Omics profile of active organizing myofibroblastic centrilobular areas as potential early diagnostic biomarkers of bronchiocentric interstitial pneumonia

• Grant number: 19/01517-3
• Support Opportunities: Regular Research Grants
• Duration: December 01, 2019 - May 31, 2022
• Field of knowledge: Health Sciences - Medicine - Medical Clinics
• Principal Investigator: Alexandre Todorovic Fabro
• Grantee: Alexandre Todorovic Fabro
• Host Institution: Faculdade de Medicina de Ribeirão Preto (FMRP). Universidade de São Paulo (USP). Ribeirão Preto , SP, Brazil
• Associated researchers: Agma Juci Machado Traina ; Claudia Aparecida Rainho ; José Antônio Baddini Martinez ; Marcel Koenigkam Santos ; Paulo Mazzoncini de Azevedo Marques ; Rosane de Oliveira Duarte Achcar ; Tales Rubens de Nadai ; Tomonori Tanaka ; Vera Luiza Capelozzi

Abstract

Interstitial lung diseases (ILDs) represents a broad and diverse spectrum group of disorders that cause variable degrees of interstitial inflammation and scarring, which may ultimately promote dramatic remodeling of the underlying pulmonary parenchyma architecture. Classification of ILD may be a challenging problem and although multidisciplinary discussions (MDD) by clinicians, radiologists, and pathologists have improved agreement and diagnostic confidence, many cases remain difficult to classify. Bronchiolocentric interstitial pneumonitis (BIP) is a chronic interstitial lung disease characterized by variable amounts of inflammation and scarring, accentuation around airways (airway-centered), variable amounts of peribronchiolar metaplasia, and a minor organizing pneumonia component. Possible etiologies include smoking, chronic bronchopulmonary aspiration, a pulmonary manifestation of underlying systemic autoimmune/collagen vascular disease, hypersensitivity pneumonitis, drug toxicity, some dust exposures, idiopathic disease, among other conditions. This disease is associated with high morbidity and mortality and accurate early diagnosis is critical to improving effective treatment outcomes. This project will study the molecular profile of active organizing myofibroblastic centrilobular areas, where the specific mechanism for lung remodeling is present and validate translational diagnostic biomarkers. The molecular pathways of these injury areas will reveal detailed pathophysiologic mechanisms of interstitial lung diseases in an etiology-related manner. Molecular diagnostic biomarkers will play a vital role to support bronchiolocentric ILD specific diagnosis and etiology, which will be of paramount importance in patient's treatment and outcome. New therapeutic strategies and new drugs can be developed with better cost-benefit and with therapeutic efficacy. (AU)