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Survival and Extrapulmonary Course of Connective Tissue Disease After Lung Transplantation

Takagishi, Troy MD*; Ostrowski, Rochella MD*; Alex, Charles MD; Rychlik, Karen MS; Pelletiere, Karen RN; Tehrani, Rodney MD*

JCR: Journal of Clinical Rheumatology: September 2012 - Volume 18 - Issue 6 - p 283–289
doi: 10.1097/RHU.0b013e3182676089
Original Articles

Background Connective tissue disease (CTD)–related lung dysfunction is a common cause of morbidity and mortality; however, few lung transplantations (LTs) are performed in this population secondary to uncertainty regarding the posttransplant survival, outcome, and management.

Objectives The objectives were to evaluate the survival and the pulmonary and extrapulmonary courses of CTD after LT.

Methods Survival outcomes of patients documented within the Organ Procurement and Transplantation Network who had undergone a LT for CTD were compared with those who underwent LT for chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF). In addition, the pulmonary and extrapulmonary courses of the CTD were evaluated after LT.

Results From 1991 to 2009, there were 284 documented LT in patients with CTD. Post-LT cumulative survival of patients with CTD was less than that for COPD through 5 years, with a difference that peaked at 1 year (72.7% vs. 83.1%, P < 0.001). When patients with CTD were compared with those with IPF, a difference was only noted at 1 year (72.7% vs. 77.7%, P = 0.049). There were no documented post-LT pulmonary recurrences of the CTD, and extrapulmonary flares of the CTD were rare (1 possible flare per 20.3 patient-years and 1 probable flare per 81.0 patient-years).

Conclusions Cumulative survival of patients with CTD who underwent LT is similar to those with IPF and slightly less than those with COPD, with an increased risk of mortality that was most prominent at 6 months after transplant followed by subsequent narrowing of the survival differences over time. Lung transplantation may be a viable therapeutic option for patients with end-stage lung dysfunction resulting from a CTD.

From the Departments of *Rheumatology, †Pulmonary/Lung Transplant, and ‡Pulmonology, Loyola University Medical Center, Louisville, KY.

This work was supported in part by Health Resources and Services Administration contract 234-2005-370011C. The content is the responsibility of the authors alone and does not necessarily reflect the views or policies of the Department of Health and Human Services, nor does mention of trade names, commercial products, or organizations imply endorsement by the US Government.

The data reported here have been supplied by the United Network for Organ Sharing as the contractor for the Organ Procurement and Transplantation Network. The interpretation and reporting of these data are the responsibility of the author(s) and in no way should be seen as an official policy of or interpretation by the Organ Procurement and Transplantation Network or the US Government.

The authors declare no conflict of interest.

Correspondence: Troy Takagishi, MD, Department of Rheumatology, Loyola University Medical Center, 3991 Dutchmans Ln, Suite 211, Louisville, KY 40207. E-mail:

© 2012 Lippincott Williams & Wilkins, Inc.