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Pretransplant Considerations in Patients with Pulmonary Fibrosis

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

Abstract

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.

Keywords

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

Abbreviations

6-MWT

6-minute walk test

ATS

American Thoracic Society

BOS

Bronchiolitis obliterans syndrome

COPD

Chronic obstructive pulmonary disease

DLCO

Diffusing capacity of CO

EMA

European Medicine Agency

ERS

European Respiratory Society

FDA

Food and Drug Administration

FPF

Familial pulmonary fibrosis

FVC

Forced vital capacity

GER

Gastroesophageal reflux

HRCT

High-resolution CT of the lung

ILD

Interstitial lung diseases

IPF

Idiopathic pulmonary fibrosis

ISHLT

International Society of Heart and Lung Transplantation

LVRS

Lung volume reduction surgery

NSIP

Non-specific interstitial pneumonia

NYHA

New York Heart Association

OSA

Obstructive sleep apnea

PAH

Pulmonary arterial hypertension

PAP

Pulmonary arterial pressure

PH

Pulmonary hypertension

QOL

Quality of life

RA

Rheumatoid arthritis

RHC

Right heart catheterization

SSc

Systemic sclerosis

UIP

Usual interstitial pneumonia

Notes

Acknowledgment

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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