Lung and heart-lung transplantation for systemic sclerosis patients. A monocentric experience of 13 patients, review of the literature and position paper of a multidisciplinary Working Group

Presse Med. 2014 Oct;43(10 Pt 2):e345-63. doi: 10.1016/j.lpm.2014.01.020. Epub 2014 Jul 11.


Systemic sclerosis per se should not be considered as an a priori contraindication for a pre-transplantation assessment in patients with advanced interstitial lung disease and/or pulmonary hypertension. For lung or heart-lung transplantation, a multidisciplinary approach, adapting the pre-transplant assessment to systemic sclerosis and optimizing systemic sclerosis patient management before, during and after surgery should improved the short- and long-term prognosis. Indications and contraindications for transplantation have to be adapted to the specificities of systemic sclerosis. A special focus on the digestive tract involvement and its thorough evaluation are mandatory before transplantation in systemic sclerosis. As the esophagus is almost always involved, isolated gastro-oesophageal reflux disease, pH metry and/or manometry abnormalities should not be a systematic per se contraindication for pre-transplantation assessment. Corticosteroids may be harmful in systemic sclerosis as they are associated with acute renal crisis. A low dose corticosteroids protocol for immunosuppression is therefore advisable in systemic sclerosis.

Publication types

  • Review

MeSH terms

  • Heart Diseases / etiology
  • Heart Diseases / surgery
  • Heart-Lung Transplantation*
  • Humans
  • Lung Diseases / etiology
  • Lung Diseases / surgery
  • Middle Aged
  • Retrospective Studies
  • Scleroderma, Systemic / complications
  • Scleroderma, Systemic / surgery*