Lung transplantation for idiopathic pulmonary fibrosis: A narrative review

Abstract

Idiopathic pulmonary fibrosis (IPF) is a debilitating and progressive lung disease without an identifiable cause. It is the most common form of interstitial pneumonias. The prognosis is worst in this disease with median survival of just 2–3 years after diagnosis without a lung transplant. Currently, there are no proven medical therapies to cure IPF. Pharmacological agents such as nintedanib and pirfenidone retard the progression of the disease to an extent but without any survival benefit. The only therapeutic option for IPF is lung transplantation with proven survival benefit. The major concern with lung transplantation is waiting time mortality. Lung allocation score was introduced in 2005 to reduce this mortality. Both single- and double-lung transplantations are used worldwide for IPF. Bilateral lung transplantation has been seen to have better survival rates in some studies, but there are no randomized trials which favor this recent trend. The posttransplant survival is lower than seen in other indications for lung transplantation. Posttransplant follow-up should be vigilant to detect complications as early as possible and treat them accordingly.

Keywords

• idiopathic pulmonary fibrosis
• lung allocation score
• lung transplantation
• survival