Idiopathic Pulmonary Fibrosis: A Historical Perspective

Meyer, Keith C.

doi:10.1007/978-1-62703-682-5_1

Cited by 3 publications

(1 citation statement)

References 21 publications

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“…Interstitial lung diseases (ILDs) comprise nearly a fifth of all lung diseases. ILDs are a heterogeneous group of disorders classified into distinct entities, each with specific clinical, radiological, and pathological characteristics [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 ]. Most ILDs are characterized by a progressive distortion of airway architecture, leading to reduced lung volume and gradual loss of lung function, which can eventually result in respiratory failure [ 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 ].…”

Section: Introductionmentioning

confidence: 99%

Pirfenidone and Nintedanib in Pulmonary Fibrosis: Lights and Shadows

Chianese,

Screm,

Salton

et al. 2024

Pharmaceuticals

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Pirfenidone and Nintedanib are specific drugs used against idiopathic pulmonary fibrosis (IPF) that showed efficacy in non-IPF fibrosing interstitial lung diseases (ILD). Both drugs have side effects that affect patients in different ways and have different levels of severity, making treatment even more challenging for patients and clinicians. The present review aims to assess the effectiveness and potential complications of Pirfenidone and Nintedanib treatment regimens across various ILD diseases. A detailed search was performed in relevant articles published between 2018 and 2023 listed in PubMed, UpToDate, Google Scholar, and ResearchGate, supplemented with manual research. The following keywords were searched in the databases in all possible combinations: Nintedanib; Pirfenidone, interstitial lung disease, and idiopathic pulmonary fibrosis. The most widely accepted method for evaluating the progression of ILD is through the decline in forced vital capacity (FVC), as determined by respiratory function tests. Specifically, a decrease in FVC over a 6–12-month period correlates directly with increased mortality rates. Antifibrotic drugs Pirfenidone and Nintedanib have been extensively validated; however, some patients reported several side effects, predominantly gastrointestinal symptoms (such as diarrhea, dyspepsia, and vomiting), as well as photosensitivity and skin rashes, particularly associated with Pirfenidone. In cases where the side effects are extremely severe and are more threatening than the disease itself, the treatment has to be discontinued. However, further research is needed to optimize the use of antifibrotic agents in patients with PF-ILDs, which could slow disease progression and decrease all-cause mortality. Finally, other studies are requested to establish the treatments that can stop ILD progression.

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Section: Introductionmentioning

confidence: 99%

Pirfenidone and Nintedanib in Pulmonary Fibrosis: Lights and Shadows

Chianese,

Screm,

Salton

et al. 2024

Pharmaceuticals

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Pulmonary fibrosis, part I: epidemiology, pathogenesis, and diagnosis

Meyer

2017

Expert Review of Respiratory Medicine

140

122

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Many forms of interstitial lung disease (ILD) can progress to extensive fibrosis and respiratory failure. Idiopathic pulmonary fibrosis (IPF), which generally has a poor prognosis, has been thoroughly studied over the past two decades, and many important discoveries have been made that pertain to genetic predisposition, epidemiology, disease pathogenesis, diagnosis, and management. Additionally, non-IPF forms of ILD can have radiologic and histopathologic manifestations that mimic IPF, and making an accurate diagnosis is key to providing personalized medicine to patients with pulmonary fibrosis. Areas covered: This manuscript discusses current knowledge pertaining to the genetics, epidemiology, pathogenesis, and diagnosis of pulmonary fibrosis with an emphasis on IPF. The material upon which this discussion is based was obtained from various published texts and manuscripts identified via literature searching (e.g. PubMed). Expert commentary: Many genetic variants have been identified that are associated with risk of developing pulmonary fibrosis, and an improved understanding of the influence of both genomic and epigenomic factors in the development of pulmonary fibrosis is rapidly evolving. Because many forms of fibrosing ILD can have similar radiologic and histopathologic patterns yet have different responses to therapeutic interventions, making an accurate diagnosis of specific forms of pulmonary fibrosis is increasingly important.

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