Treatment of Idiopathic Pulmonary Fibrosis

Abuserewa, Sherif T; Duff, Richard; Becker, Gregory M.

doi:10.7759/cureus.15360

Cited by 9 publications

(9 citation statements)

References 94 publications

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“…This included a recommendation for home oxygen therapy for hypoxemic patients to maintain their SpO 2 levels above 90% throughout the day, and the appropriate management of comorbid gastroesophageal reflux disease (GERD) in patients with IPF. The benefits of home oxygen therapy may range from improved exercise tolerance [ 58 ] and lesser dyspnea [ 58 ] to the attenuation of cardiac dysfunction [ 59 ]. Symptoms of GERD are present in more 33% of patients with IPF [ 38 ].…”

Section: Discussionmentioning

confidence: 99%

GRADE-ADOLOPMENT of clinical practice guidelines and creation of clinical pathways for the primary care management of chronic respiratory conditions in Pakistan

et al. 2023

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Introduction In Pakistan, chronic respiratory conditions contribute a large burden of morbidity and mortality. A major reason for this is the lack of availability of local evidence-based clinical practice guidelines (EBCPGs) in Pakistan, particularly at the primary care level. Thus, we developed EBCPGs and created clinical diagnosis and referral pathways for the primary care management of chronic respiratory conditions in Pakistan. Methods The source guidelines were selected by two local expert pulmonologists after a thorough literature review on PubMed and Google Scholar from 2010 to December 2021. The source guidelines covered idiopathic pulmonary fibrosis, asthma, chronic obstructive pulmonary disorders, and bronchiectasis. The GRADE-ADOLOPMENT process consists of three key elements: adoption (using recommendations as is or with minor changes), adaptation (effective context-specific changes to recommendations) or additions (including new recommendations to fill a gap in the EBCPG). We employed the GRADE-ADOLOPMENT process to adopt, adapt, adopt with minor changes, or exclude recommendations from a source guideline. Additional recommendations were added to the clinical pathways based on a best-evidence review process. Results 46 recommendations were excluded mainly due to the unavailability of recommended management in Pakistan and scope beyond the practice of general physicians. Clinical diagnosis and referral pathways were designed for the four chronic respiratory conditions, explicitly delineating the role of primary care practitioners in the diagnosis, basic management, and timely referral of patients. Across the four conditions, 18 recommendations were added (seven for IPF, three for bronchiectasis, four for COPD, and four for asthma). Conclusion The widespread use of the newly created EBCPGs and clinical pathways in the primary healthcare system of Pakistan can help alleviate the morbidity and mortality related to chronic respiratory conditions disease in the country.

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Section: Discussionmentioning

confidence: 99%

GRADE-ADOLOPMENT of clinical practice guidelines and creation of clinical pathways for the primary care management of chronic respiratory conditions in Pakistan

et al. 2023

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“…Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible lung disease with a poor prognosis. It usually occurs in the adults and the 5-year survival rate is only ~20-25%, worse than most types of cancer ( 1 , 2 ). The etiology of IPF remains unknown and it is often characterized by the abnormal activation of alveolar epithelial cells and fibroblasts, leading to the continuous accumulation of collagen and extracellular matrix, ultimately causing structural damage to the lung tissues and lung dysfunction ( 3 ).…”

Section: Introductionmentioning

confidence: 99%

Airway basal cell‑derived exosomes suppress epithelial‑mesenchymal transition of lung cells by inhibiting the expression of ANO1

Gu,

Liu,

Shan

et al. 2024

Exp Ther Med

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Disruption of the epithelial-mesenchymal transition (EMT) of activated lung cells is an important strategy to inhibit the progression of idiopathic pulmonary fibrosis (IPF). The present study investigated the role of exosomes derived from airway basal cells on EMT of lung cells and elucidate the underlying mechanism. Exosomes were characterized by nanoparticle tracking analysis, transmission electron microscopy imaging and markers detection. The role of exosome on the EMT of lung epithelial cells and lung fibroblasts induced by TGF-β1 was detected. RNA sequencing screened dysregulated genes in exosome-treated group, followed by the bioinformatical analysis. One of the candidates, anoctamin-1 (ANO1), was selected for further gain-and-loss phenotype assays. A bleomycin-induced pulmonary fibrosis model was used to evaluate the treatment effect of exosomes. Exosomes were round-like and positively expressed CD63 and tumor susceptibility gene 101 protein. Treatment with exosomes inhibited the EMT of lung cells activated by TGF-β1. 4158 dysregulated genes were identified in exosome-treated group under the threshold of |log2 fold-change| value >1 and they were involved in the metabolism of various molecules, as well as motility-related biological processes. A key gene, ANO1, was verified by reverse transcription-quantitative PCR, whose overexpression induced the EMT of lung cells. By contrast, ANO1 knockdown reversed the EMT induced by TGF-β1. In vivo assay indicated that exosome treatment ameliorated pulmonary fibrosis and inhibited the upregulation of ANO1 induced by bleomycin. In conclusion, airway basal cell-derived exosomes suppressed the EMT of lung cells via the downregulation of ANO1. These exosomes represent a potential therapeutic option for patients with IPF.

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“…IPF primarily occurs among the middle-aged and elderly populations, where it is limited to the lungs (5,6). The treatment of IPF typically involves a combination of medications, pulmonary rehabilitation and, in some cases, lung transplantation (7). IPF is a chronic and progressive disease, and existing treatments, including antifibrotic medications, aim to slow down the progression rather than cure the condition (7).…”

Section: Introductionmentioning

confidence: 99%

“…The treatment of IPF typically involves a combination of medications, pulmonary rehabilitation and, in some cases, lung transplantation (7). IPF is a chronic and progressive disease, and existing treatments, including antifibrotic medications, aim to slow down the progression rather than cure the condition (7). However, the antifibrotic medications may have side effects, and not all individuals with IPF can tolerate these drugs (8).…”

Section: Introductionmentioning

confidence: 99%

Role and targeting of the AGC kinase family in pulmonary fibrosis (Review)

Mei,

Chen,

Huang

et al. 2024

Exp Ther Med

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Pulmonary fibrosis (PF) is a progressive and irreversible pulmonary disease with a high mortality rate and limited treatment options. The cAMP-dependent protein kinase A, cGMP-dependent protein kinase G and phospholipid-dependent protein kinase C, collectively known as AGC kinases, are evolutionarily conserved protein kinases that are widely distributed among eukaryotes. AGC kinases serve a crucial role in a variety of cellular functions and pathological processes, including cancer, diabetes, inflammation and viral infections, where they have been implicated the pathogenesis of PF. The present review summarizes the evidence for the involvement of specific AGC kinases in the pathogenesis of PF, and provides a theoretical basis for the development of targeted AGC kinase small molecule inhibitors or targeted drugs, offering more effective treatment options and strategies for patients with PF. Contents1. Introduction 2. AGC kinases 3. AGC kinases in pulmonary fibrosis 4. Conclusions

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Treatment of Idiopathic Pulmonary Fibrosis

Cited by 9 publications

References 94 publications

GRADE-ADOLOPMENT of clinical practice guidelines and creation of clinical pathways for the primary care management of chronic respiratory conditions in Pakistan

GRADE-ADOLOPMENT of clinical practice guidelines and creation of clinical pathways for the primary care management of chronic respiratory conditions in Pakistan

Airway basal cell‑derived exosomes suppress epithelial‑mesenchymal transition of lung cells by inhibiting the expression of ANO1

Role and targeting of the AGC kinase family in pulmonary fibrosis (Review)

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