Skip to main content
Top
Published in: BMC Pulmonary Medicine 1/2016

Open Access 01-12-2016 | Research article

Safety and efficacy of bridging to lung transplantation with antifibrotic drugs in idiopathic pulmonary fibrosis: a case series

Authors: Isabelle Delanote, Wim A. Wuyts, Jonas Yserbyt, Eric K. Verbeken, Geert M. Verleden, Robin Vos

Published in: BMC Pulmonary Medicine | Issue 1/2016

Login to get access

Abstract

Background

Following recent approval of pirfenidone and nintedanib for idiopathic pulmonary fibrosis (IPF), questions arise about the use of these antifibrotics in patients awaiting lung transplantation (LTx).

Methods

Safety and efficacy of antifibrotic drugs in IPF patients undergoing LTx were investigated in a single-centre retrospective cohort analysis.

Results

A total of nine patients, receiving antifibrotic therapy for 419 ± 315 days until subsequent LTx, were included. No major side effects were noted. Significant weight loss occurred during antifibrotic treatment (p = 0.0062). FVC tended to stabilize after 12 weeks of treatment in most patients. A moderate decline in FVC, TLC and DLCO was noted during the whole pretransplant time period of antifibrotic therapy. Functional exercise capacity and lung allocation score remained unchanged. No post-operative thoracic wound healing problems, nor severe early anastomotic airway complications were attributable to prior antifibrotic treatment. None of the patients developed chronic lung allograft dysfunction after a median follow-up of 19.8 (11.2–26.5) months; and post-transplant survival was 100% after 1 year and 80% after 2 years.

