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

Case Reports in Pulmonology
Tiphaine GolettoJean-Marc Naccache

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.

References

Jul 21, 2007·American Journal of Respiratory and Critical Care Medicine·Ivan O RosasBernadette R Gochuico
Nov 18, 2008·American Journal of Respiratory and Critical Care Medicine·Toru NyunoyaGary W Hunninghake
Dec 17, 2009·The New England Journal of Medicine·Rodrigo T Calado, Neal S Young
Nov 15, 2011·Mutation Research·Mary Armanios
Dec 4, 2012·European Respiratory Review : an Official Journal of the European Respiratory Society·Luba NalysnykDirk Esser
Jan 24, 2015·The Journal of Heart and Lung Transplantation : the Official Publication of the International Society for Heart Transplantation·Raphael BorieUNKNOWN Groupe d’Etudes et de Recherche sur les Maladies “Orphelines” Pulmonaires (GERM“O”P)

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Methods Mentioned

BETA
biopsy
bronchoalveolar lavage

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