Pirfenidone in restrictive allograft syndrome after lung transplantation: A case series

Abstract

Pirfenidone may attenuate the decline of pulmonary function in restrictive allograft syndrome (RAS) after lung transplantation. We retrospectively assessed all lung transplant recipients with RAS who were treated with pirfenidone for at least 3 months (n = 11) in our lung transplant center and report on their long-term outcomes following initiation of pirfenidone. Main outcome parameters included evolution of pulmonary function and overall survival. Pirfenidone appears to attenuate the decline in forced vital capacity and forced expiratory volume in 1 second. Notably, 3 patients were bridged to redo-transplantation with pirfenidone for 11 (5-12) months and are currently alive, while 3 other patients demonstrate long-term stabilization of pulmonary function after 26.6 (range 18.4-46.6) months of treatment. Median overall 3-year survival after RAS diagnosis was 54.5%. Subjective intolerance, mainly anorexia and nausea, necessitating pirfenidone dose de-escalation in 55% of patients, as well as calcineurin dose increase requirements with about 20% are important complications during pirfenidone treatment after lung transplantation. Our findings provide further evidence that pirfenidone appears to be safe and may attenuate the rate of decline in lung function in patients with RAS, but the actual clinical benefit cannot be assessed in the context of this study design and requires further investigation in a larger randomized trial.

Restrictive allograft dysfunction after lung transplantation: is there a place for nintedanib?—a case report

“…A dose de‐escalation was performed in 6/11 patients mainly because of anorexia and nausea. The monthly decline of FEV1 and FVC was attenuated with pirfenidone, three patients got redo‐transplanted, and three patients are still alive without redo‐transplantation .…”

Restrictive allograft syndrome after lung transplantation

“…The average 3‐year survival after RAS diagnosis was increased to 54.5% in the treatment group and CT scans in patients treated with pirfenidone showed improvement after treatment. While promising, this was a retrospective report which was unable to confirm the benefit of pirfenidone in patients with RAS 38 . Nintedanib, another anti‐fibrotic agent, was also shown to stabilize pulmonary functions and improve quality of life after 6 months of treatment in one case report 39 .…”

“…While promising, this was a retrospective report which was unable to confirm the benefit of pirfenidone in patients with RAS. 38 Nintedanib, another anti-fibrotic agent, was With a better understanding of the pathogenesis of CLAD, treatment and prevention strategies can be investigated to improve patient outcomes.…”

A Review of Pirfenidone as an Anti-Fibrotic in Idiopathic Pulmonary Fibrosis and Its Probable Role in Other Diseases

“…PFD seemed to lower the decline in the forced vital capacity and forced expiratory volume in one second. However, more extensive studies are needed [32]. Hence, both studies show there is a scope for PFD to be used in the transplantation aspect.…”