Pulmonary fibrolysis in a patient with idiopathic pulmonary fibrosis: improvement of clinical and radiological pattern after treatment with pirfenidone

Varone, Francesco; Mastrobattista, Annelisa; Franchi, Paola; Viglietta, Luca; Poletti, Venerino; Tomassetti, Sara; Dubini, Alessandra; Tagliaboschi, Linda; Calandriello, Lucio; Farchione, Alessandra; Larici, Anna Rita

doi:10.1111/crj.12515

Cited by 3 publications

(3 citation statements)

References 15 publications

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“…IPF is caused by the accumulation of matrix proteins in the lungs due to an imbalance between fibrogenesis and fibrolysis. 32,33 The transition from epithelial cells to fibroblasts followed by myofibroblasts is an important process in lung fibrosis. In this study, stearic acid alleviated bleomycin-induced lung fibrosis and suppressed TGF-β1-induced α-SMA and collagen type 1 expression in fibroblasts.…”

Section: Discussionmentioning

confidence: 99%

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Stearic acid attenuates profibrotic signalling in idiopathic pulmonary fibrosis

et al. 2020

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Background and objective: Lipid metabolism dysregulation has been implicated in the pathogenesis of IPF; however, the roles of most lipid metabolites in lung fibrosis remain unexplored. Therefore, we aimed to identify changes in lipid metabolites in the lung tissues of IPF patients and determine their roles in pulmonary fibrosis. Methods: Free fatty acids in the lung tissues of IPF patients and controls were quantified using a metabolomic approach. The roles of free fatty acids in fibroblasts or epithelial cells treated with TGF-β1 were evaluated using fibrotic markers. The antifibrotic role of stearic acid was also assessed in a bleomycin-induced lung fibrosis mouse model. Protein levels in cell lysates or tissues were measured by western blotting. Results: The levels of stearic acid were lower in IPF lung tissues than in control lung tissues. Stearic acid significantly reduced TGF-β1-induced α-SMA and collagen type 1 expression in MRC-5 cells. Furthermore, stearic acid decreased the levels of p-Smad2/3 and ROS in MRC-5 cells treated with TGF-β1 and disrupted TGF-β1-induced EMT in Beas-2B cells. Stearic acid reduced the levels of bleomycin-induced hydroxyproline in a mouse model. Conclusion: Changes in the free fatty acid profile, including low levels of stearic acid, were observed in IPF patients. Stearic acid may exert antifibrotic activity by regulating profibrotic signalling.

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Section: Discussionmentioning

confidence: 99%

“…IPF is caused by the accumulation of matrix proteins in the lungs due to an imbalance between fibrogenesis and fibrolysis 32,33 . The transition from epithelial cells to fibroblasts followed by myofibroblasts is an important process in lung fibrosis.…”

Section: Discussionmentioning

confidence: 99%

Stearic acid attenuates profibrotic signalling in idiopathic pulmonary fibrosis

et al. 2020

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“…Moreover, PFD, as well as nintedanib, rarely make marked improvement of pulmonary function and chest high-resolution computed tomography (HRCT) findings. To our knowledge, there are only 4 reported cases of so-called "super-responder" to PFD treatment (7)(8)(9)(10). In addition, these cases have not been compared in detail.…”

Section: Introductionmentioning

confidence: 99%

Distinct improvement of pulmonary function, ground-glass opacity, hypoxia and physical findings in an idiopathic pulmonary fibrosis patient after pirfenidone treatment : a case report with a review of the literature

et al. 2020

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Background : Pirfenidone (PFD), an anti-fibrosis drug for idiopathic pulmonary fibrosis (IPF), suppresses disease progression and delays decline of forced vital capacity. However, this drug rarely makes marked improvement of pulmonary function, chest high-resolution computed tomography (HRCT) findings and hypoxia. Case presentation : A 59 year-old-man, who was a former smoker and had a history of alcoholic liver cirrhosis, developed exertional dyspnea and was referred to our hospital. HRCT showed honeycomb changes with surrounding ground-glass opacity (GGO) in a predominantly basal and subpleural distribution. He was diagnosed with IPF and the treatment with PFD was started. At 16 months after the start of treatment, the predicted forced vital capacity value markedly improved from 82.9% to 98.6%. His resting-state partial pressure of arterial oxygen while breathing room air increased from a minimum of 54.7 mmHg (at 2 months treatment) to 72.5 mmHg. The GGO observed at diagnosis disappeared in HRCT. But after 32 months of treatment, his general condition got worse gradually, and he died from chronic progression of IPF after 48 months of treatment. Conclusion : Our case suggests that a complication of chronic liver disease and the existence of GGO may be characteristics of super-responder to PFD treatment for IPF patients.

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Genistein alleviates pulmonary fibrosis by inactivating lung fibroblasts

Kwon,

Chung,

Seo

et al. 2023

BMB Rep

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Pulmonary fibrosis is a serious lung disease that occurs predominantly in men. Genistein is an important natural soybean-derived phytoestrogen that affects various biological functions, such as cell migration and fibrosis. However, the antifibrotic effects of genistein on pulmonary fibrosis are largely unknown. The antifibrotic effects of genistein were evaluated using in vitro and in vivo models of lung fibrosis. Proteomic data were analyzed using nano-LC-ESI-MS/MS. Genistein significantly reduced transforming growth factor (TGF)-β1induced expression of collagen type I and α-smooth muscle actin (SMA) in MRC-5 cells and primary fibroblasts from patients with idiopathic pulmonary fibrosis (IPF). Genistein also reduced TGF-β1-induced expression of p-Smad2/3 and p-p38 MAPK in fibroblast models.Comprehensive protein analysis confirmed that genistein exerted an anti-fibrotic effect by regulating various molecular mechanisms, such as unfolded protein response, epithelial mesenchymal transition (EMT), mTORC1 signaling, cell death, and several metabolic pathways. Genistein was also found to decrease hydroxyproline levels in the lungs of BLMtreated mice. Genistein exerted an anti-fibrotic effect by preventing fibroblast activation, suggesting that genistein could be developed as a pharmacological agent for the prevention and treatment of pulmonary fibrosis.

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Pulmonary fibrolysis in a patient with idiopathic pulmonary fibrosis: improvement of clinical and radiological pattern after treatment with pirfenidone

Cited by 3 publications

References 15 publications

Stearic acid attenuates profibrotic signalling in idiopathic pulmonary fibrosis

Stearic acid attenuates profibrotic signalling in idiopathic pulmonary fibrosis

Distinct improvement of pulmonary function, ground-glass opacity, hypoxia and physical findings in an idiopathic pulmonary fibrosis patient after pirfenidone treatment : a case report with a review of the literature

Genistein alleviates pulmonary fibrosis by inactivating lung fibroblasts

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