Mortality on the Waiting List for Lung Transplantation in Patients with Idiopathic Pulmonary Fibrosis: A Single-Centre Experience

Bennett, David; Fossi, Antonella; Bargagli, Elena; Refini, Rosa Metella; Pieroni, Maria; Luzzi, Luca; Ghiribelli, Claudia; Paladini, Piero; Voltolini, Luca; Rottoli, Paola

doi:10.1007/s00408-015-9767-x

Cited by 47 publications

(30 citation statements)

References 22 publications

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“…Alveolar hypoventilation (hypercapnia) while awake is not common in IPF and is considered a feature of end-stage disease [74], when respiratory muscles fail in the face of a highly increased mechanical load (strongly reduced lung compliance). Alveolar hypoventilation is frequent during sleep in IPF [75].…”

Section: Chronic Arterial Hypoxaemiamentioning

confidence: 99%

Physiology of the lung in idiopathic pulmonary fibrosis

et al. 2018

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The clinical expression of idiopathic pulmonary fibrosis (IPF) is directly related to multiple alterations in lung function. These alterations derive from a complex disease process affecting all compartments of the lower respiratory system, from the conducting airways to the lung vasculature. In this article we review the profound alterations in lung mechanics (reduced lung compliance and lung volumes), pulmonary gas exchange (reduced diffusing capacity, increased dead space ventilation, chronic arterial hypoxaemia) and airway physiology (increased cough reflex and increased airway volume), as well as pulmonary haemodynamics related to IPF. The relative contribution of these alterations to exertional limitation and dyspnoea in IPF is discussed.

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Section: Chronic Arterial Hypoxaemiamentioning

confidence: 99%

Physiology of the lung in idiopathic pulmonary fibrosis

et al. 2018

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“…A recent paper from a single centre in Italy also shows that the diagnosis of IPF was associated with the highest mortality on the lung transplant waiting list. Factors associated with greater mortality include higher levels of CO 2 and the need for higher flows of O 2 …”

Section: Non‐pharmacological Therapymentioning

confidence: 99%

Treatment of idiopathic pulmonary fibrosis in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia

Troy

Keir

et al. 2017

Respirology

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Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease (ILD) of unknown aetiology with a median survival of only 2-5 years. It is characterized by progressive dyspnoea and worsening lung function, ultimately resulting in death. Until recently, there were no effective therapies for IPF; however, with the publication of two landmark clinical trials in 2014, the anti-fibrotic therapies, nintedanib and pirfenidone, have gained widespread approval. This position paper aims to highlight the current evidence for the treatment of IPF, with particular application to the Australian and New Zealand population. We also consider areas in which evidence is currently lacking, especially with regard to the broader IPF severity spectrum and treatment of co-morbid conditions. The utility of non-pharmacological therapies including pulmonary rehabilitation, oxygen as well as symptom management thought to be important in the holistic care of IPF patients are also discussed.

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“…This recommendation reflects the phenotypic heterogeneity and thus difficulties of predicting the individual course. The overall prognosis for IPF is poor compared with other indications for lung transplantation, illustrated by the fact that IPF has the greatest waiting list mortality, despite the shortest median waiting list time 47 …”

Section: Non‐pharmacological Therapymentioning

confidence: 99%

“…The overall prognosis for IPF is poor compared with other indications for lung transplantation, illustrated by the fact that IPF has the greatest waiting list mortality, despite the shortest median waiting list time. 47 Chronological age is no longer an absolute contraindication to transplant; rather, the comorbidities with end-organ damage and physiological reserve are the main criteria used. Indications for lung transplant listing include desaturation to < 88% or distance < 250 m on 6-minute walk testing or > 50 m decline in 6-minute walk distance over a 6-month period, a fall in FVC 10% and/or in DL co 15% during 6-month follow-up, the presence of pulmonary hypertension, or hospitalisation for acute exacerbation, respiratory decline or pneumothorax.…”

Section: Lung Transplantationmentioning

confidence: 99%

Diagnosis and management of idiopathic pulmonary fibrosis: Thoracic Society of Australia and New Zealand and Lung Foundation Australia position statements summary

Prasad

Troy

et al. 2017

Medical Journal of Australia

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Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease associated with debilitating symptoms of dyspnoea and cough, resulting in respiratory failure, impaired quality of life and ultimately death. Diagnosing IPF can be challenging, as it often shares many features with other interstitial lung diseases. In this article, we summarise recent joint position statements on the diagnosis and management of IPF from the Thoracic Society of Australia and New Zealand and Lung Foundation Australia, specifically tailored for physicians across Australia and New Zealand. Main suggestions: A comprehensive multidisciplinary team meeting is suggested to establish a prompt and precise IPF diagnosis. Antifibrotic therapies should be considered to slow disease progression. However, enthusiasm should be tempered by the lack of evidence in many IPF subgroups, particularly the broader disease severity spectrum. Non-pharmacological interventions including pulmonary rehabilitation, supplemental oxygen, appropriate treatment of comorbidities and disease-related symptoms remain crucial to optimal management. Despite recent advances, IPF remains a fatal disease and suitable patients should be referred for lung transplantation assessment.

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Mortality on the Waiting List for Lung Transplantation in Patients with Idiopathic Pulmonary Fibrosis: A Single-Centre Experience

Cited by 47 publications

References 22 publications

Physiology of the lung in idiopathic pulmonary fibrosis

Physiology of the lung in idiopathic pulmonary fibrosis

Treatment of idiopathic pulmonary fibrosis in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia

Diagnosis and management of idiopathic pulmonary fibrosis: Thoracic Society of Australia and New Zealand and Lung Foundation Australia position statements summary

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