The Extent of Inflammatory Cell Infiltrate and Fibrosis in Lungs of Telomere- and Surfactant-Related Familial Pulmonary Fibrosis

The Extent of Inflammatory Cell Infiltrate and Fibrosis in Lungs of Telomere- and Surfactant-Related Familial Pulmonary Fibrosis

Familial pulmonary fibrosis (FPF) is a monogenic disease most commonly involving telomere- (TERT) or surfactant- (SFTP) related mutations. These mutations have been shown to alter lymphocytic inflammatory responses, and FPF biopsies with histological lymphocytic infiltrates have been reported. Recen...

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Bibliographic Details
Main Authors: Aernoud A. van Batenburg, Matthijs F. M. van Oosterhout, Sebastiaan N. Knoppert, Karin M. Kazemier, Joanne J. van der Vis, Jan C. Grutters, Roel Goldschmeding, Coline H. M. van Moorsel
Format: Article
Language:English
Published: Frontiers Media S.A. 2021-09-01
Series:Frontiers in Medicine
Subjects:
inflammation
fibrosis
fibroblast foci
telomere mutations
surfactant mutations
idiopathic pulmonary fibrosis
Online Access:https://www.frontiersin.org/articles/10.3389/fmed.2021.736485/full

Internet

https://www.frontiersin.org/articles/10.3389/fmed.2021.736485/full

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