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.
Highlights
Progressive fibrosing interstitial lung diseases (ILDs) are associated with high mortality; in particular, patients developing the fibrotic form of hypersensitivity pneumonitis have a poor prognosis
The CT scan showed an alternative diagnosis pattern according to the Official ATS/ERS/JRS/ALAT clinical practice guidelines [18], with predominant ground glass opacity and peribronchovascular distribution
We further show here higher mRNA levels of the receptor for FGF2 in SCI13D than in the lung control cell line, which could represent an advantage for cell proliferation
Summary
Progressive fibrosing interstitial lung diseases (ILDs) are associated with high mortality; in particular, patients developing the fibrotic form of hypersensitivity pneumonitis have a poor prognosis. Fibrotic HP develops in response to a long-term exposure to low levels of antigen; it usually occurs with more subtle symptoms, gradually leading to a rapid decline in lung function and to early mortality [8]. Fibroblasts, chronically infiltrating the lung in fHP, assume typical features of myofibroblasts, overexpressing alpha-smooth muscle actin (α-SMA) and producing high levels of collagen. These markers are typically expressed in cancer-associated fibroblasts (CAFs); notably, the overexpansion of CAFs in the tumor microenvironment of the lung is strongly associated with poor prognosis [9]. The most prevalent histopathological type of lung cancer was squamous cell carcinoma [10], which is dominant in IPF-associated lung cancer [11,12]
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