Transcriptomic profile of airway-centered myofibroblastic areas as potential biomarkers of bronchiocentric interstitial pneumonia

Abstract

Interstitial lung diseases (ILDs) are a large heterogenic group of disorders that cause scarring with architectural distortion of pulmonary interstitium. Nonspecific clinical, radiological and pathological findings could not allow an accurate diagnosis despite the multidisciplinary discussion (MDD) with pneumologists, thoracic radiologist and pulmonary pathologist. The bronchiolocentric interstitial pneumonitis (BIP), which is defined by a predominantly bronchiolocentric fibrosing remodeling associated with bronchiolar or peribronchiolar lymphocytic infiltrate and peribronchiolar metaplasia as well as a patchy interstitial fibrosis and/or focal plugs of fibromyxoid connective tissue, is a new pattern related to a wide range of differential diagnosis, specially aspiration, collagenosis, auto-immune diseases, toxic-drug effects, pneumoconiosis, smoking, idiopathic and others. Furthermore, this disease is associated with high morbid-mortality. Therefore, the early etiologic diagnosis is critical to improve the best treatment and outcome of these patients. This project will study molecular screnning of space-activity injury areas, where the early specific mechanism is present, to validate translational diagnostic biomarkers. The molecular pathways of space-activity injury areas will reveal detailed pathophysiologic mechanisms of interstitial lung diseases in an etiology-related manner. The molecular diagnostic biomarker to surgical lung biopsy will improve patients' management due to a particular and unique lung interstitial disease. The outcome and treatment will be focused on the etiologic cause, providing a precision medicine. New therapeutic strategies and new drugs can be developed with better cost-benefit and withtherapeutic efficacy. (AU)