Lung transplantation in telomerase mutation carriers with pulmonary fibrosis

Silhan, Leann L., Shah, Pali D., Chambers, Daniel C., Snyder, Laurie D., Riise, Gerdt C., Wagner, Christa L., Hellstrom-Lindberg, Eva, Orens, Jonathan B., Mewton, Juliette F., Danoff, Sonye K., Arcasoy, Murat O. and Armanios, Mary (2014) Lung transplantation in telomerase mutation carriers with pulmonary fibrosis. European Respiratory Journal, 44 1: 178-187. doi:10.1183/09031936.00060014

Author Silhan, Leann L.
Shah, Pali D.
Chambers, Daniel C.
Snyder, Laurie D.
Riise, Gerdt C.
Wagner, Christa L.
Hellstrom-Lindberg, Eva
Orens, Jonathan B.
Mewton, Juliette F.
Danoff, Sonye K.
Arcasoy, Murat O.
Armanios, Mary
Title Lung transplantation in telomerase mutation carriers with pulmonary fibrosis
Journal name European Respiratory Journal   Check publisher's open access policy
ISSN 1399-3003
Publication date 2014-07-01
Year available 2014
Sub-type Article (original research)
DOI 10.1183/09031936.00060014
Open Access Status DOI
Volume 44
Issue 1
Start page 178
End page 187
Total pages 10
Place of publication Lausanne, Switzerland
Publisher European Respiratory Society
Language eng
Abstract 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 USA, 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.
Keyword Lung transplantation
Idiopathic pulmonary fibrosis
Q-Index Code C1
Q-Index Status Confirmed Code
Grant ID P30 CA006973
Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
Collections: Official 2015 Collection
School of Medicine Publications
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