Condition: Fibrosis, Pulmonary · Sponsor: University of Modena and Reggio Emilia
Idiopathic Pulmonary Fibrosis (IPF) is a fibrosing progressive interstitial lung disease with unknown etiology, with a median survival of 3 years since first diagnosis. The typical radiologic pattern of the disease is usual interstitial pneumonia (UIP) defined by basal and peripheral (subpleural) predominance and a typical cystic degeneration of lung parenchyma (honeycombing), interstitial fibrotic thickening and traction bronchiectasis. Despite the recent introduction of two antifibrotic treatments (Pirfenidone and Nintendanib) which proved to be successful in slowing the decline of pulmonary function in patients with IPF, a benefit of these therapies on average survival remains yet to be demonstrated. A significant part of patients affected by IPF die due to progressive worsening of respiratory failure, often accelerated by the insurgence of acute events, like acute exacerbations. Processes leading to the development and progression of IPF are not yet completely understood. We might hypothesize a regenerative deficit in the lungs of subjects affected, due to a dysregulation of repair mechanism in response to repeated damage (inflammatory, mechanics, infectious, chemical) to the alveolar and vascular epithelium. Moreover, mechanism of damage caused by aging in tissues, with a dysfunction in resident stem cell, might contribute to progression. Patients with IPF undergo mechanical alterations of respiratory system due to progressive restrictive deficit caused by reduction in …
This description comes directly from the study's public registry record.
Roberto Tonelli, MD · +390594225934 · roberto.tonelli@unimore.it
Alessandro Marchioni, MD · +390594225859 · marchioni.alessandro@unimore.it
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| Azienda Ospedaliero Universitaria Policlinico di Modena | Modena, Italy | Recruiting |
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Source record: clinicaltrials.gov/study/NCT05310669