Idiopathic pulmonary fibrosis (IPF) is a progressive scarring disease of the lungs with no known cause, gradually replacing healthy tissue with stiff scar tissue. Breathing becomes harder over time, and median survival after diagnosis has historically been 3–5 years, though two approved antifibrotic drugs have slowed decline for many patients.
What's actually going on in research
Two antifibrotic drugs — pirfenidone and nintedanib — are now standard care and slow lung function decline, but neither halts or reverses the disease. Trials are testing novel targets including lysophosphatidic acid receptor antagonists, integrin inhibitors, and TGFB pathway blockers. Autotaxin inhibitors and monoclonal antibodies targeting fibrotic signaling are in mid-stage trials, and researchers are also studying combination antifibrotic strategies.
Novel antifibrotic targets
Lysophosphatidic acid receptor antagonists and autotaxin inhibitors are being tested as alternatives or additions to existing antifibrotic drugs, aiming to slow fibrosis through distinct mechanisms.
Integrin and TGFB blockade
Monoclonal antibodies blocking integrin alphav and TGFB signaling pathways are in mid-stage trials, targeting the fibrotic cascade at steps upstream of current therapies.
Combination antifibrotic therapy
Trials are evaluating whether combining pirfenidone or nintedanib with newer agents produces greater slowing of decline than either drug alone, without adding unacceptable side effects.
What to know before you search
Eligibility typically depends on lung function measurements, disease duration, oxygen requirements, and whether prior antifibrotic therapy was used.
What types of trials are currently open
- Antifibrotic drug trials — Testing new drugs and combinations that slow or halt progressive lung scarring.
- Biomarker trials — Identifying blood and imaging markers to predict disease progression and guide treatment decisions.
- Oxygen and rehabilitation trials — Studying pulmonary rehabilitation and supplemental oxygen timing to improve quality of life.
- Lung transplant trials — Improving outcomes before and after lung transplantation in IPF patients.
- Stem cell trials — Early-phase studies testing whether cell-based therapies can reduce fibrosis.
Recently added Idiopathic Pulmonary Fibrosis trials
Advanced Imaging to Assess the Effect of Immunosuppression on Progressive Fibrosis
The purpose of this study is to investigate how immunosuppression treatment affects measurements of active collagen deposition using \[68Ga\]CBP8 positron emission tomography (PET) and tissue injury using dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) in individuals with non-idiopathic pulmonary fibrosis interstitial lung disease (non-IPF ILD).
Optimizing the Follow-Up Journey in Interstitial Lung Disease: The OPTIMIZE-ILD-2 Trial
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.
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