Pirfenidone may alleviate disease burden in DRCB

Pirfenidone reduced peribronchiolar fibrosis in a murine model of deployment-related constrictive bronchiolitis (DRCB). Since there are no approved therapies for this condition, it is a potentially practice-changing result. According to the authors, future studies should first investigate the underlying cellular and molecular pathways of pirfenidone’s therapeutic efficacy through in-vivo studies and proteomic and transcriptomic analyses.

“DRCB comes with symptoms of chronic cough, dyspnoea, and exercise intolerance,” according to Ms Helen Hicks (University of Michigan, MI, USA). “Currently there is no treatment to help patients with this condition.” She further outlined that events that lead to exposure to airborne toxins, such as sand and dust storms and oil field fires, can result in DRCB. In the current study, Ms Hicks and co-investigators targeted club cells, which is a type of bronchiolar epithelial cell that may be affected in patients with DRCB [1].

The research team showed that sustained club cell injury in mice results in peribronchiolar fibrosis. “We also found that sustained club cell injury is associated with histopathologic features of DRCB in these mice, such as airway wall thickening and chronic inflammation of the small airways,” mentioned Ms Hicks. The team reasoned that pirfenidone, currently used in idiopathic pulmonary fibrosis, other forms of interstitial lung disease, and bronchiolitis obliterans syndrome, may be a viable option to target constrictive bronchiolitis in patients with DRCB. The mice received doxycycline for 10 days, followed by pirfenidone or a vehicle on a daily base up to day 19.

Ms Hicks revealed that mice treated with pirfenidone displayed improved peribronchiolar fibrosis as compared to the ‘control’ mice, with significantly smaller areas of collagen disposition. “Thus, pirfenidone can abrogate the development of peribronchiolar fibrosis in mice exposed to sustained club cell injury,” concluded Ms Hicks. “Therefore, pirfenidone is a potential therapy for patients with DRCB.”

1. Hicks H, et al. Pirfenidone ameliorates peribronchiolar fibrosis in a murine model of deployment-related constrictive bronchiolitis. Late-breaking abstracts: science that will impact clinical care

Medical writing support was provided by Robert van den Heuvel.

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Posted on 11 June 202426 July 2024