We recommend research into the efficacy, effectiveness, and safety of nintedanib in specific types of non-IPF ILD manifesting PPF.

May 1, 2022 · 2) PPF: PPF was defined as at least two of three criteria (worsening symptoms, radiological progression, and physiological progression) occurring within the past year with no alternative explanation in a patient with an ILD other than IPF.

Idiopathic pulmonary fibrosis (IPF) is the most common type of idiopathic interstitial lung disease. But there are multiple forms of ILD that are idiopathic. Your doctor will use detailed X-rays of your lungs called high-resolution computed tomography (HRCT) and sometimes a lung biopsy to look for a specific pattern of scarring on your lungs ...

Feb 26, 2025 · Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease (ILD) associated with loss of lung function and high mortality. 1 Decline in FVC or diffusing capacity of the lungs for carbon monoxide (D lco) % predicted in patients with IPF is indicative of disease progression, and these lung function parameters are ...

FIBRONEER™-ILD was a double-blind, randomized, placebo-controlled trial evaluating the efficacy and safety of nerandomilast (BI 1015550) over at least 52 weeks in patients with PPF. Primary endpoint: Absolute change from baseline in FVC (mL) at …

Feb 28, 2025 · Background Idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) are interstitial lung diseases (ILD) that carry a high burden and mortality. IPF/PPF experts and patients call for standardized care, outcome harmonization and holistic management in these complex and devastating diseases, with a focus on person-centeredness. In this cross-sectional international survey ...

A new drug has shown positive results in a clinical trial for people with progressive pulmonary fibrosis (PPF). The results from the FIBRONEER™-ILD trial could represent an important step forward for the pulmonary fibrosis community.

Feb 28, 2025 · A subset of patients with fibrosing interstitial lung disease (ILD) develop progressive pulmonary fibrosis [].The term progressive pulmonary fibrosis, or PPF, is generally used to describe progressive lung fibrosis in patients with an ILD other than idiopathic pulmonary fibrosis (IPF) [].Various criteria have been proposed to define PPF, based on decline in lung function, worsening radiologic ...

Oct 9, 2024 · For patients with interstitial lung diseases (ILDs) presenting with a progressive pulmonary fibrosis (PPF) phenotype, current knowledge of disease characteristics at diagnosis, patient journey, and treatment is limited.

Patients were divided into four ILD diagnosis groups: Idiopathic pulmonary fibrosis (IPF), Idiopathic Interstitial Pneumonia (IIP), Connective Tissue Disease-associated ILDs (CTD-ILD), and Other based on available data and sample sizes (Supplementary Table 2).