Lung transplantation in telomerase mutation carriers with pulmonary fibrosis. Eur Respir J 2014 Jul;44(1):178-87
Date
05/17/2014Pubmed ID
24833766Pubmed Central ID
PMC4076528DOI
10.1183/09031936.00060014Scopus ID
2-s2.0-84903834962 (requires institutional sign-in at Scopus site) 146 CitationsAbstract
Lung transplantation is the only intervention that prolongs survival in idiopathic pulmonary fibrosis (IPF). Telomerase mutations are the most common identifiable genetic cause of IPF, and at times, the telomere defect manifests in extrapulmonary disease such as bone marrow failure. The relevance of this genetic diagnosis for lung transplant management has not been examined. We gathered an international series of telomerase mutation carriers who underwent lung transplant in the U.S.A., Australia and Sweden. The median age at transplant was 52 years. Seven recipients are alive with a median follow-up of 1.9 years (range 6 months to 9 years); one died at 10 months. The most common complications were haematological, with recipients requiring platelet transfusion support (88%) and adjustment of immunosuppressives (100%). Four recipients (50%) required dialysis for tubular injury and calcineurin inhibitor toxicity. These complications occurred at significantly higher rates relative to historic series (p<0.0001). Our observations support the feasibility of lung transplantation in telomerase mutation carriers; however, severe post-transplant complications reflecting the syndromic nature of their disease appear to occur at higher rates. While these findings need to be expanded to other cohorts, caution should be exercised when approaching the transplant evaluation and management of this subset of pulmonary fibrosis patients.
Author List
Silhan LL, Shah PD, Chambers DC, Snyder LD, Riise GC, Wagner CL, Hellström-Lindberg E, Orens JB, Mewton JF, Danoff SK, Arcasoy MO, Armanios MAuthor
Christa Wagner PhD Director, Assistant Professor in the Institute for Health and Equity department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AdultAustralia
Cohort Studies
Female
Humans
Idiopathic Pulmonary Fibrosis
Lung Transplantation
Male
Middle Aged
Mutation
Postoperative Complications
Renal Insufficiency
Sweden
Telomerase
Treatment Outcome
United States
