RT Journal Article SR Electronic T1 Management of suspected monogenic lung fibrosis in a specialised centre JF European Respiratory Review JO EUROPEAN RESPIRATORY REVIEW FD European Respiratory Society SP 160122 DO 10.1183/16000617.0122-2016 VO 26 IS 144 A1 Borie, Raphael A1 Kannengiesser, Caroline A1 Sicre de Fontbrune, Flore A1 Gouya, Laurent A1 Nathan, Nadia A1 Crestani, Bruno YR 2017 UL http://err.ersjournals.com/content/26/144/160122.abstract AB At least 10% of patients with interstitial lung disease present monogenic lung fibrosis suspected on familial aggregation of pulmonary fibrosis, specific syndromes or early age of diagnosis. Approximately 25% of families have an identified mutation in genes mostly involved in telomere homeostasis, and more rarely in surfactant homeostasis.Beyond pathophysiological knowledge, detection of these mutations has practical consequence for patients. For instance, mutations involved in telomere homeostasis are associated with haematological complications after lung transplantation and may require adapted immunosuppression. Moreover, relatives may benefit from a clinical and genetic evaluation that should be specifically managed.The field of genetics of pulmonary fibrosis has made great progress in the last 10 years, raising specific problems that should be addressed by a specialised team.Monogenic pulmonary fibrosis raises specific problems that should be addressed by a specialised team http://ow.ly/YJUM30aoREG