Aerodone (aerosol pirfenidone) / Avalyn Pharma - LARVOL DELTA

Nebulized Pirfenidone (AP01) for Progressive Pulmonary Fibrosis in Connective Tissue Disease-associated and Other Interstitial Lung Diseases: 4-year Data from the ATLAS Open Label Extension Trial (ACR Convergence 2026) - No abstract available

Clinical • Immunology • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases • Rheumatology

Avalyn Announces Multiple Presentations at the European Respiratory Society International Congress 2026 (GlobeNewswire) - "Presentations describe integration of patient perspective into clinical trial design and data modeling AP01 distribution in the lungs."

Clinical • Idiopathic Pulmonary Fibrosis

MIST: A Study Evaluating the Safety and Efficacy of Inhaled AP01 in Participants With Progressive Pulmonary Fibrosis (clinicaltrials.gov) - P2 | N=375 | Active, not recruiting | Sponsor: Avalyn Pharma Inc. | Recruiting ➔ Active, not recruiting

Enrollment closed • Fibrosis • Immunology • Inflammation • Inflammatory Arthritis • Interstitial Lung Disease • Pneumonia • Pulmonary Disease • Respiratory Diseases • Rheumatoid Arthritis • Rheumatology • Scleroderma • Systemic Sclerosis

Avalyn Pharma,,,announced that it has completed target enrollment of 375 patients in the Phase 2b MIST clinical trial of AP01 (inhaled pirfenidone) in patients with progressive pulmonary fibrosis (PPF) (GlobeNewswire) - "Additional patients currently in screening remain eligible for randomization and may be enrolled in the trial over the next several weeks....MIST is a global, randomized, double-blinded, placebo-controlled Phase 2b clinical trial evaluating the safety and efficacy of two doses of AP01 in patients with PPF....Company expects to report topline 12-month clinical results in the second half of 2027."

Enrollment status • P2b data • Fibrosis • Interstitial Lung Disease

Nebulized pirfenidone (AP01) for progressive pulmonary fibrosis in connective tissue disease-associated and other interstitial lung diseases: 4-year data from the ATLAS open label extension trial (EULAR 2026) - P1/2, P2 | "Of the full PPF cohort, 3 (10.7%) were on background nintedanib. Despite limitations in assessing efficacy outcomes from this study, AP01 showed a favourable FVC trajectory among patients with PPF. AP01 is now being studied in the MIST study (NCT06329401), a phase 2b, randomised, placebo-controlled trial for patients with PPF, with study completion expected in 2027."

Clinical • Gastroenterology • Gastrointestinal Disorder • Idiopathic Pulmonary Fibrosis • Immunology • Infectious Disease • Inflammatory Arthritis • Interstitial Lung Disease • Pneumonia • Pulmonary Disease • Rare Diseases • Respiratory Diseases • Rheumatoid Arthritis • Rheumatology • Scleroderma • Sjogren's Syndrome • Systemic Sclerosis

Access to Pirfenidone Solution for Inhalation (AP01) for Treatment of Progressive, Fibrosing Interstitial Lung Diseases, including Idiopathic Pulmonary Fibrosis (ANZCTR) - P2 | N=100 | Completed | Sponsor: Avalyn Pharma Pty Ltd | Active, not recruiting ➔ Completed

Trial completion • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases

Avalyn Announces Additional Long-term Data on AP01, Inhaled Pirfenidone, for the Treatment of Pulmonary Fibrosis to be Presented at the European Alliance of Associations for Rheumatology 2026 (The Manila Times) - "Updated findings from the ATLAS open-label extension trial will highlight the long-term tolerability profile of AP01 (inhaled pirfenidone) among the cohort of patients with progressive pulmonary fibrosis at up to four years of treatment."

P1 data • Idiopathic Pulmonary Fibrosis

Improved quality of life, reduced quantitative lung fibrosis in a trial of inhaled pirfenidone for idiopathic pulmonary fibrosis. (PubMed, BMC Pulm Med) - No abstract available

HEOR • Journal • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases

Avalyn Announces Publication Demonstrating Correlation Between Imaging, Improved Lung Function, and Quality of Life with AP01 in Patients with Idiopathic Pulmonary Fibrosis (The Manila Times) - "More than 70% of patients who received AP01 100 mg twice daily and had imaging data at both baseline and Week 24 achieved stabilization or improvement in fibrosis score on HRCT at week 24, with some demonstrating reductions in fibrotic volume (as measured by Quantitative Lung Fibrosis), and increases in whole lung volume; Dose-dependent reductions in quantitative ground glass (QGG), a marker associated with early fibrosis or potentially reversible lung injury, supporting the opportunity for AP01 for earlier intervention and broader patient impact; Quantitative analysis further confirmed patients with HRCT-documented fibrosis improvement experienced the greatest and most rapid QOL gains....Additional data, including longitudinal findings from the company’s ongoing open-label extension trial, are expected in mid-2026."

