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
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.
SUPPLEMENTAL MATERIAL
Coming soon ....
REFERENCES (25)
CITATIONS (70)
EXTERNAL LINKS
PlumX Metrics
RECOMMENDATIONS
FAIR ASSESSMENT
Coming soon ....
JUPYTER LAB
Coming soon ....