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Related Concept Videos

Pulmonary Function Tests01:25

Pulmonary Function Tests

402
Pulmonary Function Tests (PFTs)
Pulmonary Function Tests are crucial diagnostic tools for assessing respiratory function, particularly in patients with chronic respiratory disorders. They comprehensively evaluate lung volumes, ventilatory function, breathing mechanics, diffusion, and gas exchange. These tests help diagnose pulmonary diseases and play a significant role in monitoring disease progression, evaluating disability, and assessing response to therapy.
PFTs involve using a spirometer, a...
402
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists01:23

Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists

233
Prostacyclin receptor agonists are a class of therapeutic agents integral to managing pulmonary arterial hypertension (PAH). These drugs operate by mimicking the action of prostaglandin I2, or PGI2, a naturally occurring compound in the body.
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
233
Inhaled Medications01:23

Inhaled Medications

333
Inhaled medications are crucial for managing chronic obstructive pulmonary disease (COPD) and asthma. They are essential for effective treatment and control, ensuring optimal respiratory health and well-being. Inhaled medication delivers drugs directly to the lungs, providing a rapid onset of action and reducing systemic side effects compared to oral or injectable medications. Three primary types of inhalation devices are used to administer these medications: nebulizers, metered-dose inhalers...
333
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure01:16

Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure

286
Oxygen therapy has emerged as a significant tool in enhancing the quality of life for patients suffering from pulmonary arterial hypertension (PAH). While this therapy has principally been studied on patients with significant hypoxemia, this therapeutic approach helps prevent potential organ damage and can be administered in the comfort of one's home.
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue,...
286
Pulmonary Tuberculosis V01:28

Pulmonary Tuberculosis V

220
Medical management of tuberculosis (TB) patients involves a comprehensive approach that includes diagnosis, treatment, and monitoring. The specific strategies can vary depending on the type of tuberculosis (latent or active), the patient's overall health status, and other considerations.
Latent tuberculosis infection occurs when TB bacteria are present in a person's body, but are not causing illness or symptoms. It is not contagious, and preventive treatment is crucial to avoid the...
220
Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors01:28

Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors

207
Phosphodiesterase 5 (PDE5) inhibitors are potent enzymes that function to hydrolyze cyclic nucleotides to their corresponding 5' monophosphates. Their unique biochemical properties have been applied in treating Pulmonary Arterial Hypertension (PAH).
Among the PDE5 inhibitors, sildenafil (Revatio) stands out as a competitive and selective inhibitor. It operates by elevating cellular levels of cGMP and augmenting signaling through the cGMP-PKG pathway, promoting vasodilation. Upon oral...
207

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Related Experiment Video

Updated: Aug 6, 2025

Refined Murine Model of Idiopathic Pulmonary Fibrosis
07:51

Refined Murine Model of Idiopathic Pulmonary Fibrosis

Published on: June 17, 2025

180

Inhaled pirfenidone solution (AP01) for IPF: a randomised, open-label, dose-response trial.

Alex West1, Nazia Chaudhuri2, Adam Barczyk3

  • 1Guy's and St Thomas' Hospital, London, UK.

Thorax
|March 22, 2023
PubMed
Summary
This summary is machine-generated.

Inhaled pirfenidone (AP01) shows promise for idiopathic pulmonary fibrosis (IPF) treatment, with fewer side effects than oral forms. The 100 mg twice-daily dose maintained lung function in a Phase 1b trial.

Keywords:
cough/mechanisms/pharmacologyidiopathic pulmonary fibrosis

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Area of Science:

  • Pulmonary Medicine
  • Pharmacology
  • Clinical Trials

Background:

  • Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease.
  • Oral pirfenidone is effective but associated with significant systemic side effects.
  • Lower doses of oral pirfenidone may not be sufficiently effective.

Purpose of the Study:

  • To assess the safety, tolerability, and efficacy of inhaled pirfenidone (AP01) in IPF patients.
  • To evaluate dose-response relationship of inhaled pirfenidone.
  • To compare inhaled pirfenidone with oral antifibrotic treatments.

Main Methods:

  • Phase 1b, randomized, open-label, dose-response trial.
  • 25 sites in six countries; ACTRN12618001838202.
  • 91 IPF patients received nebulized AP01 (50 mg once daily or 100 mg twice daily) for up to 72 weeks.

Main Results:

  • Most treatment-related adverse events were mild/moderate (cough, rash, nausea).
  • Week 48: Forced vital capacity (FVC) decline was -4.9 mL in the 50 mg group and -34 mL in the 100 mg group.
  • The 100 mg twice-daily dose group showed stable mean FVC % predicted.

Conclusions:

  • Inhaled pirfenidone (AP01) demonstrated a favorable safety profile with less frequent side effects compared to oral pirfenidone.
  • The 100 mg twice-daily dose of inhaled pirfenidone appears to stabilize lung function in IPF patients.
  • Further investigation of inhaled pirfenidone for IPF treatment is warranted.