Outcomes in Systemic Sclerosis–Related Lung Disease After Lung Transplantation

• DOI: 10.1097/tp.0b013e3182845f23
• Publication Date: 2013-05-23T19:42:48Z
• Authors: 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
• Keywords: 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

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.