Non-respiratory Comorbidities Observed in Pulmonary French Transplant Patients With Cystic Fibrosis

Cystic Fibrosis, Pulmonary Failure

Pulmonary transplantation is the reference treatment for chronic terminal respiratory failure in patients with cystic fibrosis. These are mainly bi-pulmonary transplants (cardiopulmonary transplants are exceptional). The annual number of pulmonary transplants in France for cystic fibrosis is about 90. In 2013, the transplant involves a total of more than 600 patients with cystic fibrosis. The average age at the time of the transplant is 28.5 years (2013 data, French cystic fibrosis register), compared to 58 years for patients transplanted to all pathologies. Cystic fibrosis accounts for 25% of adult bi-pulmonary grafts. Pediatric transplants are currently very rare. The median survival after pulmonary transplantation in cystic fibrosis is currently 8.5 years (and 10 years when considering patients surviving 3 months, ie excluding early mortality). Cystic fibrosis is the pathology associated with better survival after pulmonary transplantation given the young age of patients (28.5 years on average). The non-respiratory comorbidities associated with transplantation, all underlying pathologies combined, and referenced in the Registry of the International Society for Heart and Lung Transplantation (ISHLT) are: hypertension, diabetes, renal insufficiency, Dyslipidemia, cancers. Their frequency increases with the survival time of transplanted patients. Cystic fibrosis is associated with non-respiratory comorbidities, the frequency of which increases with age - diabetes, osteoporosis, renal insufficiency, hepatopathy, neoplastic pathologies - and may become worse after transplantation. The main objective is to estimate the incidence of non-respiratory co-morbidities after lung transplantation in the cohort of patients with cystic fibrosis grafted in the Rhône-Alpes region.

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