Title
Dyspnoea with progressive "idiopathic" pulmonary fibrosis
Document Type
Article
Publication Title
Respirology Case Reports
Abstract
Telomeres are repetitive nucleotide sequences that prevent chromosomal shortening in cell replication. Short telomeres have been implicated in the pathogenesis of interstitial lung disease. Patients with short telomere related pulmonary fibrosis can have computed tomography (CT) findings inconsistent with pro-typical usual interstitial pneumonia/idiopathic pulmonary fibrosis (UIP/IPF) pattern. They can have rapid progression and overall worse prognosis. Antifibrotic drugs, like pirfenidone, can be used to slow the progression of disease, but there is conflicting data in patients with Telomerase reverse transcriptase/Telomerase RNA component (TERT/TERC) mutations, hence genetic testing plays an important role in determining the therapeutic options. These patients should be referred for lung transplantation early. We present a case of rapidly progressive pulmonary fibrosis associated with short telomere.
DOI
https://doi.org/10.1002/rcr2.800
Publication Date
Summer 6-6-2021
Recommended Citation
Puri C, Harish V, Rhee K, Slack DF. Dyspnoea with progressive "idiopathic" pulmonary fibrosis. Respirol Case Rep. 2021 Jun 6;9(7):e00800. doi: 10.1002/rcr2.800. PMID: 34141436; PMCID: PMC8180387.