Medscape InDiscussion: Idiopathic Pulmonary Fibrosis

Dr Jeffrey Swigris and Janet Talbert discuss the current understanding of the genetics of pulmonary fibrosis, explore the identified genetic pathways, and discuss future research directions.

Relevant disclosures can be found with the episode show notes on Medscape https://www.medscape.com/viewarticle/999863. The topics and discussions are planned, produced, and reviewed independently of advertisers. This podcast is intended only for US healthcare professionals.

Resources

Pulmonary Fibrosis Predisposition Overview https://pubmed.ncbi.nlm.nih.gov/20301408/

Aging and Pulmonary Fibrosis https://pubmed.ncbi.nlm.nih.gov/27103043/

Telomere Length and Risk of Idiopathic Pulmonary Fibrosis and Chronic Obstructive Pulmonary Disease: A Mendelian Randomisation Study https://pubmed.ncbi.nlm.nih.gov/33197388/

Lung Fibrosis, Premature Graying, and Macrocytosis https://pubmed.ncbi.nlm.nih.gov/22942352/

Idiopathic Pulmonary Fibrosis Is Associated With Common Genetic Variants and Limited Rare Variants https://pubmed.ncbi.nlm.nih.gov/36602845/

Idiopathic Pulmonary Fibrosis and the Role of Genetics in the Era of Precision Medicine https://pubmed.ncbi.nlm.nih.gov/37181377/

Telomeres in Interstitial Lung Disease: The Short and the Long of It https://pubmed.ncbi.nlm.nih.gov/30540921/

Perceptions of Genetic Testing: A Mixed-Methods Study of Patients With Pulmonary Fibrosis and Their First-Degree Relatives https://pubmed.ncbi.nlm.nih.gov/35584322/

American College of Medical Genetics and Genomics https://www.acmg.net/

National Society of Genetic Counselors https://www.nsgc.org/

American Board of Genetic Counselors https://www.abgc.net/

Drs Jeffrey Swigris and M. Patricia George discuss the complexities of diagnosing and treating pulmonary hypertension in patients with interstitial lung disease.

Relevant disclosures can be found with the episode show notes on Medscape https://www.medscape.com/viewarticle/999862. The topics and discussions are planned, produced, and reviewed independently of advertisers. This podcast is intended only for US healthcare professionals.

Resources

Idiopathic Pulmonary Fibrosis (IPF) https://emedicine.medscape.com/article/301226-overview

Pulmonary Hypertension in Interstitial Lung Disease: An Area of Unmet Clinical Need https://pubmed.ncbi.nlm.nih.gov/36382238/

Assessing the Accuracy of the Lung Allocation Score https://pubmed.ncbi.nlm.nih.gov/34802876/

The 6-Minute Walk Test: Indications and Guidelines for Use in Outpatient Practices https://pubmed.ncbi.nlm.nih.gov/35578650/

Pulmonary Rehabilitation https://emedicine.medscape.com/article/319885-overview

Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease https://pubmed.ncbi.nlm.nih.gov/33440084/

Provider Perspectives on and Access to Palliative Care for Patients With Interstitial Lung Disease https://pubmed.ncbi.nlm.nih.gov/35305969/

PDE5 to Keep Them Alive: The Use of Phosphodiesterase Type-5 Inhibitors in Severe Pulmonary Hypertension Associated With Interstitial Lung Disease https://pubmed.ncbi.nlm.nih.gov/36318987/

Pulmonary Arterial Hypertension https://emedicine.medscape.com/article/303098-overview

A Controlled Trial of Sildenafil in Advanced Idiopathic Pulmonary Fibrosis https://pubmed.ncbi.nlm.nih.gov/20484178/

Diffusing Capacity of the Lungs for Carbon Monoxide https://pubmed.ncbi.nlm.nih.gov/32310609/

Exercise-Induced Pulmonary Hypertension: A Valid Entity or Another Factor of Confusion? https://pubmed.ncbi.nlm.nih.gov/36676077/

Echocardiogram https://www.ncbi.nlm.nih.gov/books/NBK558940/

Dr Jeffrey Swigris and Scott Staszak discuss how patient advocacy at the Pulmonary Fibrosis Foundation is transforming IPF care and research.

Relevant disclosures can be found with the episode show notes on Medscape https://www.medscape.com/viewarticle/999861. The topics and discussions are planned, produced, and reviewed independently of advertisers. This podcast is intended only for US healthcare professionals.

