title: Interstitial Lung Disease in Werner Syndrome: A Case Report of a 55-Year-Old Male Patient.
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.
authors.affiliation: Service de Pneumologie, Hôpital Universitaire Tenon, 75020 Paris, France.
authors.forename: Tiphaine
authors.initials: T
authors.lastname: Goletto
authors.affiliation: Service de Pneumologie, Hôpital Universitaire Tenon, 75020 Paris, France.
authors.forename: Flora
authors.initials: F
authors.lastname: Crockett
authors.affiliation: Service de Dermatologie, Hôpital Universitaire Cochin, 75014 Paris, France.
authors.forename: Selim
authors.initials: S
authors.lastname: Aractingi
authors.affiliation: Service de Pneumologie, Hôpital Universitaire Tenon, 75020 Paris, France.
authors.forename: Cecile
authors.initials: C
authors.lastname: Toper
authors.affiliation: Service de Dermatologie, Hôpital Universitaire Tenon, 75020 Paris, France.
authors.forename: Patricia
authors.initials: P
authors.lastname: Senet
authors.affiliation: Service de Pneumologie, Hôpital Universitaire Tenon, 75020 Paris, France.
authors.forename: Jacques
authors.initials: J
authors.lastname: Cadranel
authors.affiliation: Service de Pneumologie, Hôpital Universitaire Tenon, 75020 Paris, France.
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Naccache