Document Detail

Outcomes in systemic sclerosis-related lung disease after lung transplantation.
MedLine Citation:
PMID:  23545509     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Lung disease is the leading cause of death in systemic sclerosis (SSc). The diagnosis of SSc-related lung disease (SSc-LD) is often a contraindication to lung transplantation (LT) due to concerns that extrapulmonary involvement will yield worse outcomes. We sought to evaluate posttransplantation outcomes in persons with SSc-LD with esophageal involvement compared with persons with nonconnective tissue disease-related interstitial lung disease (nCTD-ILD).
METHODS: From 1998 to 2012, persons undergoing LT for SSc-LD were age and gender matched in a 2:1 fashion to controls undergoing LT for nCTD-ILD. Esophageal function was assessed by pH testing and manometry. We defined esophageal dysfunction as the presence of a DeMeester score >14 or dysmotility more severe than "mild nonspecific disorder". The primary outcome was posttransplantation survival. Secondary outcomes included freedom from bronchiolitis obliterans syndrome (fBOS) and rates of acute rejection. Survival and fBOS were estimated with Kaplan-Meier methods. Acute rejection was compared with Student's t test.
RESULTS: Survival was similar in 23 persons with SSc-LD and 46 controls who underwent LT (P = 0.47). For the SSc-LD group, 1- and 5-year survival was 83% and 76% compared with 91% and 64% in the nCTD-ILD group, respectively. There were no differences in fBOS (P = 0.83). Rates of acute rejection were less in SSc-ILD (P = 0.05). Esophageal dysfunction was not associated with worse outcomes (P>0.55).
CONCLUSIONS: Persons with SSc-LD appear to have similar survival and fBOS as persons transplanted for nCTD-ILD. The risk of acute rejection after transplantation may be reduced in persons with SSc-LD. Esophageal involvement does not appear to impact outcomes.
Peter D Sottile; David Iturbe; Tamiko R Katsumoto; M Kari Connolly; Harold R Collard; Lorriana A Leard; Steven Hays; Jeffrey A Golden; Charles Hoopes; Jasleen Kukreja; Jonathan P Singer
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Transplantation     Volume:  95     ISSN:  1534-6080     ISO Abbreviation:  Transplantation     Publication Date:  2013 Apr 
Date Detail:
Created Date:  2013-04-02     Completed Date:  2013-07-08     Revised Date:  2014-10-24    
Medline Journal Info:
Nlm Unique ID:  0132144     Medline TA:  Transplantation     Country:  United States    
Other Details:
Languages:  eng     Pagination:  975-80     Citation Subset:  IM    
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MeSH Terms
Acute Disease
Bronchiolitis Obliterans / etiology
Chi-Square Distribution
Esophageal Motility Disorders / etiology
Graft Rejection / etiology
Kaplan-Meier Estimate
Logistic Models
Lung Diseases, Interstitial / etiology,  surgery*
Lung Transplantation* / adverse effects,  mortality
Middle Aged
Multivariate Analysis
Retrospective Studies
Risk Assessment
Risk Factors
San Francisco
Scleroderma, Systemic / complications*,  mortality
Time Factors
Treatment Outcome
Grant Support

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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