Lung transplantation in scleroderma: recent advances and lessons

Curr Opin Rheumatol. 2018 Nov;30(6):562-569. doi: 10.1097/BOR.0000000000000546.


Purpose of review: The purpose of this review is to highlight recent data regarding feasibility and outcomes following lung transplantation for patients with systemic sclerosis related pulmonary disease as well as to emphasize areas of uncertainly in need of further study. We include a description of our centre's approach to lung transplant evaluation and posttransplant management in this complex patient population.

Recent findings: Historical data have demonstrated that patients with scleroderma have an increased risk of complications following lung transplantation owing to the multisystem nature of disease, particularly concurrent gastrointestinal, cardiac and renal involvement. Emerging data support the safety of lung transplant in appropriately selected patients with scleroderma-related interstitial lung disease and pulmonary arterial hypertension.

Summary: Accumulating evidence validates that a diagnosis of scleroderma is not a priori a contraindication to lung transplant. In the carefully selected patient, both short-term and long-term outcomes following lung transplantation are comparable to counterparts with fibrotic lung disease or pulmonary arterial hypertension. However, further prospective study to detail how these patients should be evaluated and managed posttransplant is definitely needed. Cardiac disease is an emerging cause of morbidity and mortality in the scleroderma population and deserves particular attention during the pre and posttransplant period.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Humans
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / surgery*
  • Lung Diseases, Interstitial / etiology
  • Lung Diseases, Interstitial / surgery*
  • Lung Transplantation / methods*
  • Patient Selection*
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / surgery