Lung transplantation in patients with cystic fibrosis: special focus to infection and comorbidities

Abstract

Despite advances in medical care, patients with cystic fibrosis still face limited life expectancy. The most common cause of death remains respiratory failure. End-stage cystic fibrosis can be treated with lung transplantation and is the third most common reason for which the procedure is performed. Outcomes for cystic fibrosis are better than most other lung diseases, but remain limited (5-year survival 60%). For patients with advanced disease lung transplantation appears to improve survival. Outcomes for patients with Burkholderia cepacia remain poor, although they are better for patients with certain genomovars. Controversy exists about Mycobacterium abscessus infection and appropriateness for transplant. More information is also becoming available for comorbidities, including diabetes and pulmonary hypertension among others. Extra-corporeal membrane oxygenation is used more frequently for end-stage disease as a bridge to lung transplantation and will likely be used more in the future.

Keywords::

• Burkholderia cepacia
• cystic fibrosis
• cystic fibrosis-related diabetes
• extracorporeal membrane oxygenation
• lung transplantation
• Mycobacterium abscessus

Financial & competing interests disclosure

DJ Dorgan has been a PI for clinical trials by Insmed and Gilead. D Hadjiliadis has served on the advisory board for Gilead and Vertex; has been a PI for clinical trials Savara, Gilead and MPEX. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending or royalties.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Cystic fibrosis patients have the best outcomes after lung transplantation compared with other end-stage lung disease patients.

• Lung transplantation improves survival for end-stage cystic fibrosis patients with severe disease.

• Burkholderia cenocepacia is the main genomovar that leads to poor outcomes after lung transplantation, while other genomovars are not as ominous.

• Other bacteria and fungi are not associated with poor outcomes when appropriately treated, but controversy exists around Mycobacterium abscessus.

• Poor weight is associated with somewhat poor outcomes after lung transplantation.

• Diabetes likely leads to somewhat worse outcomes after lung transplantation, likely related to whether it is controlled or not.

• Renal dysfunction, malignancy, gastroesophageal reflux disease, pulmonary hypertension and medication non-adherence are emerging comorbidities that could affect pre- or post-transplant outcomes.

• Immunosuppression regimen is not associated with post-transplant outcomes.

• Extracorporeal membrane oxygenation is utilized more as a bridge to lung transplantation for end-stage patients, but more data are needed to learn whether outcomes will be acceptable.