title: A case report of living-donor lobar lung transplantation for scleroderma-associated usual interstitial pneumonia: eight years and counting.
abstract: BACKGROUND
  Scleroderma-associated interstitial lung disease is a life-limiting complication of scleroderma, often requiring lung transplantation. Living-donor lobar lung transplantation (LDLLT) is a viable alternative to deceased-donor lung transplantation in specialized centers under select circumstances.
  
  
  METHODS
  A 47-year-old female underwent LDLLT after nine years of symptomatic scleroderma-associated usual interstitial pneumonia and three years awaiting deceased-donor lung transplantation. Her manifestations of scleroderma included mild sclerodactyly, periungual erythema, Raynaud's phenomenon, and gastroesophageal reflux, with positive antinuclear autoantibodies. Several years post-transplantation, manometry revealed feeble lower esophageal sphincteric pressure with ineffective esophageal motility. Bronchiolitis obliterans syndrome developed 64 months post-transplantation without evidence of aspiration or reflux on transbronchial biopsy. Currently, she has normal renal function and good allograft function [FEV1 1.52 L (73% predicted) and FVC 2.50 L (99% predicted)].
  
  
  CONCLUSIONS
  This is the second reported case of LDLLT in scleroderma, and the first reporting long-term pulmonary, renal, and esophageal function post-transplantation.
authors: Department of Medicine, University of Alberta, Edmonton, Alberta, Canada.
authors: C
authors: C
authors: Laratta
authors: Lung Transplant Program, University of Alberta, Edmonton, Alberta, Canada; Division of Respirology, University of Alberta, Edmonton, Alberta, Canada.
authors: D
authors: D
authors: Lien
authors: Department of Pathology, University of Alberta, Edmonton, Alberta, Canada.
authors: L
authors: L
authors: Puttagunta
authors: Lung Transplant Program, University of Alberta, Edmonton, Alberta, Canada.
authors: K
authors: K
authors: Jackson
authors: Lung Transplant Program, University of Alberta, Edmonton, Alberta, Canada; Department of Cardiothoracic Surgery, University of Alberta, Edmonton, Alberta, Canada.
authors: J
authors: J
authors: Mullen
authors: Lung Transplant Program, University of Alberta, Edmonton, Alberta, Canada; Division of Respirology, University of Alberta, Edmonton, Alberta, Canada.
authors: A
authors: A
authors: Kapasi
authors: Lung Transplant Program, University of Alberta, Edmonton, Alberta, Canada; Division of Respirology, University of Alberta, Edmonton, Alberta, Canada. Electronic address: justin.weinkauf@ualberta.ca.
authors: J
authors: J
authors: Weinkauf
mesh_terms: D001989:Bronchiolitis Obliterans / Q000209:etiology; D005260:Female; D005764:Gastroesophageal Reflux / Q000209:etiology; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D019520:Living Donors; D016040:Lung Transplantation; D008875:Middle Aged; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D013997:Time Factors; D016896:Treatment Outcome