Whether magnetic resonance imaging (MRI) using inhaled hyper-polarized 129 Xenon gas can help visualize impaired lung function to detect changes over time in Idiopathic Pulmonary Fibrosis (IPF) patients receiving approved IPF treatments

Idiopathic Pulmonary Fibrosis

Hyperpolarized 129 Xenon Gas Comparing Idiopathic Pulmonary Fibrosis (IPF) Treatment

Duke University Medical Center

Genentech MRI Study

Using Xenon MRI to Evaluate the Efficacy of Therapies for Idiopathic Pulmonary Fibrosis

RBC:barrier ratio will be determined using 129 Xenon MRI. This ratio will be assessed in each individual prior to initiation of IPF therapy. Following initiation of therapy additional 129 Xenon MRIs will be performed at 3, 6 and 12 months. The outcome measure will be assessment of rate of RBC:barrier decline following initiation of therapy across the imaging studies from the initial 129 Xenon MRI performed prior to initiation of therapy.

Lung function will be assessed in all subjects. This will include forced vital capacity (FVC) and diffusion capacity for carbon monoxide (DLCO). The outcome measure will be for FVC decline >10% or DLCO decline >20% over the 12 months. These measures have been identified as surrogate measures of IPF outcomes in prior clinical studies.

Bastiaan Driehuys

Professor of Medicine

Duke University

All participants to receive study intervention.

Retinitis Pigmentosa

After completing the pretreatment visits, all patients will enter into the treatment phase where patients will receive 1800 mg of NAC effervescent tablets twice a day. The patients will be followed up every 3 months for 2 years. Therefore, there will be a total of 9 treatment visits (baseline, months 3, 6, 9, 12, 15, 18, 21 and 24).

NAC effervescent tablets

Wilmer Eye Institute at Johns Hopkins University

FIGHT-RP 1 Extension Study

A Phase 1 Open-Label Extension Study to Assess the Long-Term Safety and Tolerability of N-Acetylcysteine (NAC) in Patients With Retinitis Pigmentosa

Blood pressure in mmHg will be used in assessing tolerability of N-Acetylcysteine.

Participants will be assessed for the occurrence of any drug-related symptoms which includes nausea, stomach upset, diarrhea, heartburn, constipation, and vomiting. Based on the number and severity of the symptoms, the physician would conclude whether the patient is tolerating the medication or not.

Medication diary provided to participants will be reviewed to see if participants are taking the medication within the designated time frame.

Medication diary provided to participants will be reviewed to see if participants are taking the medication twice a day

Scoring is determined by the number of letters gained or lost per month using Early Treatment Diabetic Retinopathy Study (ETDRS) Letter Score and visual acuity score together with an overall score range of 0 to 20/20 where 0 is the worst vision and 20/20 is the best.

Microperimetry (MAIA) is used to test whether there is an increase or decrease in sensitivity (dB) in the retina after initiation of N-Acetylcysteine

This will be assessed by spectral domain optical coherence tomography (SD-OCT) after initiation of N-Acetylcysteine.

This will be calculated from the proportions of reduced glutathione and oxidized glutathione in the aqueous.

Johns Hopkins University

Treatment with low dose oral interferon alpha lozenges taken 3 times daily (approximately 6 hours apart). Lozenge is to be dissolved under tongue or by moving around in mouth.

Interferon Alpha

Sex: Female, Male

Region of Enrollment

Disease progression was determined by comparing results of the High Resolution Computed Tomography(HRCT) and pulmonary function at one year to the baseline HRCT & pulmonary function. The same radiologist did the comparsion for all subjects.

Minimal/no Progression (1 yr) by High Resolution Computed Tomography (HRCT) & Pulmonary Function

Minimal/no Change in Quality of Life

changes in cough status after treatment for 1 month.

Participants With Change in Cough

Overall Study

Lorenz Lutherer, MD, PhD

Respiratory Tract Diseases

Lung Diseases

Lung Diseases, Interstitial

Pulmonary Fibrosis

dose form - oral lozenge dose - 150 International Units (IU) frequency - 3 times a day duration - at least 1 year

idiopathic pulmonary fibrosis/تهوع

Genentech MRI Study

FIGHT-RP 1 Extension Study

Clinical Trial of Low Dose Oral Interferon Alpha in Idiopathic Pulmonary Fibrosis

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