Outcomes of lung transplantation in patients with  telomere-related forms of progressive fibrosing  interstitial lung disease pulmonary fibrosis:  A systematic review

Merel Hellemons; Bas Mathot; Leonard Seghers; Marlies Lourens

doi:10.1016/j.jhlto.2024.100054

Outcomes of lung transplantation in patients with telomere-related forms of progressive fibrosing interstitial lung disease pulmonary fibrosis: A systematic review

Merel Hellemons^*, Bas Mathot, Leonard Seghers, Marlies Lourens

^*Corresponding author for this work

Pulmonary Medicine

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Abstract

BACKGROUND: Lung transplantation (LTX) is the last life-extending option for patients with progressive fibrosing interstitial lung diseases (fILD). Between 12% and 71% of patients with fILD are patients with underlying telomere-dysfunction (trILD) related to pathogenic telomere-related gene (TRG) variants and/or short telomere length. TrILD patients tend to have earlier disease onset, faster progression, and worse prognosis causing them to be referred for LTX more often. Regarding LTX outcomes in trILD, there are contradictory reports on patient and graft survival, as well as numerous other outcomes. There is no consensus on whether trILD is associated ith poorer outcomes after LTX and what considerations regarding candidacy are appropriate.
METHODS: We aimed to systematically review LTX outcomes of patients with trILD in comparison to those with non-trILD.
RESULTS: A systematic literature search yielded 13 studies that met the inclusion criteria including
933 LTX, 281 in trILD, and 652 in non-trILD. Despite large heterogeneity in the methodological study

Original language	English
Article number	100054
Journal	Journal of Heart and Lung Transplantation
Volume	3
DOIs	https://doi.org/10.1016/j.jhlto.2024.100054
Publication status	Published - Feb 2024

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10.1016/j.jhlto.2024.100054Licence: CC BY-NC-ND

PIIS295013342400003XFinal published version, 2.01 MBLicence: CC BY-NC-ND

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@article{37aabaa4e8874431926eec8a0ea2866d,

title = "Outcomes of lung transplantation in patients with telomere-related forms of progressive fibrosing interstitial lung disease pulmonary fibrosis: A systematic review",

abstract = "BACKGROUND: Lung transplantation (LTX) is the last life-extending option for patients with progressive fibrosing interstitial lung diseases (fILD). Between 12% and 71% of patients with fILD are patients with underlying telomere-dysfunction (trILD) related to pathogenic telomere-related gene (TRG) variants and/or short telomere length. TrILD patients tend to have earlier disease onset, faster progression, and worse prognosis causing them to be referred for LTX more often. Regarding LTX outcomes in trILD, there are contradictory reports on patient and graft survival, as well as numerous other outcomes. There is no consensus on whether trILD is associated ith poorer outcomes after LTX and what considerations regarding candidacy are appropriate.METHODS: We aimed to systematically review LTX outcomes of patients with trILD in comparison to those with non-trILD. RESULTS: A systematic literature search yielded 13 studies that met the inclusion criteria including933 LTX, 281 in trILD, and 652 in non-trILD. Despite large heterogeneity in the methodological study",

author = "Merel Hellemons and Bas Mathot and Leonard Seghers and Marlies Lourens",

note = "{\textcopyright} 2024 The Authors. Published by Elsevier Inc. on behalf of International Society for Heart and Lung Transplantation.",

year = "2024",

month = feb,

doi = "10.1016/j.jhlto.2024.100054",

language = "English",

volume = "3",

journal = "Journal of Heart and Lung Transplantation",

issn = "1053-2498",

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Outcomes of lung transplantation in patients with telomere-related forms of progressive fibrosing interstitial lung disease pulmonary fibrosis: A systematic review. / Hellemons, Merel ; Mathot, Bas ; Seghers, Leonard et al.
In: Journal of Heart and Lung Transplantation, Vol. 3, 100054, 02.2024.

Research output: Contribution to journal › Article › Academic › peer-review

TY - JOUR

T1 - Outcomes of lung transplantation in patients with telomere-related forms of progressive fibrosing interstitial lung disease pulmonary fibrosis

T2 - A systematic review

AU - Hellemons, Merel

AU - Mathot, Bas

AU - Seghers, Leonard

AU - Lourens, Marlies

PY - 2024/2

Y1 - 2024/2

N2 - BACKGROUND: Lung transplantation (LTX) is the last life-extending option for patients with progressive fibrosing interstitial lung diseases (fILD). Between 12% and 71% of patients with fILD are patients with underlying telomere-dysfunction (trILD) related to pathogenic telomere-related gene (TRG) variants and/or short telomere length. TrILD patients tend to have earlier disease onset, faster progression, and worse prognosis causing them to be referred for LTX more often. Regarding LTX outcomes in trILD, there are contradictory reports on patient and graft survival, as well as numerous other outcomes. There is no consensus on whether trILD is associated ith poorer outcomes after LTX and what considerations regarding candidacy are appropriate.METHODS: We aimed to systematically review LTX outcomes of patients with trILD in comparison to those with non-trILD. RESULTS: A systematic literature search yielded 13 studies that met the inclusion criteria including933 LTX, 281 in trILD, and 652 in non-trILD. Despite large heterogeneity in the methodological study

AB - BACKGROUND: Lung transplantation (LTX) is the last life-extending option for patients with progressive fibrosing interstitial lung diseases (fILD). Between 12% and 71% of patients with fILD are patients with underlying telomere-dysfunction (trILD) related to pathogenic telomere-related gene (TRG) variants and/or short telomere length. TrILD patients tend to have earlier disease onset, faster progression, and worse prognosis causing them to be referred for LTX more often. Regarding LTX outcomes in trILD, there are contradictory reports on patient and graft survival, as well as numerous other outcomes. There is no consensus on whether trILD is associated ith poorer outcomes after LTX and what considerations regarding candidacy are appropriate.METHODS: We aimed to systematically review LTX outcomes of patients with trILD in comparison to those with non-trILD. RESULTS: A systematic literature search yielded 13 studies that met the inclusion criteria including933 LTX, 281 in trILD, and 652 in non-trILD. Despite large heterogeneity in the methodological study

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DO - 10.1016/j.jhlto.2024.100054

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SN - 1053-2498

VL - 3

JO - Journal of Heart and Lung Transplantation

JF - Journal of Heart and Lung Transplantation

M1 - 100054

ER -

Outcomes of lung transplantation in patients with telomere-related forms of progressive fibrosing interstitial lung disease pulmonary fibrosis: A systematic review

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