Fibrotic Interstitial Lungs Diseases

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.

Fibrotic interstitial lungs diseases (ILD) are a group of chronic and rare diseases characterized by an altered blood oxygenation in the lung, leading to dyspnea and physical limitation. Physical training is efficient to fight vicious circle of physical deconditioning observed in ILD. Eccentric cycling is an interesting modality of endurance training, allowing better exercise tolerance and adherence while developing cardiorespiratory function. Feasibility and efficacity of eccentric cycling were shown in chronic obstructive pulmonary disease but never in ILD. The primary aim of our work is to compare concentric and eccentric cycling training on physical capacities in ILD. Moreover, we will study the effects of training on the different steps of oxygen transport at exercise, from the lung to the muscles through the blood transport. Our second goal is then to characterize the physiological mechanisms underlying the benefits of exercising in ILD patients. Evaluations will be led during one year to study the long-term effects of training