Progressive Pulmonary Fibrosis

Study RIN-PF-305 is designed to evaluate the safety and efficacy of inhaled treprostinil in subjects with progressive pulmonary fibrosis (PPF) over a 52-week period.

This is an open-label extension study for participants who were previously enrolled in and completed an Avalyn Pharma Sponsored study with an inhaled antifibrotic, such as AP01. Eligible participants will have their final dose of drug at the end of study visit from the lead-in study and first AP-LTE-008 study visit on the same day.

This study is open to people with idiopathic pulmonary fibrosis (IPF) or progressive pulmonary fibrosis (PPF). They can only take part if they have completed treatment in a previous study with a medicine called nerandomilast or BI 1015550. The goal of this study is to find out how well people with pulmonary fibrosis tolerate long- term treatment with nerandomilast. The study also tests whether nerandomilast improves lung function and prolongs the time until symptoms get worse, participants need to go to the hospital, or die. Every participant takes nerandomilast as tablets for up to 1 year and 10 months. The participants may also continue their regular treatment for pulmonary fibrosis during the study. Participants visit their doctors regularly. During these visits, the doctors collect information on any health problems of the participants. Participants also regularly do lung function tests.

Study RIN-PF-305 is designed to evaluate the safety and efficacy of inhaled treprostinil in subjects with progressive pulmonary fibrosis (PPF) over a 52-week period.

The OPTIMIZE-ILD-2 trial is a prospective, randomized, open-label clinical trial designed to evaluate the impact of a coordinated follow-up pathway on patients with established interstitial lung disease (ILD). In routine clinical practice, follow-up workflows for ILD are frequently fragmented, requiring multiple hospital visits for pulmonary function tests, laboratory analysis, treatment administration, and consultations with various specialists, which increases the burden for both patients and caregivers. This study compares the standard follow-up care against an optimized circuit where all routine monitoring procedures and interdisciplinary consultations are pre-bundled and scheduled within a single, coordinated hospital visit. All eligible patients under active ILD follow-up are included consecutively to ensure a pragmatic, real-world representation of the treated ILD population. The primary objective is to measure the total follow-up time burden, defined as the total home-to-home time required to complete the follow-up circuit. As a cross-sectional assessment within a longitudinal context, secondary objectives include assessing socioeconomic cost-burden, the environmental carbon footprint of the follow-up journey, health-related quality of life, and clinical frailty. Caregiver-related outcomes, including burden and experience measures, are contingent upon the presence of a primary caregiver and the provision of their independent informed consent. The design of this protocol was informed by a patient focus group and is officially endorsed by the 'AIRE' Associació Catalana de Malalts i Trasplantats Pulmonars, ensuring a patient-centered approach that prioritizes follow-up efficiency and human impact.

This study is open to adults with PPF . The main objective is to evaluate of the efficacy and the secondary objective is to evaluate the safety and pharmacokinetic.

Fibrotic interstitial lung diseases (F-ILDs), including both idiopathic pulmonary fibrosis (IPF) and non-IPF, are chronic and progressive lung diseases characterized by excessive scarring of lung tissue, leading to declining lung function, respiratory failure, and high mortality, despite the currently approved antifibrotic treatment. While its exact cause remains unknown, pulmonary fibrosis is strongly linked to aging, genetic predisposition, environmental factors, and cellular senescence. Ongoing research aims to identify reliable biomarkers and develop targeted treatments to enhance patient outcomes. This randomized controlled trial will examine the effects of quercetin supplementation (500 mg/day for two 12-week cycles, with one 8-week washout periods) on telomere length, senescence-associated secretory phenotype (SASP) factors, and lung function in patients with IPF and F-ILDs. A total of 100 patients will be recruited, with half receiving quercetin (despite their standard of care therapy) and the other half receiving standard care (SOC). Primary outcomes will include changes in telomere length, SASP protein levels (IL-6, MMPs), fractional exhaled nitric oxide (FeNO), spirometry (FVC decline), and oscillometry measurements. Additionally, quality of life will be assessed using the L-IPF Questionnaire. This study aims to explore quercetin's potential to reduce fibrosis, decrease inflammation, and improve lung function in F-ILDs, offering new insights into potential novel strategies for F-ILD management.

This pilot randomized controlled trial evaluates the feasibility, acceptability, and accessibility of SUPPORT-T, a digitally delivered palliative care intervention for patients with Progressive Pulmonary Fibrosis (PPF) and their caregivers. The intervention includes a 9-week digital application guided by a nurse interventionist and quarterly virtual support groups over one year. Participants will be recruited from rural South Carolina and urban New York City. The study compares SUPPORT-T to Enhanced Usual Care and aims to inform a future multi-site trial.

To assess the impact on body mass composition from anti-fibrotic medications used in fibrotic lung disease by using BIA and muscle ultrasound

This is a prospective, observational cohort study. Participants with non-idiopatic pulmonary fibrosis, interstitial lung disease (ILD) will be followed for 24 months to systematically collect clinical, imaging, and biospecimen data. The primary objective is to optimize progressive pulmonary fibrosis (PPF) classification and establish PPF incidence for key ILD subtypes. Additional exploratory objectives are to 1) Prospectively validate a novel PPF classifier and assess performance durability over time, and 2) Determine whether multi-dimensional PPF prediction outperforms component approaches.