Idiopathic Pulmonary Fibrosis Among Young Patients: Challenges in Diagnosis and Management

Lung
Gabriela LeuschnerClaus Neurohr

Abstract

Idiopathic pulmonary fibrosis (IPF) is considered a disease of older patients, being rare in patients ≤ 50 years. Still, IPF can occur in younger patients, but this particular patient group is not well characterised so far. The aim of this study was to compare the diagnostic certainty, clinical features, comorbidities and survival in young versus older IPF patients. We reviewed our medical records from February 2011 until February 2015, to identify IPF patients, who were then classified as young (≤ 50 years) or older IPF (> 50 years). Radiographic and histological findings, lung function parameters, comorbidities, disease progression and survival were analysed and compared between the two groups. Of 440 patients with interstitial lung disease, 129 patients with IPF were identified, including 30 (23.3%) ≤50 years and 99 (76.7%) > 50 years. There were no differences between age groups in baseline demographics; younger patients were less likely to have a confirmed diagnosis by high-resolution computed tomography (p = 0.014), more likely to require a biopsy (p = 0.08) and less likely to have received antifibrotic therapy (p = 0.006). Despite an overall limited prognosis, younger patients had a significantly better median survival a...Continue Reading

References

Oct 24, 2001·American Journal of Respiratory and Critical Care Medicine·T E KingR M Cherniack
Jul 2, 2002·American Journal of Respiratory and Critical Care Medicine·UNKNOWN ATS Committee on Proficiency Standards for Clinical Pulmonary Function Laboratories
Mar 29, 2003·American Journal of Respiratory and Critical Care Medicine·Athol U WellsDavid M Hansell
Mar 5, 2004·Journal of the American College of Cardiology·Hanno H LeuchteJürgen Behr
Jan 26, 2005·Mayo Clinic Proceedings·Hassan F NadrousJay H Ryu
Jul 1, 2006·American Journal of Respiratory and Critical Care Medicine·Ganesh RaghuGerry Oster
Apr 8, 2011·American Journal of Respiratory and Critical Care Medicine·Ganesh RaghuUNKNOWN ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis
May 16, 2012·Annals of Internal Medicine·Brett LeyHarold R Collard
Dec 21, 2013·Journal of the American College of Cardiology·Marius M HoeperDavid B Badesch
Mar 4, 2014·Mayo Clinic Proceedings·Teng MouaJay H Ryu
Apr 5, 2014·Respirology : Official Journal of the Asian Pacific Society of Respirology·Gregor S ZimmermannClaus Neurohr
May 27, 2015·Respirology : Official Journal of the Asian Pacific Society of Respirology·Claus Neurohr, Juergen Behr
Jul 15, 2015·The European Respiratory Journal·Aryeh FischerUNKNOWN “ERS/ATS Task Force on Undifferentiated Forms of CTD-ILD”
Oct 2, 2015·The European Respiratory Journal·Ganesh RaghuSusanne Stowasser
Feb 4, 2016·PloS One·Sergio HarariGiancarlo Cesana
Apr 23, 2016·The European Respiratory Journal·Justin M OldhamMary E Strek

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Citations

Nov 23, 2018·BMJ Supportive & Palliative Care·Jee Whang KimAndrew M Wilson
May 31, 2019·Respiratory Research·Laurie SnyderUNKNOWN IPF-PRO™ Registry investigators
Dec 15, 2020·Frontiers in Medicine·Gabriela LeuschnerUNKNOWN INSIGHTS-IPF Registry Group

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