Obstacles to early treatment of idiopathic pulmonary fibrosis: current perspectives

Moua, Teng; Ryu, Jay H.

doi:10.2147/tcrm.s160248

Cited by 12 publications

(9 citation statements)

References 73 publications

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“…2 With an appearance of characteristic radiography, IPF mostly appeared in the pathological lesion of common interstitial pneumonia. 2 With an appearance of characteristic radiography, IPF mostly appeared in the pathological lesion of common interstitial pneumonia.…”

Section: Introductionmentioning

confidence: 99%

“…Idiopathic pulmonary fibrosis (IPF), a progressive and chronic fibrosing interstitial pneumonia without a clear cause, 1 may worsen lung function or lead to death. 2 With an appearance of characteristic radiography, IPF mostly appeared in the pathological lesion of common interstitial pneumonia. 3 With a 2 to 5 years of survival time from diagnosis, IPF mainly occurs among elderly adults.…”

Section: Introductionmentioning

confidence: 99%

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Protective effect of bicyclol against pulmonary fibrosis via regulation of microRNA‐455‐3p in rats

Zhou

Chai

2019

J of Cellular Biochemistry

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Idiopathic pulmonary fibrosis (IPF), a chronic, progressive and irreversible disease, needs long‐term treatment. Bicyclol was found to play a great role in pulmonary fibrosis, and the present study is to explore how bicyclol affects IPF with the involvement of microRNA‐455‐3p (miR‐455‐3p) and Bax. Bleomycin (BLM) was used to induce the IPF model in Sprague‐Dawley rats to detect the expression of miR‐455‐3p, Bax, and B‐cell lymphoma factor 2 (Bcl‐2). Moreover, to further investigate the mechanisms of bicyclol, the BLM‐induced fibrotic cell model was used after the lung epithelial cells HPAEpiC received miR‐455‐3p knockout treatment. The rats were then treated with vehicle and bicyclol, respectively. The apoptosis of fibrotic cells and Bax/Bcl‐2 were identified. Inhibition function of bicyclol was optimal at a dose of 150 mg/kg. Bicyclol inhibited cell apoptosis and reduced Bax/Bcl‐2 expression in rats. miR‐455‐3p could potentially bind to Bax gene. Bicyclol reduced the levels of methylenedioxyamphetamine, superoxide dismutase, and glutathione in rat lung tissue, inhibited the apoptosis of rats with IPF and upregulated miR‐455‐3p expression. In vitro studies showed that bicyclol significantly promoted miR‐455‐3p expression in HPAEpiC fibrosis. Bicyclol inhibited fibrosis‐induced apoptosis of HPAEpiC in alveolar epithelial cells through promoting miR‐455‐3p, which inhibited Bax expression in IPF. Bicyclol may suppress the apoptosis of alveolar epithelial cells by upregulating miR‐455‐3p. This study laid a theoretical foundation for further understanding of IPF and searching for new molecular therapeutic targets.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Protective effect of bicyclol against pulmonary fibrosis via regulation of microRNA‐455‐3p in rats

Zhou

Chai

2019

J of Cellular Biochemistry

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“… 56 , 57 Since spirometric endpoints can function as determinants in initiating antifibrotic therapy, the presence of emphysema potentially is problematic since it can affect FVC in a manner dissimilar to fibrosis. 58 A single center retrospective study of 45 CPFE patients detected no significant difference in the efficacy of either pirfenidone or nintedanib on measured variables including mortality. 59 Randomized control trials, meta-analysis, and an observation study of pirfenidone in IPF have observed improved progression-free survival and reduced all-cause mortality.…”

Section: Discussionmentioning

confidence: 97%

Combined Pulmonary Fibrosis Emphysema: Role of Cigarette Smoking and Pulmonary Hypertension in a Rural Cohort

