Abstract
There have been over 120 diffuse parenchymal lung diseases (DPLD) identified, and these diseases have similar clinical and pathological findings. These diseases are associated with autoimmune disorders, environmental or drug exposures, or are idiopathic in nature. Unfortunately, many of these diseases have limited effective therapies and may require lung transplantation for ongoing survival. Because of changes that have been made in lung allocation, lung transplantation in patients with DPLD is becoming more common. For successful lung transplantation, the DPLD patient requires optimized global medical management. Once transplanted, patients typically have a change in their lifestyle that requires multiple medications, dedication to lifelong exercise, and chronic medical management; however, they also garner a survival benefit and significant improvement in quality of life. Part of the transplant process is to inform patients fully of the risks, costs, and benefits associated with the procedure. It is imperative that patients are evaluated early so that a relationship between transplant centers and patients can be established.
Similar content being viewed by others
References
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
Glazer CS, Newman LS. Occupational interstitial lung disease. Clin Chest Med. 2004;25(3):467–78, vi. doi:10.1016/j.ccm.2004.04.004.
Wade 3rd JF, King Jr TE. Infiltrative and interstitial lung disease in the elderly patient. Clin Chest Med. 1993;14(3):501–21.
King T. Interstitial lung disease: general approaches. In: Parson PE HJ, editor. Pulmonary and respiratory therapy secrets. Philadelphia: Handley & Belfus; 1997. p. 231.
Schwarz MI KT, King TE Jr, Schwarz MI, ed. Interstitial lung disease. 4th ed. B.C. Decker; 2003:1.
Raghu G, Collard HR, Egan JJ, et al. 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. doi:10.1164/rccm.2009-040GL. Guidelines for diagnosis of IPF and management of the disease.
Hardy JD, Webb WR, Dalton ML, Walker Jr GR. Lung homotransplantation in man. JAMA. 1963;186:1065–74.
Reitz BA, Wallwork JL, Hunt SA, et al. Heart-lung transplantation: successful therapy for patients with pulmonary vascular disease. N Engl J Med. 1982;306(10):557–64. doi:10.1056/NEJM198203113061001.
Unilateral lung transplantation for pulmonary fibrosis. Toronto Lung Transplant Group. N Engl J Med. 1986;314(18):1140–1145. doi:10.1056/NEJM198605013141802.
Yusen RD, Christie JD, Edwards LB, et al. The registry of the international society for heart and lung transplantation: thirtieth adult lung and heart-lung transplant report–2013; focus theme: age. J Heart Lung Transplant. 2013;32(10):965–78. doi:10.1016/j.healun.2013.08.007. Review of survival statistics associated with lung transplantation and causes of death in lung transplant recipients.
Valapour M, Paulson K, Smith JM, et al. OPTN/SRTR 2011 annual data report: lung. Am J Transplant. 2013;13 Suppl 1:149–77. doi:10.1111/ajt.12024.
Orens JB, Estenne M, Arcasoy S, et al. International guidelines for the selection of lung transplant candidates: 2006 update—a consensus report from the pulmonary scientific council of the international society for heart and lung transplantation. J Heart Lung Transplant. 2006;25(7):745–55.
Madill J, Gutierrez C, Grossman J, et al. Nutritional assessment of the lung transplant patient: body mass index as a predictor of 90-day mortality following transplantation. J Heart Lung Transplant. 2001;20(3):288–96.
Bittner HB, Lehmann S, Rastan A, et al. Outcome of extracorporeal membrane oxygenation as a bridge to lung transplantation and graft recovery. Ann Thorac Surg. 2012;94(3):942–9; author reply 949–50. doi:10.1016/j.athoracsur.2012.05.006.
US Department of Health and Human Services. www.organdonor.gov. Accessed 15 April 2014.
Levvey BJ, Harkess M, Hopkins P, et al. Excellent clinical outcomes from a national donation-after-determination-of-cardiac-death lung transplant collaborative. Am J Transplant. 2012;12(9):2406–13. doi:10.1111/j.1600-6143.2012.04193.x.
Browne A. The ethics of organ donation after cardiocirculatory death: do the guidelines of the canadian council for donation and transplantation measure up? Open Med. 2010;4(2):e129–33.
