Teng Moua, M.D.

The primary purpose of this study is to see how GLPG1690 works together with current standard treatment on lung function and IPF disease in general. The study will also investigate how well GLPG1690 is tolerated (side effects).

Systemic Sclerosis (SSc) is a devastating disease of unknown etiology. Patients suffer from multiple organ fibrosis whereas lung fibrosis (interstitial lung disease, ILD) is one of the main driver for mortality. There is preclinical evidence for efficacy of nintedanib in SSc and associated ILD (SSc-ILD) and the anti-fibrotic efficacy of nintedanib was proven in idiopathic pulmonary fibrosis patients, who are presenting a similar pattern regarding lung fibrosis. Hence it is the purpose of the trial to confirm the efficacy and safety of nintedanib 150 mg bid in treating patients with SSc-ILD, compared with placebo. The trial will be conducted as a double blind, randomised, placebo-controlled trial with primary efficacy evaluation at week 52 and placebo-controlled treatment until last patient out (up to a maximum of 100 weeks). Respiratory function is globally accepted for assessment of treatment effects in patients with lung fibrosis. The chosen endpoint (Forced Vital Capacity (FVC) decline) is easy to obtain and is part of the usual examinations done in patients with SSc-ILD.

The aim of the current study is to investigate the efficacy and safety of nintedanib over 52 weeks in patients with Progressive Fibrosing Interstitial Lung Disease (PF-ILD) defined as patients who present with features of diffuse fibrosing lung disease of >10% extent on high-resolution computed tomography (HRCT) and whose lung function and respiratory symptoms or chest imaging have worsened despite treatment with unapproved medications used in clinical practice to treat ILD. There is currently no efficacious treatment available for PF-ILD. Based on its efficacy and safety in Idiopatic Pulmonary Fibrosis (IPF), it is anticipated that Nintedanib will be a new treatment option for patients with PF-ILD.

The purpose of this trial is to demonstrate proof of concept of clinical activity of BI 1015550 on the change of Forced Vital Capacity (FVC) between baseline and 12 weeks.  New treatments are needed that further reduce the decline in FVC, positively affect symptoms and improve quality of life in patients with Idiopathic Pulmonary Fibrosis.  This trial will  investigate BI 1015550 to be used in this patient population either as stand-alone treatment or in addition to local standard of care (SoC), which may or may not include currently approved antifibrotic treatments (nintedanib or pirfenidone). 

Mayo Clinic will not be participating in the optional Digital lung Auscultation Test substudy.

The main objective is to assess long term safety of treatment with oral nintedanib in patients with Systemic Sclerosis associated Interstitial Lung Disease (SSc-ILD).

The purpose of this study is to assess the long-term tolerability and safety of oral nintedanib treatment in patients with Progressive Fibrosing Interstitial Lung Disease who have completed (and did not prematurely discontinue trial medication in) the phase III parent trial, 1199.247 (INBUILD®).

The purpose of this study is to establish and validate testing for disease progression in peripheral mononuclear cells (PMBC) from patients with Chronic Hypersensitivity Pneumonitis (CHP) at initial presentation, and to validate blood tests over time to predict disease progression.

Chronic fibrosing interstitial lung disease has significant negative impact on functional status and quality of life through often progressive and debilitating symptom burden. Prior studies have used various surveillance tools for assessing patient reported parameters and attested to the utility of these measurements for directing therapy and predicting outcome. We hypothesize that directed prospective measurements of such physiologic, emotional, and psychologic domains may correlate with baseline or presenting functional parameter and be incorporated into disease progression or staging models, perhaps capable of predicting outcomes as well as be targets of directed medical and non-medical therapies.  

The purpose of the Pulmonary Fibrosis Foundation Patient Registry is to collect data on well-characterized patients with interstitial lung disease, especially idiopathic pulmonary fibrosis, for participation in retrospective and prospective research.

The purpose of this study is to compare the effect of standard care, versus standard of care plus antimicrobial therapy (co-trimoxazole or doxycycline), on clinical outcomes in patients diagnosed with idiopathic pulmonary fibrosis (IPF).

The main objectives of this study are to determine the difference in change from baseline in Six Minute Walk Distance (6MWD) when pulmonary rehabilitation (PR) is added to stable underlying nintedanib therapy in patients with idiopathic pulmonary fibrosis (IPF), to determine the difference in change in Quality of Life (QoL) when pulmonary rehabilitation (PR) is added to stable underlying nintedanib therapy in patients with idiopathic pulmonary fibrosis (IPF), and to determine if there is an enduring effect in 6MWD, QoL and lung function from pulmonary rehabilitation (PR) when pulmonary rehabilitation (PR) is added to stable underlying nintedanib therapy in patients with idiopathic pulmonary fibrosis (IPF).