Resources

Pulmonary Fibrosis Foundation https://www.pulmonaryfibrosis.org/

Managing Cough in Idiopathic Pulmonary Fibrosis https://pubmed.ncbi.nlm.nih.gov/34171385/

PFF Help Center https://www.pulmonaryfibrosis.org/patients-caregivers/medical-and-support-resources/pff-help-center

PFF Scholars https://www.pulmonaryfibrosis.org/researchers-healthcare-providers/research-opportunities/scholars

PFF Registry https://www.pulmonaryfibrosis.org/pff-registry/pff-patient-registry

The Pulmonary Fibrosis Foundation Patient Registry. Rationale, Design, and Methods https://pubmed.ncbi.nlm.nih.gov/32776789/

Circulating Biomarker Analyses in a Longitudinal Cohort of Patients With IPF https://pubmed.ncbi.nlm.nih.gov/38226605/

PFF Community Registry https://www.pulmonaryfibrosis.org/pff-registry/community

Supplemental Oxygen Access Reform https://www.congress.gov/bill/118th-congress/senate-bill/3821

NHLBI Idiopathic Pulmonary Fibrosis Research https://www.nhlbi.nih.gov/research/idiopathic-pulmonary-fibrosis

Department of Defense Basic Research Directorate https://basicresearch.defense.gov/

PFF Become an Advocate https://www.pulmonaryfibrosis.org/advocacy

Dr Jeffrey Swigris and Isaac Kirubakaran Sundar discuss the complex role of circadian rhythms in patients with idiopathic pulmonary fibrosis.

Relevant disclosures can be found with the episode show notes on Medscape https://www.medscape.com/viewarticle/999860. The topics and discussions are planned, produced, and reviewed independently of advertisers. This podcast is intended only for US healthcare professionals.

Resources

Idiopathic Pulmonary Fibrosis (IPF) https://emedicine.medscape.com/article/301226-overview

Circadian Clock-Based Therapeutics in Chronic Pulmonary Diseases https://pubmed.ncbi.nlm.nih.gov/36302705/

Interstitial Lung Disease: Pathophysiology and Genetic Predisposition https://www.medscape.com/viewarticle/408750

Circadian Timing in the Lung; A Specific Role for Bronchiolar Epithelial Cells https://pubmed.ncbi.nlm.nih.gov/18787022/

The History and Mystery of Alveolar Epithelial Type II Cells: Focus on Their Physiologic and Pathologic Role in Lung https://pubmed.ncbi.nlm.nih.gov/33806395/

TGF-β Signaling in Lung Health and Disease https://pubmed.ncbi.nlm.nih.gov/30127261/

Drs Jeffrey Swigris and Anna Gersten discuss the importance of discussing palliative care with patients and integrating it into IPF treatment plans.

Relevant disclosures can be found with the episode show notes on Medscape https://www.medscape.com/viewarticle/999859. The topics and discussions are planned, produced, and reviewed independently of advertisers. This podcast is intended only for US healthcare professionals.

Resources

Idiopathic Pulmonary Fibrosis (IPF) https://emedicine.medscape.com/article/301226-overview

Provider Perspectives on and Access to Palliative Care for Patients With Interstitial Lung Disease https://pubmed.ncbi.nlm.nih.gov/35305969/

Interstitial Lung Disease https://www.medscape.com/viewarticle/408750

The Role of Pulmonary Rehabilitation in Idiopathic Pulmonary Fibrosis: An Overview of Systematic Reviews https://pubmed.ncbi.nlm.nih.gov/38127914/

Antifibrotic Therapy for Idiopathic Pulmonary Fibrosis: Time to Treat https://pubmed.ncbi.nlm.nih.gov/31492155/

Dr Jeffrey Swigris and Susan Jacobs discuss considerations around supplemental oxygen for patients with idiopathic pulmonary fibrosis.

Relevant disclosures can be found with the episode show notes on Medscape https://www.medscape.com/viewarticle/999858. The topics and discussions are planned, produced, and reviewed independently of advertisers. This podcast is intended only for US healthcare professionals.

