Pretransplant Considerations in Patients with Pulmonary Fibrosis

  • Roberto G. Carbone
  • Assaf Monselise
  • Keith M. Wille
  • Giovanni Bottino
  • Francesco Puppo


Lung transplantation is an established treatment modality for patients with end-stage lung disease and can improve both quality of life (QOL) and survival. Over 6400 lung transplants have been performed since the first successful operation in the early 1980s. According to the International Society for Heart and Lung Transplantation Registry, between January 1995 and June 2015, there were 24.5% lung transplants performed worldwide for interstitial lung disease (ILD) idiopathic interstitial pneumonia (IIP), 5.2% for ILD non-IIP, and 36.4% for chronic obstructive pulmonary disease (COPD). Median survival after lung transplantation is currently 5.5 years, and long-term outcomes are limited mainly by primary graft dysfunction, infection, and allograft rejection. When considering candidates for transplantation, the expected posttransplant survival is carefully weighed against the prognosis of the underlying disease and expected survival if transplant is not performed. The aim of lung transplantation is to improve survival and quality of life, and these goals can be achieved for selected patients with idiopathic pulmonary fibrosis and pulmonary fibrosis associated with connective tissue disease. This chapter will summarize the clinical features of the interstitial lung diseases, such as IPF, connective tissue diseases, systemic sclerosis, rheumatoid arthritis, sarcoidosis, and other rare interstitial lung diseases. Comorbid conditions associated with the ILDs will be discussed, and guidelines for lung transplant candidacy and referral will be presented.


Lung transplantation Interstitial lung disease Idiopathic pulmonary fibrosis Quality of life Evidence Survival Outcome Mortality End-stage lung disease 



6-minute walk test


American Thoracic Society


Bronchiolitis obliterans syndrome


Chronic obstructive pulmonary disease


Diffusing capacity of CO


European Medicine Agency


European Respiratory Society


Food and Drug Administration


Familial pulmonary fibrosis


Forced vital capacity


Gastroesophageal reflux


High-resolution CT of the lung


Interstitial lung diseases


Idiopathic pulmonary fibrosis


International Society of Heart and Lung Transplantation


Lung volume reduction surgery


Non-specific interstitial pneumonia


New York Heart Association


Obstructive sleep apnea


Pulmonary arterial hypertension


Pulmonary arterial pressure


Pulmonary hypertension


Quality of life


Rheumatoid arthritis


Right heart catheterization


Systemic sclerosis


Usual interstitial pneumonia



We thank Maurilio Tavormina for the graphic design artwork.


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Copyright information

© The Author(s) 2018

Authors and Affiliations

  • Roberto G. Carbone
    • 1
  • Assaf Monselise
    • 2
  • Keith M. Wille
    • 3
  • Giovanni Bottino
    • 4
  • Francesco Puppo
    • 1
  1. 1.Department of Internal MedicineUniversity of GenoaGenoaItaly
  2. 2.Departments of Dermatology and Internal MedicineClalit Health ServicesTel-AvivIsrael
  3. 3.Department of Medicine, Pulmonary, Allergy, and Critical Care MedicineUniversity of Alabama at BirminghamBirminghamUSA
  4. 4.Department of MedicineUniversity of Genoa—DIMIGenoaItaly

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