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Pretransplant Considerations in Patients with Pulmonary Fibrosis

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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.

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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

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Acknowledgment

We thank Maurilio Tavormina for the graphic design artwork.

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Authors and Affiliations

  1. Department of Internal Medicine, University of Genoa, Genoa, Italy

    Roberto G. Carbone & Francesco Puppo

  2. Departments of Dermatology and Internal Medicine, Clalit Health Services, Tel-Aviv, Israel

    Assaf Monselise

  3. Department of Medicine, Pulmonary, Allergy, and Critical Care Medicine, University of Alabama at Birmingham, Birmingham, AL, USA

    Keith M. Wille

  4. Department of Medicine, University of Genoa—DIMI, Genoa, Italy

    Giovanni Bottino

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  1. Roberto G. Carbone
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  2. Assaf Monselise
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  3. Keith M. Wille
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Editors and Affiliations

  1. Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine University of Washington, Seattle, Washington, USA

    Ganesh Raghu

  2. Department of Internal Medicine, University of Genoa, Genoa, Italy

    Roberto G. Carbone

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Carbone, R.G., Monselise, A., Wille, K.M., Bottino, G., Puppo, F. (2018). Pretransplant Considerations in Patients with Pulmonary Fibrosis. In: Raghu, G., Carbone, R. (eds) Lung Transplantation. Springer, Cham. https://doi.org/10.1007/978-3-319-91184-7_4

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