Sangani¹,

Ghio²,

Culp³

et al. 2021

COPD

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Background Disease heterogeneity in idiopathic pulmonary fibrosis (IPF) often complicates the systematic study of disease, management of patients and clinical investigations. Objective To describe combined pulmonary fibrosis emphysema (CPFE) phenotype in a rural Appalachian IPF cohort with the highest smoking rates in the United States. Methods CPFE patients (n = 60) in a developed IPF cohort (n = 153) were characterized. Groups (CPFE vs IPF without emphysema) were categorized based on the predominant HRCT patterns of UIP (n = 109). Demographics, clinical variables, and treatment details were recorded. Kaplan–Meier survival and multivariate logistic regression analysis were performed. Results The prevalence of CPFE in our IPF cohort was 45% (n = 49). The CPFE group was younger (73.9 vs 78.2), had a more extensive smoking history (93.9% vs 53.3%) with greater mean smoking pack years (49.09 vs 15.39) and had lower percentage predicted DL CO on presentation (38.35 vs 51.09) compared to IPF without emphysema group. Both groups shared equivalent higher burden of comorbidities, including pulmonary hypertension (PH) (46.9% vs 33.3%). One-fifth of patients were prescribed antifibrotics and only a subset (5%) of patients underwent lung transplantation. There was a non-significant trend towards reduced survival in CPFE (p = 0.076). Smoking status and DL CO predicted CPFE in our cohort. Body mass index (BMI), PH, and pirfenidone use were significant predictors of mortality. Conclusion CPFE was highly prevalent in our rural IPF cohort. In contrast to previous studies, CPFE group was older and had higher female (approx. 30%) occurrence. A greater exposure to cigarette smoke and reduced DL CO at diagnosis predicted CPFE. Lower BMI and PH predicted higher mortality whereas use of pirfenidone improved survival in our cohort. This study highlights a complex interaction of cigarette smoking, advanced fibrosis of UIP, PH and potential utility of antifibrotic agents in CPFE phenotype. Substantial burden of comorbidities, older age, and the limited utilization of advanced therapeutics in the cohort emphasize the challenges faced by rural Appalachian patients.

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“…Since then, several studies have confirmed the clinical efficacy of these drugs, including one recent observational study demonstrating that patients may have lower mortality and hospitalization rates when compared to those not on treatment [ 8 – 10 ]. Despite these benefits and the lack of other effective treatment options, there are still concerns about anti-fibrotics, including their cost (more than $100,000 per year in the United States), which may add to the economic burden of patients with IPF [ 11 ].…”

Section: Introductionmentioning

confidence: 99%

Cost-effectiveness of the anti-fibrotics for the treatment of idiopathic pulmonary fibrosis in the United States

et al. 2022

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Background The anti-fibrotic medications nintedanib and pirfenidone were approved in the United States for use in patients with idiopathic pulmonary fibrosis several years ago. While there is a growing body of evidence surrounding their clinical effectiveness, these medications are quite expensive and no prior cost-effectiveness analysis has been performed in the United States. Methods A previously published Markov model performed in the United Kingdom was replicated using United States data to project the lifetime costs and health benefits of treating idiopathic pulmonary fibrosis with: (1) symptom management; (2) pirfenidone; or (3) nintedanib. For the cost-effectiveness analysis, strategies were ranked by increasing costs and then checked for dominating treatment strategies. Then an incremental cost-effectiveness ratio was calculated for the dominant therapy. Results The anti-fibrotic medications were found to cost more than $110,000 per year compared to $12,291 annually for symptom management. While pirfenidone was slightly more expensive than nintedanib and provided the same amount of benefit, neither medication was found to be cost-effective in this U.S.-based analysis, with an average cost of $1.6 million to gain one additional quality-adjusted life year over symptom management. Conclusions Though the anti-fibrotics remain the only effective treatment option for patients with idiopathic pulmonary fibrosis and the data surrounding their clinical effectiveness continues to grow, they are not considered cost-effective treatment strategies in the United States due to their high price.

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Obstacles to early treatment of idiopathic pulmonary fibrosis: current perspectives

Cited by 12 publications

References 73 publications

Protective effect of bicyclol against pulmonary fibrosis via regulation of microRNA‐455‐3p in rats

Protective effect of bicyclol against pulmonary fibrosis via regulation of microRNA‐455‐3p in rats

Combined Pulmonary Fibrosis Emphysema: Role of Cigarette Smoking and Pulmonary Hypertension in a Rural Cohort

Cost-effectiveness of the anti-fibrotics for the treatment of idiopathic pulmonary fibrosis in the United States

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