Resources

Idiopathic Pulmonary Fibrosis (IPF) https://emedicine.medscape.com/article/301226-overview

Summary for Clinicians: Clinical Practice Guideline on Home Oxygen Therapy for Adults With Chronic Lung Disease https://pubmed.ncbi.nlm.nih.gov/33857391/

Interstitial Lung Disease: Pathophysiology and Genetic Predisposition https://www.medscape.com/viewarticle/408750

The Role of Pulmonary Rehabilitation in Idiopathic Pulmonary Fibrosis: An Overview of Systematic Reviews https://pubmed.ncbi.nlm.nih.gov/38127914/

In this podcast, Drs Jeffrey Swigris and Toby Maher discuss the future of idiopathic pulmonary fibrosis. Find out where we're headed regarding diagnostic and treatment innovations on the near horizon.

Relevant disclosures can be found with the episode show notes on Medscape (https://www.medscape.com/viewarticle/982426). The topics and discussions are planned, produced, and reviewed independently of advertisers. This podcast is intended only for US healthcare professionals.

Resources

Idiopathic Pulmonary Fibrosis: Present Understanding and Future Options https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9584113/

Predicting Prognosis in Idiopathic Pulmonary Fibrosis https://pubmed.ncbi.nlm.nih.gov/21319590/

Trial of a Preferential Phosphodiesterase 4B Inhibitor for Idiopathic Pulmonary Fibrosis https://pubmed.ncbi.nlm.nih.gov/35569036/

Inhaled Treprostinil and Forced Vital Capacity in Patients With Interstitial Lung Disease and Associated Pulmonary Hypertension: A Post-hoc Analysis of the INCREASE Study https://pubmed.ncbi.nlm.nih.gov/34214475/

Pulmonary Fibrosis Foundation https://www.pulmonaryfibrosis.org/

Predictors of Progression in Systemic Sclerosis Patients With Interstitial Lung Disease https://pubmed.ncbi.nlm.nih.gov/32079645/

Dr Jeffrey J. Swigris and Professor Anne Holland discuss the finer points of oxygen therapy for patients with idiopathic pulmonary fibrosis.

Relevant disclosures can be found with the episode show notes on Medscape (https://www.medscape.com/viewarticle/982425). The topics and discussions are planned, produced, and reviewed independently of advertisers. This podcast is intended only for US healthcare professionals.

Resources

Home Oxygen Therapy for Adults With Chronic Lung Disease: An Official ATS Clinical Practice Guideline Implementation Tools https://www.thoracic.org/statements/guideline-implementation-tools/home-oxygen-therapy-for-adults.php

The King's Brief Interstitial Lung Disease (KBILD) Questionnaire: An Updated Minimal Clinically Important Difference https://bmjopenrespres.bmj.com/content/6/1/e000363

Ambulatory Oxygen for Treatment of Exertional Hypoxaemia in Pulmonary Fibrosis (PFOX Trial): A Randomised Controlled Trial https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7737108/

Drs Jeffrey Swigris and Meena Kalluri discuss palliative care practices that ameliorate idiopathic pulmonary fibrosis–related symptom burden for patients and their caregivers.

Relevant disclosures can be found with the episode show notes on Medscape (https://www.medscape.com/viewarticle/982424). The topics and discussions are planned, produced, and reviewed independently of advertisers. This podcast is intended only for US healthcare professionals.

Resources

Pharmacological and Nonpharmacological Interventions to Improve Symptom Control, Functional Exercise Capacity and Quality of Life in Interstitial Lung Disease: An Evidence Synthesis https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7836673/

The Multidisciplinary Approach in the Diagnosis of Idiopathic Pulmonary Fibrosis: A Patient Case-Based Review https://err.ersjournals.com/content/24/135/69

Assessing Exertional Dyspnea in Patients With Idiopathic Pulmonary Fibrosis https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3951298/

Drs Jeffrey Swigris and Marlies Wijsenbeek discuss how to best manage care for patients with idiopathic pulmonary fibrosis using remote monitoring to address quality-of-life concerns.

Relevant disclosures can be found with the episode show notes on Medscape (https://www.medscape.com/viewarticle/982423). The topics and discussions are planned, produced, and reviewed independently of advertisers. This podcast is intended only for US healthcare professionals.

Resources

Remote Monitoring in Idiopathic Pulmonary Fibrosis: Home Is Where the Bluetooth-Enabled Spirometer Is https://www.atsjournals.org/doi/full/10.1164/rccm.202005-1532ED

Challenges in Pulmonary Fibrosis – 1: Use of High Resolution CT Scanning of the Lung for the Evaluation of Patients With Idiopathic Interstitial Pneumonias https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2117220/

Spirometry: Step by Step https://breathe.ersjournals.com/content/8/3/232