Establishment and characterization of a novel fibroblastic cell line (Sci13d) derived from the broncho-alveolar lavage of a patient with fibrotic hypersensitivity pneumonitis

Paolo Giannoni, Marco Grosso, Giuseppina Fugazza, Mario Nizzari, Maria Cristina Capra, Rita Bianchi, Roberto Fiocca, Sandra Salvi, Fabrizio Montecucco, Maria Bertolotto, Franco Fais, Mario Salio, Emanuela Barisione, Daniela de Totero

Research output: Contribution to journalArticlepeer-review

Abstract

Hypersensitivity pneumonitis (HP) is a diffuse interstitial lung disease (ILD) caused by the inhalation of a variety of antigens in susceptible individuals. Patients with fibrotic HP (fHP) may show histopathological and radiological manifestations similar to patients with idiopathic pulmonary fibrosis (usual interstitial pneumonia-like pattern of fibrosis) that are associated with a worse prognosis. We describe here the establishment and characterization of a fibroblastic cell line derived from the broncho-alveolar lavage (BAL) of a patient with fHP, a 53 year old man who presented at our Pneumology Unit with cough and dyspnea. The fHP diagnosis was based on international criteria and multidisciplinary discussion. Primary fibroblasts were expanded in vitro until passage 36. These fibroblasts displayed morpho/phenotypical features of myofibroblasts, showing high positivity for α-smooth muscle actin, type I collagen, and fibronectin as determined by quantitative RT-PCR and cyto-fluorographic analysis. Cytogenetic analyses further evidenced trisomy of chromosome 10, which interestingly harbors the FGF2R gene. To our knowledge, this is the first fibroblastic cell line derived from an fHP patient and might, therefore, represent a suitable tool to model the disease in vitro. We preliminarily assessed here the activity of pirfenidone, further demonstrating a consistent inhibition of cells growth by this antifibrotic drug.

Original languageEnglish
Article number1193
JournalBiomedicines
Volume9
Issue number9
DOIs
Publication statusPublished - Sep 2021

Keywords

  • Fibrosis
  • Hypersensitivity pneumonitis
  • Myofibroblasts
  • Pirfenidone

ASJC Scopus subject areas

  • Medicine (miscellaneous)
  • Biochemistry, Genetics and Molecular Biology(all)

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