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 - 12/07/14
, Laurent Savale 4, 5, 6, Alice Berezne 7, 17, Jérôme Le Pavec 4, 6, 8, 17, Eric Hachulla 1, 2, 3, Luc Mouthon 7, Olivier Sitbon 4, 5, 6, Benoit Lambert 9, Marianne Gaudric 10, Xavier Jais 4, 5, 6, Francois Stephan 11, Pierre-Yves Hatron 1, 2, Nicolas Lamblin 1, 12, Olivier Vignaux 13, Vincent Cottin 14, Dominique Farge 15, Benoît Wallaert 1, 16, Loic Guillevin 7, Gerald Simonneau 4, 5, 6, Olaf Mercier 4, 6, 8, Elie Fadel 4, 6, 8, Philippe Dartevelle 4, 6, 8, Marc Humbert 4, 5, 6, Sacha Mussot 4, 6, 8, ⁎ 
On behalf of the Working Group on Heart/Lung transplantation in systemic sclerosis of the French Network on Pulmonary Hypertension
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Summary |
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.
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