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

Diagnosis criteria for AE-IPF:
Diagnosed IPF patient experiences unexplained dyspnea within 1 month
With objective evidence of hypoxia and new onset of pulmonary infiltration based on imaging examination
With other diagnosis like pulmonary embolism, pneumothorax or heart failure excluded.

Diagnosis criteria for Stable-IPF:
Exclusion of other known causes of ILDs
Presence of a usual interstitial pneumonitis (UIP) pattern on high-resolution computed tomography (HRCT)
Specific combinations of HRCT and surgical lung biopsy pattern in patients subjected to surgical lung biopsy.

Sputum, throat swab, BALF and blood will be collected in this study.

Interstitial Lung Diseases

No intervention

Shanghai Pulmonary Hospital, Tongji University

Study of the Etiology and Immunological Pathogenesis in Acute Exacerbation of Idiopathic Pulmonary Fibrosis (AE-IPF)

Testing of pathogen nucleic acid chips:Based on PathGEN® Pathogen Chip Kit (PathGEN Dx, Singapore). Next Generation Sequencing (NGS) has become the powerful tool in pathogen identification based on its rapidness and sensitivity. The final report will provide the species classification of the bacteria and viruses, as well as corresponding designator.

Subtype and functional testing on phenotype and function of Macrophage cell in IPF/AE-IPF patients.

Subtype and functional testing on phenotype and function of DC in IPF/AE-IPF patients.

Subtype and functional testing on phenotype and function of T cell in IPF/AE-IPF patients.

Subtype and functional testing on phenotype and function of B cell in IPF/AE-IPF patients.

The investigators will evaluate the expression of IL-17 by proteomics in IPF/AE-IPF patients.

The investigators will evaluate the expression of MIG by proteomics in IPF/AE-IPF patients.

Huiping Li

Chief Physician

Shanghai Pulmonary Hospital, Shanghai, China

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/headache

FIGHT-RP 1 Extension Study

Study of the Etiology and Immunological Pathogenesis in Acute Exacerbation of Idiopathic Pulmonary Fibrosis (AE-IPF)

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

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