Cypel M, Yeung JC, Liu M, et al. Normothermic ex vivo lung perfusion in clinical lung transplantation. N Engl J Med. 2011;364(15):1431–40. doi:10.1056/NEJMoa1014597. Describes how Ex vivo lung perfusion and how it can utilized.
de Perrot M, Liu M, Waddell TK, Keshavjee S. Ischemia-reperfusion-induced lung injury. Am J Respir Crit Care Med. 2003;167(4):490–511. doi:10.1164/rccm.200207-670SO.
Suzuki Y, Cantu E, Christie JD. Primary graft dysfunction. Semin Respir Crit Care Med. 2013;34(3):305–19. doi:10.1055/s-0033-1348474. Excellent review of primary graft dysfunction.
Lee JC, Christie JD, Keshavjee S. Primary graft dysfunction: definition, risk factors, short- and long-term outcomes. Semin Respir Crit Care Med. 2010;31(2):161–71. doi:10.1055/s-0030-1249111.
Lee JC, Christie JD. Primary graft dysfunction. Proc Am Thorac Soc. 2009;6(1):39–46. doi:10.1513/pats.200808-082GO.
Hayes Jr D. A review of bronchiolitis obliterans syndrome and therapeutic strategies. J Cardiothorac Surg. 2011;6:92-8090-6-92. doi:10.1186/1749-8090-6-92.
Whitson BA, Prekker ME, Herrington CS, et al. Primary graft dysfunction and long-term pulmonary function after lung transplantation. J Heart Lung Transplant. 2007;26(10):1004–11.
Kugler C, Fischer S, Gottlieb J, et al. Health-related quality of life in two hundred-eighty lung transplant recipients. J Heart Lung Transplant. 2005;24(12):2262–8.
Estenne M, Maurer JR, Boehler A, et al. Bronchiolitis obliterans syndrome 2001: an update of the diagnostic criteria. J Heart Lung Transplant. 2002;21(3):297–310.
Verleden GM, Dupont LJ. Azithromycin therapy for patients with bronchiolitis obliterans syndrome after lung transplantation. Transplantation. 2004;77(9):1465–7.
Vos R, Vanaudenaerde BM, Verleden SE, et al. A randomised controlled trial of azithromycin to prevent chronic rejection after lung transplantation. Eur Respir J. 2011;37(1):164–72. doi:10.1183/09031936.00068310.
Vos R, Vanaudenaerde BM, Verleden SE, Van Raemdonck DE, Dupont LJ, Verleden GM. Azithromycin in posttransplant bronchiolitis obliterans syndrome. Chest. 2011;139(5):1246; author reply 1247–2944. doi:10.1378/chest.10-2944. Review of azithromycin therapy for BOS.
Whelan TP, Dunitz JM, Kelly RF, et al. Effect of preoperative pulmonary artery pressure on early survival after lung transplantation for idiopathic pulmonary fibrosis. J Heart Lung Transplant. 2005;24(9):1269–74.
King TE Jr, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083–92. doi:10.1056/NEJMoa1402582.
Richeldi L, du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071–82. doi:10.1056/NEJMoa1402584.
Latsi PI, du Bois RM, Nicholson AG, et al. Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends. Am J Respir Crit Care Med. 2003;168(5):531–7. doi:10.1164/rccm.200210-1245OC.
Ryerson CJ, Vittinghoff E, Ley B, et al. Predicting survival across chronic interstitial lung disease: the ILD-GAP model. Chest. 2014;145(4):723–8. doi:10.1378/chest.13-1474. Validates GAP model for IPF survival, aids when to transplant patients.
Lama VN, Flaherty KR, Toews GB, et al. Prognostic value of desaturation during a 6-minute walk test in idiopathic interstitial pneumonia. Am J Respir Crit Care Med. 2003;168(9):1084–90. doi:10.1164/rccm.200302-219OC.
du Bois RM, Albera C, Bradford WZ, et al. 6-minute walk test distance is an independent predictor of mortality in patients with idiopathic pulmonary fibrosis. Eur Respir J. 2013.
du Bois RM, Weycker D, Albera C, et al. Ascertainment of individual risk of mortality for patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;184(4):459–66. doi:10.1164/rccm.201011-1790OC.
