Early diagnosis of idiopathic pulmonary fibrosis (IPF) is definitely a challenge.
Several studies have been investigating how to achieve this goal. IPF is the most
common type of idiopathic interstitial pneumonia, the median age at diagnosis is approximately
65 years and it's more common in smokers and males [
[1]
]. To date, there are only two therapeutic options available (pirfenidone and nintedanib)
that have shown to be effectively slowing disease progression in terms of forced vital
capacity (FVC) [
[2]
] with a further decrease in the risk of acute exacerbations for nintedanib [
[3]
]. Regardless of the disease severity, the delay between first respiratory symptoms
and referral to a tertiary care centre could be years and this ‘waiting’ leads to
an increase in mortality [
[4]
]. Furthermore, the absence of accurate short-term indicators of therapeutic response
leaves clinicians with uncertainty regarding management. Reduced survival time has
been associated with various factors such as advanced age, severe physiological impairment,
low body-mass index, radiological extent and severity of fibrosis determined by chest
high-resolution computed tomography (HRCT), presence of comorbidities including pulmonary
hypertension (PH), emphysema and bronchogenic cancer. Several risk prediction models
have been developed in order to improve clinicians’ ability to predict prognosis in
IPF and to modify the natural course of the disease, however accurate prognostication
in patients with IPF is challenging due to the highly variable natural course of the
disease [
[5]
]. Formulating a diagnosis of IPF at an early stage and increasing prognostic accuracy
are fundamental strategies to optimize clinical management and allow timely referral
for lung transplantations.To read this article in full you will need to make a payment
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References
- Idiopathic pulmonary fibrosis.Lancet. 2017; 389: 1941-1952
- An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis. An update of the 2011 clinical practice guideline.Am J Respir Crit Care Med. 2015; 192: e3-19
- Delayed access and survival in idiopathic pulmonary fibrosis: a cohort study.Am J Respir Crit Care Med. 2011; 184: 842-847
- Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis.N Engl J Med. 2014; 370: 2071-2082
- Clinical course and prediction of survival in idiopathic pulmonary fibrosis.Am J Respir Crit Care Med. 2011; 183: 431-440
- Cluster analysis based clinical profiling of Idiopathic Pulmonary Fibrosis patients according to comorbidities evident prior to diagnosis: a single-center observational study.Eur J Intern Med. 2020; : 1-6
- Neglected evidence in idiopathic pulmonary fibrosis: from history to earlier diagnosis.Eur Respir J. 2013; 42: 916-923
- Velcro-type " crackles predict specific radiologic features of fibrotic interstitial lung disease.BMC Pulm Med. 2018; 18: 103
- Timing of onset of symptoms in people with idiopathic pulmonary fibrosis.Thorax. 2018; 73: 683-685
- Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity.Eur Respir J. 2005; 26: 586-593
- Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension.Chest. 2009; 136: 10-15
- Clinical features and outcomes in combined pulmonary fibrosis and emphysema in idiopathic pulmonary fibrosis.Chest. 2013; 144: 234-240
- Thyroid disease is prevalent and predicts survival in patients with idiopathic pulmonary fibrosis.Chest. 2015; 148: 692-700
- Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review.Eur Respir J. 2015; 46: 1113-1130
- Use of cluster analysis to define COPD phenotypes.Eur Respir J. 2010; 36: 472-474
Article info
Publication history
Published online: August 23, 2020
Accepted:
August 17,
2020
Received:
August 15,
2020
Identification
Copyright
© 2020 Published by Elsevier B.V. on behalf of European Federation of Internal Medicine.
