Dyspnoea with progressive “idiopathic” pulmonary fibrosis
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 in...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Wiley
2021-07-01
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| Series: | Respirology Case Reports |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/rcr2.800 |
| _version_ | 1819106361662767104 |
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| author | Chahat Puri Vignesh Harish Kyunghoon Rhee Donald F. Slack |
| author_facet | Chahat Puri Vignesh Harish Kyunghoon Rhee Donald F. Slack |
| author_sort | Chahat Puri |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-22T02:36:55Z |
| format | Article |
| id | doaj.art-80dd2e1cf5a84830b1a2b74ac7de071f |
| institution | Directory Open Access Journal |
| issn | 2051-3380 |
| language | English |
| last_indexed | 2024-12-22T02:36:55Z |
| publishDate | 2021-07-01 |
| publisher | Wiley |
| record_format | Article |
| series | Respirology Case Reports |
| spelling | doaj.art-80dd2e1cf5a84830b1a2b74ac7de071f2022-12-21T18:41:44ZengWileyRespirology Case Reports2051-33802021-07-0197n/an/a10.1002/rcr2.800Dyspnoea with progressive “idiopathic” pulmonary fibrosisChahat Puri0Vignesh Harish1Kyunghoon Rhee2Donald F. Slack3Department of Internal Medicine Greater Baltimore Medical Center Towson MD USADepartment of Internal Medicine Greater Baltimore Medical Center Towson MD USADepartment of Internal Medicine Greater Baltimore Medical Center Towson MD USADepartment of Pulmonary Medicine and Critical care Greater Baltimore Medical Center Towson MD USAAbstract 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.https://doi.org/10.1002/rcr2.800Familial pulmonary fibrosisinterstitial lung diseaseshort telomeres |
| spellingShingle | Chahat Puri Vignesh Harish Kyunghoon Rhee Donald F. Slack Dyspnoea with progressive “idiopathic” pulmonary fibrosis Respirology Case Reports Familial pulmonary fibrosis interstitial lung disease short telomeres |
| title | Dyspnoea with progressive “idiopathic” pulmonary fibrosis |
| title_full | Dyspnoea with progressive “idiopathic” pulmonary fibrosis |
| title_fullStr | Dyspnoea with progressive “idiopathic” pulmonary fibrosis |
| title_full_unstemmed | Dyspnoea with progressive “idiopathic” pulmonary fibrosis |
| title_short | Dyspnoea with progressive “idiopathic” pulmonary fibrosis |
| title_sort | dyspnoea with progressive idiopathic pulmonary fibrosis |
| topic | Familial pulmonary fibrosis interstitial lung disease short telomeres |
| url | https://doi.org/10.1002/rcr2.800 |
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