Outcomes in Systemic Sclerosis–Related Lung Disease After Lung Transplantation
Lung Diseases
Graft Rejection
Male
Time Factors
Survival
Clinical sciences
Kaplan-Meier Estimate
Medical and Health Sciences
Scleroderma
0302 clinical medicine
Risk Factors
2.1 Biological and endogenous factors
Aetiology
Lung
Bronchiolitis Obliterans
Middle Aged
Esophageal dysmotility
3. Good health
Lung transplantation
Treatment Outcome
Acute Disease
Respiratory
Systemic sclerosis
Female
Lung Transplantation
Adult
Clinical Sciences
Immunology
Interstitial lung disease
Autoimmune Disease
Risk Assessment
03 medical and health sciences
Rare Diseases
Clinical Research
Humans
Esophageal Motility Disorders
Aged
Retrospective Studies
Transplantation
Chi-Square Distribution
Biomedical and Clinical Sciences
Inflammatory and immune system
Bronchiolitis obliterans syndrome
Systemic
Organ Transplantation
Logistic Models
Multivariate Analysis
Surgery
San Francisco
Digestive Diseases
Interstitial
Lung Diseases, Interstitial
DOI:
10.1097/tp.0b013e3182845f23
Publication Date:
2013-05-23T19:42:48Z
AUTHORS (11)
ABSTRACT
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).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.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).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.
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