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Lung transplantation (LTX) is the last life-extending option for selected patients with progressive fibrosing interstitial lung diseases (ILD). In around 30% (range 11 to 71%) of patients with severe ILD this concerns a hereditary form of pulmonary fibrosis (HPF) based on an underlying disease-associated genetic mutation (GM) and/or telomere shortening (TS) syndrome, and/or clustering in the family indicated as familial pulmonary fibrosis (FPF). HPF is associated with on average early onset, faster progression, and worse prognosis causing the HPF group to be referred to LTX relatively more often. Regarding outcomes of LTX in these patients there are contradictory reports on patient and graft survival, as well as numerous other outcomes. There is currently no consensus on whether HPF is associated with poorer outcomes after LTX and what considerations regarding candidacy are appropriate. We aimed to systematically review LTX outcomes of patients with HPF in comparison to those with non HPF-ILD. A systematic literature search was performed PubMed, Embase, MEDLINE, Web of Science, Cochrane Library, and Google Scholar for articles (01 June 2022). We included studies on LTX in ILD with and without underlying HPF and extracted separate outcome data on primary and secondary outcome parameters. Fourteen studies met the inclusion criteria (10 cohort studies and 4 case-series) including 1023 patients that underwent LTX, 297 for HPF and 726 for non HPF-ILD. There was large heterogeneity in methodological study quality and reported outcomes. Whereas a limited number of high quality studies report higher rate of ACR, CLAD and shorter survival in HPF, this could not be confirmed by an at least equal number of high quality studies. Specific secondary outcomes such as cytopenias, CMV reactivation and bronchial complications may be more prevalent in HPF, but there are insufficient studies for definitive conclusions. Due to large heterogeneity in methodological study quality and reported outcomes, no firm conclusions can be drawn. LTX in patients with HPF that are accepted for LTX does not unambiguously seem associated with poorer short and mid-term patient and graft outcomes, and thus this suggests that HPF in itself should not preclude from undergoing LTX. Nonetheless, differences may exist, especially regarding other transplant related outcomes. [ABSTRACT FROM AUTHOR]