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Interstitial Lung Disease in Werner Syndrome: A Case Report of a 55-Year-Old Male Patient

Authors :
Tiphaine Goletto
Flora Crockett
Selim Aractingi
Cecile Toper
Patricia Senet
Jacques Cadranel
Jean-Marc Naccache
Source :
Case Reports in Pulmonology, Vol 2015 (2015)
Publication Year :
2015
Publisher :
Wiley, 2015.

Abstract

Werner syndrome (WS) is a progeroid or premature aging syndrome characterized by early onset of age-related pathologies and cancer. The average life expectancy of affected people is 52.8 years and tends to increase. The major causes of death are malignancy and myocardial infarction. Increased telomere attrition and decay are thought to play a causative role in the clinical and pathological manifestations of the disease. Although telomere length, with or without germline mutation, is known to be associated with interstitial lung disease, the latter is not associated with WS. To the best of our knowledge, we report the first case describing a WS patient with fatal ILD. This case suggests that older patients with WS could develop ILD. Clinical outcome of WS patients may thus be improved by counselling them regarding smoking cessation or other exposure and by proposing antifibrotic therapy.

Details

Language :
English
ISSN :
20906846 and 20906854
Volume :
2015
Database :
Directory of Open Access Journals
Journal :
Case Reports in Pulmonology
Publication Type :
Academic Journal
Accession number :
edsdoj.2eaf408d4fd045dab296c2b7b1921160
Document Type :
article
Full Text :
https://doi.org/10.1155/2015/361694