Conclusions

Antifibrotic drugs can probably be safely administered in IPF patients, possibly attenuating disease progression over time, while awaiting LTx.
Literature
1.
go back to reference Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, et al. ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788–824.CrossRefPubMed Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, et al. ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788–824.CrossRefPubMed
2.
go back to reference National Clinical Guideline Centre (UK). Diagnosis and management of suspected idiopathic pulmonary fibrosis: idiopathic pulmonary fibrosis, National Institute for Health and Care Excellence: clinical guidelines. London: Royal College of Physicians (UK); 2013. National Clinical Guideline Centre (UK). Diagnosis and management of suspected idiopathic pulmonary fibrosis: idiopathic pulmonary fibrosis, National Institute for Health and Care Excellence: clinical guidelines. London: Royal College of Physicians (UK); 2013.
3.
go back to reference Noble PW, Albera C, Bradford WZ, Costabel U, Glassberg MK, Kardatzke D, CAPACITY Study Group, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377(9779):1760–9.CrossRefPubMed Noble PW, Albera C, Bradford WZ, Costabel U, Glassberg MK, Kardatzke D, CAPACITY Study Group, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377(9779):1760–9.CrossRefPubMed
4.
go back to reference King Jr TE, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, ASCEND Study Group, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083–92.CrossRefPubMed King Jr TE, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, ASCEND Study Group, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083–92.CrossRefPubMed
5.
go back to reference Richeldi L, du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, INPULSIS Trial Investigators, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071–82.CrossRefPubMed Richeldi L, du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, INPULSIS Trial Investigators, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071–82.CrossRefPubMed
6.
go back to reference Noble PW, Albera C, Bradford WZ, Costabel U, du Bois RM, Fagan EA, et al. Pirfenidone for idiopathic pulmonary fibrosis: analysis of pooled data from three multinational phase 3 trials. Eur Respir J. 2016;47(1):243–53.CrossRefPubMedPubMedCentral Noble PW, Albera C, Bradford WZ, Costabel U, du Bois RM, Fagan EA, et al. Pirfenidone for idiopathic pulmonary fibrosis: analysis of pooled data from three multinational phase 3 trials. Eur Respir J. 2016;47(1):243–53.CrossRefPubMedPubMedCentral
7.
go back to reference Mazzei ME, Richeldi L, Collard HR. Nintedanib in the treatment of idiopathic pulmonary fibrosis. Ther Adv Respir Dis. 2015;9(3):121–9.CrossRefPubMed Mazzei ME, Richeldi L, Collard HR. Nintedanib in the treatment of idiopathic pulmonary fibrosis. Ther Adv Respir Dis. 2015;9(3):121–9.CrossRefPubMed
8.
go back to reference Kistler KD, Nalysnyk L, Rotella P, Esser D. Lung transplantation in idiopathic pulmonary fibrosis: a systematic review of the literature. BMC Pulm Med. 2014;14:139.CrossRefPubMedPubMedCentral Kistler KD, Nalysnyk L, Rotella P, Esser D. Lung transplantation in idiopathic pulmonary fibrosis: a systematic review of the literature. BMC Pulm Med. 2014;14:139.CrossRefPubMedPubMedCentral
9.
go back to reference Conte E, Gili E, Fagone E, Fruciano M, Iemmolo M, Vancheri C. Effect of pirfenidone on proliferation, TGF-β-induced myofibroblast differentiation and fibrogenic activity of primary human lung fibroblasts. Eur J Pharm Sci. 2014;58:13–9.CrossRefPubMed Conte E, Gili E, Fagone E, Fruciano M, Iemmolo M, Vancheri C. Effect of pirfenidone on proliferation, TGF-β-induced myofibroblast differentiation and fibrogenic activity of primary human lung fibroblasts. Eur J Pharm Sci. 2014;58:13–9.CrossRefPubMed
10.
go back to reference Wollin L, Wex E, Pautsch A, Schnapp G, Hostettler KE, Stowasser S, et al. Mode of action of nintedanib in the treatment of idiopathic pulmonary fibrosis. Eur Respir J. 2015;45(5):1434–45.CrossRefPubMedPubMedCentral Wollin L, Wex E, Pautsch A, Schnapp G, Hostettler KE, Stowasser S, et al. Mode of action of nintedanib in the treatment of idiopathic pulmonary fibrosis. Eur Respir J. 2015;45(5):1434–45.CrossRefPubMedPubMedCentral
11.
go back to reference Yserbyt J, Dooms C, Vos R, Dupont LJ, Van Raemdonck DE, Verleden GM. Anastomotic airway complications after lung transplantation: risk factors, treatment modalities and outcome-a single-centre experience. Eur J Cardiothorac Surg. 2016;49(1):e1–8.CrossRefPubMed Yserbyt J, Dooms C, Vos R, Dupont LJ, Van Raemdonck DE, Verleden GM. Anastomotic airway complications after lung transplantation: risk factors, treatment modalities and outcome-a single-centre experience. Eur J Cardiothorac Surg. 2016;49(1):e1–8.CrossRefPubMed
