Treatment of idiopathic pulmonary fibrosis: a position paper from a Nordic expert group

Sköld, C. Magnus; Bendstrup, Elisabeth; Myllärniemi, Marjukka; Guðmundsson, Gunnar; Sjåheim, Tone; Hilberg, Ole; Altraja, Alan; Kaarteenaho, Riitta; Ferrara, Giovanni

doi:10.1111/joim.12571

Cited by 30 publications

(41 citation statements)

References 133 publications

(167 reference statements)

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“…Sildenafil has improved dyspnoea and QoL in a subgroup of IPF patients with pulmonary hypertension [23,50] and, although it is currently not recommended as a treatment for IPF by international guidelines, its use can be considered in patients with concomitant pulmonary hypertension and low diffusing capacity for carbon monoxide, to improve effort dyspnoea and QoL [4]. No studies are available on the role of bronchodilators in patients with IPF and signs of airflow obstruction at spirometry.…”

Section: Dyspnoea and Exercise Capacitymentioning

confidence: 99%

“…New antifibrotic treatments are able to reduce the loss of respiratory function over time [3,4], but their effect on symptom control and quality of life (QoL) is limited and needs further exploration [5][6][7]. A multidisciplinary approach for the diagnosis and treatment of IPF is recommended by current guidelines [1], as it has been shown to result in a more accurate diagnosis [8] and to positively affect survival in this group of patients [9].…”

Section: Introductionmentioning

confidence: 99%

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Best supportive care for idiopathic pulmonary fibrosis: current gaps and future directions

Ferrara

Luppi²,

Birring

et al. 2018

Eur Respir Rev

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Best supportive care (BSC) is generally defined as all the interventions and the multiprofessional approach aimed to improve and optimise quality of life (QoL) in patients affected by progressive diseases. In this sense, it excludes and might be complementary to other interventions directly targeting the disease. BSC improves survival in patients with different types of cancer. Patients with idiopathic pulmonary fibrosis (IPF) experience a vast range of symptoms during the natural history of the disease and might have a beneficial effect of BSC interventions. This review highlights the current evidence on interventions targeting QoL and gaps for the clinical assessment of BSC in the treatment of IPF patients. Very few interventions to improve QoL or improve symptom control are currently supported by well-designed studies. Sound methodology is paramount in evaluating BSC in IPF, as well as the use of validated tools to measure QoL and symptom control in this specific group of patients.

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Section: Dyspnoea and Exercise Capacitymentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Best supportive care for idiopathic pulmonary fibrosis: current gaps and future directions

Ferrara

Luppi²,

Birring

et al. 2018

Eur Respir Rev

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“…Fibrotic process results in extensive alteration of the structure and function of the certain organs; thus, clinical trials of fibrosis treatment are being conducted in many leading research facilities [121]. Antifibrotic therapies can be divided into nonpharmacological and pharmacological [121].…”

Section: Therapeutic Implicationsmentioning

confidence: 99%

Section: Therapeutic Implicationsmentioning

confidence: 99%

“…Antifibrotic therapies can be divided into nonpharmacological and pharmacological [121]. Because fibrosis is irreversible, nonpharmacological fibrosis therapies are based mainly on surgical restoration of organ function, either by removal of nonfunctional fibrotic scar tissue or by transplantation of whole organs, for example, lung transplantation in progressive pulmonary disease [121] or cystic fibrosis [122]. On the other hand, pharmacological therapies targeting many distinct molecular mechanisms involved in fibrosis development and progression comprise the suppression of inflammatory response by corticosteroids [123, 124], inhibition of TGF- β , TNF, PDGF, VEGFR, and FGFR signaling pathway, and collagen fibrils formation by tyrosine kinase inhibitor—nintedanib as well as by pyridone derivative—pirfenidone [125–127] in idiopathic pulmonary fibrosis.…”

Section: Therapeutic Implicationsmentioning

confidence: 99%

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Proteases Revisited: Roles and Therapeutic Implications in Fibrosis

Kryczka

Boncela

2017

Mediators of Inflammation

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Proteases target many substrates, triggering changes in distinct biological processes correlated with cell migration, EMT/EndMT and fibrosis. Extracellular protease activity, demonstrated by secreted and membrane-bound protease forms, leads to ECM degradation, activation of other proteases (i.e., proteolysis of nonactive zymogens), decomposition of cell-cell junctions, release of sequestered growth factors (TGF-β and VEGF), activation of signal proteins and receptors, degradation of inflammatory inhibitors or inflammation-related proteins, and changes in cell mechanosensing and motility. Intracellular proteases, mainly caspases and cathepsins, modulate lysosome activity and signal transduction pathways. Herein, we discuss the current knowledge on the multidimensional impact of proteases on the development of fibrosis.

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Therapeutic Strategies for Idiopathic Pulmonary Fibrosis – Thriving Present and Promising Tomorrow

Gupta,

Paryani,

Patel

et al. 2024

The Journal of Clinical Pharma

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Idiopathic pulmonary fibrosis (IPF) is a continuous, progressive, and lethal age‐related respiratory disease. It is characterized by condensed and rigid lung tissue, which leads to a decline in the normal functioning of the lungs. The pathophysiology of IPF has still not been completely elucidated, so current strategies are lagging behind with respect to improving the condition of patients with IPF and increasing their survival rate. The desire for a better understanding of the pathobiology of IPF and its early detection has led to the identification of various biomarkers associated with IPF. The use of drugs such as pirfenidone and nintedanib as a safe and effective treatment alternative have marked a new chapter in the treatment of IPF. However, nonpharmacological therapies, involving long‐term oxygen therapy, transplantation of the lungs, pulmonary rehabilitation, ventilation, and palliative care for cough and dyspnea, are still considered to be beneficial as supplementary methods for IPF therapy. A major risk factor for IPF is aging, with associated hallmarks such as telomere attrition, senescence, epigenetic drift, stem cell exhaustion, loss of proteostasis, and mitochondrial dysfunction. These are promising earmarks for the development of potential therapy for the disease. In this review, we have discussed current and emerging novel therapeutic strategies for IPF, especially for targets associated with age‐related mechanisms.

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Treatment of idiopathic pulmonary fibrosis: a position paper from a Nordic expert group

Cited by 30 publications

References 133 publications

Best supportive care for idiopathic pulmonary fibrosis: current gaps and future directions

Best supportive care for idiopathic pulmonary fibrosis: current gaps and future directions

Proteases Revisited: Roles and Therapeutic Implications in Fibrosis

Therapeutic Strategies for Idiopathic Pulmonary Fibrosis – Thriving Present and Promising Tomorrow

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