Lung Transplantation for Rare Indications

Although lung transplantation is an established procedure for the treatment of severe respiratory failure in selected patients, it remains a “niche” treatment even in Western countries. The average lung transplant rate in these countries is around four cases per million inhabitants, but large disparities also persist among advanced countries. There are several reasons to justify the limited diffusion of lung transplantation, including the scarcity of donors, technical difficulties, logistic problems, and suboptimal results the long term. In this context, a few diseases constitute the bulk of the indications for lung transplantation, such as pulmonary emphysema, idiopathic pulmonary fibrosis, and cystic fibrosis. Many other lung or systemic diseases with lung involvement occupy a small percentage of an already “limited” clinical activity. As a result, only a few hundred or even dozens of patients receive lung transplantation with a rare indication, making it difficult to accumulate experiences. This chapter deals with some of the diseases that most frequently fall into the category of rare indications for lung transplantation such as sarcoidosis or lymphangioleiomyomatosis. Finally, the chapter addresses other debated indications for lung transplantation such as acute respiratory distress syndrome or lung cancer.