|Outcomes in systemic sclerosis-related lung disease after lung transplantation.|
|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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|Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't|
|Title: Transplantation Volume: 95 ISSN: 1534-6080 ISO Abbreviation: Transplantation Publication Date: 2013 Apr|
|Created Date: 2013-04-02 Completed Date: 2013-07-08 Revised Date: 2014-06-13|
Medline Journal Info:
|Nlm Unique ID: 0132144 Medline TA: Transplantation Country: United States|
|Languages: eng Pagination: 975-80 Citation Subset: IM|
|APA/MLA Format Download EndNote Download BibTex|
Bronchiolitis Obliterans / etiology
Esophageal Motility Disorders / etiology
Graft Rejection / etiology
Lung Diseases, Interstitial / etiology, surgery*
Lung Transplantation* / adverse effects, mortality
Scleroderma, Systemic / complications*, mortality
|F32 HL107003/HL/NHLBI NIH HHS; K23 HL111115/HL/NHLBI NIH HHS; K23 HL111115-01/HL/NHLBI NIH HHS|
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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