Outcomes in Systemic Sclerosis–Related Lung Disease After Lung Transplantation

Abstract

Background Lung disease (LD) is the leading cause of death in systemic sclerosis (SSc). The diagnosis of SSc-related LD (SSc-LD) is often a contraindication to lung transplantation (LT) due to concerns that extra-pulmonary involvement will yield worse outcomes. We sought to evaluate post-transplant outcomes in persons with SSc-LD with esophageal involvement compared to persons with non-connective tissue disease related interstitial lung disease (nCTD-ILD). Methods From 1998-2012, 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 non-specific disorder”. The primary outcome was post-transplant 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 Students 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 to 91% and 64% in the nCTD-ILD group. 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 transplant may be reduced in persons with SSc-LD. Esophageal involvement does not appear to impact outcomes.

The Intersection of GERD, Aspiration, and Lung Transplantation

“…Given the specific concerns regarding transplantation in the setting of GERD, Sottile et al examined the outcome of lung transplants in patients with documented reflux as defined by a DeMeester score >14.7. 29 In comparison to a matched cohort of IPF patients, there were no differences in survival at 1 year (83% versus 91%) and 5 years (76% versus 64%; P = .47). Esophageal dysfunction was not associated with a difference in BOS or an increased mortality following transplantation.…”

“…Today, pulmonary disease is the main organ-specific cause of death in the scleroderma patient population, with 3-year mortality exceeding 50%. 29 The main manifestations of lung disease are interstitial fibrosis (50% of patients) and pulmonary arterial hypertension (5%-12%). 30 Because medical therapy has limited efficacy in scleroderma lung disease, lung transplantation is the only definitive treatment option for patients with scleroderma lung disease.…”

Lung Transplantation for Hypersensitivity Pneumonitis

“…As reported previously, 20 all patients at the University of California San Francisco facility are maintained on an immunosuppression regimen of prednisone, tacrolimus, and mycophenolate mofetil and undergo allograft surveillance (spirometry, chest CT scan, and bronchoscopy with transbronchial biopsy) to evaluate acute rejection eight times in the fi rst 24 postoperative months ( e-Appendix 1 ).…”

A Case Report of Living-donor Lobar Lung Transplantation for Scleroderma-associated Usual Interstitial Pneumonia: Eight Years and Counting

“…Sclerodermaassociated pulmonary hypertension and interstitial lung disease are common life-limiting complications of scleroderma, and often precede lung transplantation [2]. Esophageal manifestations of scleroderma include frequent symptoms of gastroesophageal reflux, often associated with a hypotensive lower esophageal sphincter (LES), and an absence of peristalsis in the mid-distal esophageal body [3].…”

“…Esophageal dysmotility and gastroesophageal reflux are frequently found in patients listed for lung transplantation even in the absence of systemic sclerosis [6e9]. Current literature reports good outcomes with deceased-donor lung transplantation in those with scleroderma and preserved critical organ function [2,10,11].…”