Gottlieb J. Lung transplantation for interstitial lung diseases and pulmonary hypertension. Semin Respir Crit Care Med. 2013;34(3):281–7. doi:10.1055/s-0033-1348462.
Thomas KW, Hunninghake GW. Sarcoidosis. JAMA. 2003;289(24):3300–3. doi:10.1001/jama.289.24.3300.
Steen VD, Lucas M, Fertig N, Medsger Jr TA. Pulmonary arterial hypertension and severe pulmonary fibrosis in systemic sclerosis patients with a nucleolar antibody. J Rheumatol. 2007;34(11):2230–5.
Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis. 2007;66(7):940–4.
D’Ovidio F, Mura M, Tsang M, et al. Bile acid aspiration and the development of bronchiolitis obliterans after lung transplantation. J Thorac Cardiovasc Surg. 2005;129(5):1144–52.
D’Ovidio F, Singer LG, Hadjiliadis D, et al. Prevalence of gastroesophageal reflux in end-stage lung disease candidates for lung transplant. Ann Thorac Surg. 2005;80(4):1254–60.
Murthy SC, Nowicki ER, Mason DP, et al. Pretransplant gastroesophageal reflux compromises early outcomes after lung transplantation. J Thorac Cardiovasc Surg. 2011;142(1):47–52.e3. doi:10.1016/j.jtcvs.2011.04.028.
Khan IY, Singer LG, de Perrot M, et al. Survival after lung transplantation in systemic sclerosis. A systematic review. Respir Med. 2013;107(12):2081–7. doi:10.1016/j.rmed.2013.09.015.
Salhi B, Troosters T, Behaegel M, Joos G, Derom E. Effects of pulmonary rehabilitation in patients with restrictive lung diseases. Chest. 2010;137(2):273–9. doi:10.1378/chest.09-0241.
Ryerson CJ, Cayou C, Topp F, et al. Pulmonary rehabilitation improves long-term outcomes in interstitial lung disease: a prospective cohort study. Respir Med. 2014;108(1):203–10. doi:10.1016/j.rmed.2013.11.016. Shows that pulmonary rehabilitation is important for all patients for interstitial lung disease.
Kenn K, Gloeckl R, Behr J. Pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis—a review. Respiration. 2013;86(2):89–99. doi:10.1159/000354112.
Lederer DJ, Arcasoy SM, Wilt JS, D’Ovidio F, Sonett JR, Kawut SM. Six-minute-walk distance predicts waiting list survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2006;174(6):659–64.
Caminati A, Bianchi A, Cassandro R, Mirenda MR, Harari S. Walking distance on 6-MWT is a prognostic factor in idiopathic pulmonary fibrosis. Respir Med. 2009;103(1):117–23. doi:10.1016/j.rmed.2008.07.022.
Allen JG, Arnaoutakis GJ, Weiss ES, Merlo CA, Conte JV, Shah AS. The impact of recipient body mass index on survival after lung transplantation. J Heart Lung Transplant. 2010;29(9):1026–33. doi:10.1016/j.healun.2010.05.005.
de la Torre MM, Delgado M, Paradela M, et al. Influence of body mass index in the postoperative evolution after lung transplantation. Transplant Proc. 2010;42(8):3026–8. doi:10.1016/j.transproceed.2010.07.078.
Bentley TS HS. 2011 US ogan and tissue trasnplant cost estimates and discussion http://publications.milliman.com/research/health-rr/pdfs/2011-us-organ-tissue.pdf. Accessed 16 April 2014.
Compliance with Ethics Guidelines
Conflicts of Interest
Luca Paoletti has reported no conflicts of interest.
Timothy P. M. Whelan is a consultant of InterMune and his institution receives grants from InterMune, Boehringer Ingelheim, Gilead Sciences, MedImmune, Sanofi, Roche for IPF Clinical Trials Research.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Paoletti, L., Whelan, T.P.M. Lung transplantation for interstitial lung disease. Curr Respir Care Rep 3, 96–102 (2014). https://doi.org/10.1007/s13665-014-0083-3
Published:
Issue Date:
DOI: https://doi.org/10.1007/s13665-014-0083-3