P1 data • P2 data • Idiopathic Pulmonary Fibrosis

Systematic Approach in Developing a User-centric Nebulizer for the Treatment of Patients Living With Pulmonary Fibrosis: The First Formative Ap01 Eflow Nebulizer System Usability Study (ATS 2026) - No abstract available

Clinical • Immunology • Pulmonary Disease • Respiratory Diseases

AP-LTE-008: An Open-Label Extension Study of Subjects who Received an Avalyn InhalednAntifibrotic Agent (clinicaltrialsregister.eu) - P1/2 | N=121 | Recruiting | Sponsor: Avalyn Pharma Inc.

New P1/2 trial • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases

Design of the MIST study: a double-blind, randomised, placebo-controlled phase 2b trial of pirfenidone solution for inhalation in patients with progressive pulmonary fibrosis. (PubMed, BMJ Open Respir Res) - P2 | "The main secondary endpoints are change in quality of life measurements from baseline to 52 weeks, time to disease progression and change in lung fibrosis scores based on high-resolution CT from baseline to 52 weeks. NCT06329401."

Clinical protocol • Journal • P2b data • Cough • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases

MIST: A Study Evaluating the Safety and Efficacy of Inhaled AP01 in Participants With Progressive Pulmonary Fibrosis (clinicaltrials.gov) - P2 | N=375 | Recruiting | Sponsor: Avalyn Pharma Inc. | Trial completion date: Apr 2026 ➔ Jun 2027 | Trial primary completion date: Apr 2026 ➔ Jun 2027

Trial completion date • Trial primary completion date • Immunology • Pulmonary Disease • Respiratory Diseases

Inhaled pirfenidone as an innovative therapeutic approach to treat autoimmune ILD and other forms of Progressive Pulmonary Fibrosis: Phase 2b Study Design (ACR Convergence 2025) - "Patients will remain on stable background immunosuppression, and up to 30% of patients will remain on background nintedanib therapy. MIST is the first study to analyze the novel concept of inhaled medications in autoimmune ILD. In addition to the safety and efficacy endpoints, MIST will examine the presence of cough in this population of patients."

P2b data • Cough • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases

Avalyn Announces Multiple Presentations at the Pulmonary Fibrosis Foundation (PFF) Summit 2025 (GlobeNewswire) - "Presentations to Highlight Patient Experience Research, AURA-IPF Phase 2 Trial Design for AP02, and Long-Term Safety Data for AP01."

Clinical protocol • P2b data • Idiopathic Pulmonary Fibrosis

Avalyn Pharma Secures $100 Million in Series D Funding, Pioneering Pulmonary Fibrosis Treatment with Inhalation Therapy (36Kr) - "The funds from this round will primarily support Avalyn's key clinical progress in the field of pulmonary fibrosis treatment. This includes advancing the global Phase IIb clinical trial of its core candidate drug AP01 (inhaled pirfenidone), the late-stage development of AP02 (inhaled nintedanib), and the entry of AP03 (pirfenidone/nintedanib fixed-dose combination) into the Phase I clinical stage."

Commercial • Idiopathic Pulmonary Fibrosis • Interstitial Lung Disease • Systemic Sclerosis

An Extension Study of Subjects Who Received an Avalyn Inhaled Antifibrotic Agent (SAIL) (clinicaltrials.gov) - P2 | N=340 | Recruiting | Sponsor: Avalyn Pharma Inc. | Not yet recruiting ➔ Recruiting

Enrollment open • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases

Avalyn Completes Oversubscribed $100 Million Series D Financing to Further Advance Clinical Development of Novel Inhaled Therapies for Pulmonary Fibrosis (GlobeNewswire) - "Avalyn Pharma Inc...announced the closing of an oversubscribed $100 million Series D financing...Proceeds will support key clinical milestones across Avalyn’s pipeline programs in pulmonary fibrosis, including the late-stage development of Avalyn’s lead programs: optimized inhaled formulations of pirfenidone (AP01) and nintedanib (AP02), as well as the advancement of the pirfenidone/nintedanib fixed dose combination (AP03) into Phase 1 clinical trials....The company plans to initiate a Phase 2 clinical trial for AP02 in patients with IPF."

Financing • New P2 trial • Idiopathic Pulmonary Fibrosis

Inhaled Pirfenidone as an innovative therapeutic approach to treat autoimmune ILD and other forms of Progressive Pulmonary Fibrosis: Phase 2b Study Design (EULAR 2025) - "Patients will remain on stable background immunosuppression and up to 30% of patients will remain on background nintedanib therapy. MIST is the first study to analyze the novel concept of inhaled medications in autoimmune ILD. Safety and efficacy of AP01 in patients with PPF will be studied. In addition to the safety and efficacy endpoints, MIST will examine the presence of cough in this population of patients."

P2b data • Cough • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases

Monte Carlo External Control Arm Generation Utilising Real-world Patient Data and Deep Learning-based Quantitative CT Metrics Demonstrates Treatment Effect in the Atlas IPF Trial (ATS 2025) - P1 | "We have shown a significant treatment effect for 100 mg BID of AP01 over 48 weeks, using external control arms generated by MCCV, based on matched clinical and quantitative CT-based variables. This represents the first known application of quantitative CT in creating a matched external control arm utilising these methods, signalling a paradigm shift in the investigation of novel drug candidates for IPF in clinical trials."