12.
go back to reference Paone G, Sebastiani A, Ialleni E, Diso D, Rose D, Quagliarini F, et al. A combined therapeutic approach in progressive idiopathic pulmonary fibrosis-pirfenidone as bridge therapy for ex vivo lung transplantation: a case report. Transplant Proc. 2015;47(3):855–7.CrossRefPubMed Paone G, Sebastiani A, Ialleni E, Diso D, Rose D, Quagliarini F, et al. A combined therapeutic approach in progressive idiopathic pulmonary fibrosis-pirfenidone as bridge therapy for ex vivo lung transplantation: a case report. Transplant Proc. 2015;47(3):855–7.CrossRefPubMed
13.
go back to reference Riddell P, Minnis P, Ging P, Egan JJ. Pirfenidone as a bridge to lung transplantation in patients with progressive IPF. Thorax. 2014;69:A183.CrossRef Riddell P, Minnis P, Ging P, Egan JJ. Pirfenidone as a bridge to lung transplantation in patients with progressive IPF. Thorax. 2014;69:A183.CrossRef
14.
go back to reference Mortensen A, Cherrier L, Walia R. Lung transplantation on pirfenidone: a single center experience. J Heart Lung Transplant. 2016;35(4S):883. Mortensen A, Cherrier L, Walia R. Lung transplantation on pirfenidone: a single center experience. J Heart Lung Transplant. 2016;35(4S):883.
15.
go back to reference Taniguchi H, Kondoh Y, Ebina M, Azuma A, Ogura T, Taguchi Y, Pirfenidone Clinical Study Group in Japan, et al. The clinical significance of 5% change in vital capacity in patients with idiopathic pulmonary fibrosis: extended analysis of the pirfenidone trial. Respir Res. 2011;12:93.CrossRefPubMedPubMedCentral Taniguchi H, Kondoh Y, Ebina M, Azuma A, Ogura T, Taguchi Y, Pirfenidone Clinical Study Group in Japan, et al. The clinical significance of 5% change in vital capacity in patients with idiopathic pulmonary fibrosis: extended analysis of the pirfenidone trial. Respir Res. 2011;12:93.CrossRefPubMedPubMedCentral
16.
go back to reference Nathan SD, du Bois RM, Albera C, Bradford WZ, Costabel U, Kartashov A, et al. Validation of test performance characteristics and minimal clinically important difference of the 6-min walk test in patients with idiopathic pulmonary fibrosis. Respir Med. 2015;109(7):914–22.CrossRefPubMed Nathan SD, du Bois RM, Albera C, Bradford WZ, Costabel U, Kartashov A, et al. Validation of test performance characteristics and minimal clinically important difference of the 6-min walk test in patients with idiopathic pulmonary fibrosis. Respir Med. 2015;109(7):914–22.CrossRefPubMed
17.
go back to reference Castleberry AW, Englum BR, Snyder LD, Worni M, Osho AA, Gulack BC, et al. The utility of preoperative six-minute-walk distance in lung transplantation. Am J Respir Crit Care Med. 2015;192(7):843–52.CrossRefPubMed Castleberry AW, Englum BR, Snyder LD, Worni M, Osho AA, Gulack BC, et al. The utility of preoperative six-minute-walk distance in lung transplantation. Am J Respir Crit Care Med. 2015;192(7):843–52.CrossRefPubMed
18.
go back to reference Egan TM, Murray S, Bustami RT, Shearon TH, McCullough KP, Edwards LB, et al. Development of the new lung allocation system in the United States. Am J Transplant. 2006;6(5 Pt 2):1212–27.CrossRefPubMed Egan TM, Murray S, Bustami RT, Shearon TH, McCullough KP, Edwards LB, et al. Development of the new lung allocation system in the United States. Am J Transplant. 2006;6(5 Pt 2):1212–27.CrossRefPubMed
19.
go back to reference Egan TM, Edwards LB. Effect of the lung allocation score on lung transplantation in the United States. J Heart Lung Transplant. 2016;35(4):433–9.CrossRefPubMed Egan TM, Edwards LB. Effect of the lung allocation score on lung transplantation in the United States. J Heart Lung Transplant. 2016;35(4):433–9.CrossRefPubMed
20.
go back to reference ESBRIET® (pirfenidone) hard capsules, for oral use. electronic Medicines Compendium. Genentech. Accessed 12 Dec 2015. ESBRIET® (pirfenidone) hard capsules, for oral use. electronic Medicines Compendium. Genentech. Accessed 12 Dec 2015.
21.
go back to reference OFEV® (nintedanib) capsules, for oral use. electronic Medicines Compendium. Boehringer Ingelheim. Accessed 12 Dec 2015. OFEV® (nintedanib) capsules, for oral use. electronic Medicines Compendium. Boehringer Ingelheim. Accessed 12 Dec 2015.
Metadata
Title
Safety and efficacy of bridging to lung transplantation with antifibrotic drugs in idiopathic pulmonary fibrosis: a case series
Authors
Isabelle Delanote
Wim A. Wuyts
Jonas Yserbyt
Eric K. Verbeken
Geert M. Verleden
Robin Vos
Publication date
01-12-2016
Publisher
BioMed Central
Published in
BMC Pulmonary Medicine / Issue 1/2016
Electronic ISSN: 1471-2466
DOI
https://doi.org/10.1186/s12890-016-0308-z

Other articles of this Issue 1/2016

BMC Pulmonary Medicine 1/2016 Go to the issue