A case report of living-donor lobar lung transplantation for scleroderma-associated usual interstitial pneumonia: eight years and counting.

Introduction: 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.

Clinical Case: 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)].

Relevance: This is the second reported case of LDLLT in scleroderma, and the first reporting long-term pulmonary, renal, and esophageal function post-transplantation.