Clinical • Late-breaking abstract • Real-world • Real-world evidence • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology

Dose-Dependent Change of Inhaled Pirfenidone Seen in Lung Volume and Fibrosis Quantification in Patients With IPF: A Deep Learning Image-Based Analysis of Data From the ATLAS Phase 1b Trial (ATS 2025) - P1 | "Patients ineligible for, intolerant of, or unwilling to start oral pirfenidone or nintedanib were randomised 1:1 to receive nebulised AP01 50mg QD or 100mg BID for up to 72 weeks... Patients on the higher dose regime of 100mg AP01 showed a smaller lung volume reduction at week 36 and reduced fibrosis progression at week 24 compared to the 50mg dose. There was a trend across both time points to less fibrosis extent progression in the higher dose regime. Deep learning-based imaging biomarkers can be used to quantify dose-dependent effects on disease progression and suggest a dose-dependent effect of AP01 in patients with IPF."

Clinical • P1 data • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology

Long-term Safety and Efficacy Data With Inhaled Pirfenidone (AP01) in the Atlas Open-label Extension Study Up to 240 Weeks (ATS 2025) - "Conclusions Over up to 240 weeks follow up, AP01 appears to have continuing efficacy with a stable safety profile over the long-term of open-label treatment. This data provides support for the ongoing evaluation of AP01 in Phase 2 and 3 studies."

Clinical • Cough • Idiopathic Pulmonary Fibrosis • Immunology • Interstitial Lung Disease

Title: Dose-Dependent Change of Inhaled Pirfenidone Seen in Lung Volume and Fibrosis Quantification in Patients With IPF: A Deep Learning Image-Based Analysis of Data from the ATLAS Phase 1b Trial (GlobeNewswire) - P1b | N=100 | ATLAS (ACTRN12618001838202) | Sponsor: Avalyn Pharma Pty Ltd | "Avalyn showcases clinical data across multiple pulmonary fibrosis programs at ATS 2025....Together with Qureight, Avalyn presented results from deep learning-based analyses of HRCT scans and patient data from the completed ATLAS trial of AP01. The Qureight platform analyzed HRCT scans from patients with IPF from the ATLAS study to quantify the volumes of the lungs, airways, pulmonary vessels, and fibrosis. Across all visits, HRCT-derived total lung volume strongly correlated with the forced vital capacity (FVC). Between the 50 mg QD and 100 mg BID cohorts, patients on the higher dose showed a smaller lung volume reduction at week 36 and reduced fibrosis progression at week 24 compared to the 50 mg QD dose. These data demonstrate dose-dependent effects of AP01 on anatomy through Qureight’s deep learning-based imaging biomarkers and efficacy as measured by FVC."

P1 data • Idiopathic Pulmonary Fibrosis

Title: Long-term Safety and Efficacy Data with Inhaled Pirfenidone (AP01) in the ATLAS Open-label Extension Study Up to 240 Weeks (GlobeNewswire) - P1b | N=100 | ATLAS (ACTRN12618001838202) | Sponsor: Avalyn Pharma Pty Ltd | "Avalyn showcases clinical data across multiple pulmonary fibrosis programs at ATS 2025....Avalyn’s open-label extension (OLE) trial enrolled a total of 100 patients, including 41 patients rolled over from the ATLAS study, 31 AP01-naïve patients with IPF, and 28 AP01-naïve patients with progressive pulmonary fibrosis (PPF)....Data from roll-over patients from the ATLAS study had an FVC change of -69 ml/year; naïve IPF patients had a FVC change of -120 ml/year, while naïve PPF patients had a FVC change of +11 ml/year. AP01 has continued to demonstrate a well-tolerated profile, with no new safety signals seen during weeks 144 to 240. These positive findings, demonstrating durable efficacy and stable safety up to approximately 4.5 years, are unprecedented as the median survival of pulmonary fibrosis is 3-5 years after diagnosis."

P1 data • Idiopathic Pulmonary Fibrosis

Title: Monte Carlo External Control Arm Generation Utilizing Real-world Patient Data and Deep Learning-based Quantitative CT Metrics Demonstrates Treatment Effect in the ATLAS IPF Trial (GlobeNewswire) - "Avalyn showcases clinical data across multiple pulmonary fibrosis programs at ATS 2025....Avalyn, in collaboration with Qureight, presented a late-breaking poster presentation on findings from a Monte Carlo Cross Validation (MCCV) approach to generate external placebo control arms using real-world data of treatment-naive IPF patients. The approach produced well-matched external placebo control arms for both treatment groups. The results, comparing the change in FVC between these external placebo control arms and the treatment arms, demonstrated a significant treatment effect for 100 mg BID over 48 weeks. This represents the first known application of quantitative HRCT in creating a matched external placebo control arm, signaling a potential paradigm shift in the investigation of novel drug candidates for IPF in clinical trials."

Real-world • Idiopathic Pulmonary Fibrosis