of Electronic Publication: 2022 Dec 26.
DOI: 10.1111/resp.14440
Abstrakt: Background and Objective: The minor T-allele of the MUC5B promoter polymorphism rs35705950 is strongly associated with idiopathic pulmonary fibrosis (IPF). However, conflicting results have been reported on the relationship between the MUC5B minor allele and survival and it is unknown whether a specific subgroup of IPF patients might benefit from MUC5B minor allele carriage. We investigated the association between MUC5B rs35705950, survival and patient characteristics in a real-world population of European IPF patients.
Methods: In this retrospective study, 1751 patients with IPF from 8 European centres were included. MUC5B rs35705950 genotype, demographics, clinical characteristics at diagnosis and survival data were analysed.
Results: In a multi-variate Cox proportional hazard model the MUC5B minor allele was a significant independent predictor of survival when adjusted for age, sex, high resolution computed tomography pattern, smoking behaviour and pulmonary function tests in IPF. MUC5B minor allele carriers were significantly older at diagnosis (p = 0.001). The percentage of MUC5B minor allele carriers increased significantly with age from 44% in patients aged <56 year, to 63% in patients aged >75. In IPF patients aged <56, the MUC5B minor allele was not associated with survival. In IPF patients aged ≥56, survival was significantly better for MUC5B minor allele carriers (45 months [CI: 42-49]) compared to non-carriers (29 months [CI: 26-33]; p = 4 × 10 -12 ).
Conclusion: MUC5B minor allele carriage associates with a better median transplant-free survival of 16 months in the European IPF population aged over 56 years. MUC5B genotype status might aid disease prognostication in clinical management of IPF patients.
(© 2022 Asian Pacific Society of Respirology.)
Databáze: MEDLINE
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