The therapy of idiopathic pulmonary fibrosis: what is next?

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial lung disease, characterised by progressive scarring of the lung and associated with a high burden of disease and early death. The pathophysiological understanding, clinical diagnostics and therapy of IPF have significantly evolved in recent years. While the recent introduction of the two antifibrotic drugs pirfenidone and nintedanib led to a significant reduction in lung function decline, there is still no cure for IPF; thus, new therapeutic approaches are needed. Currently, several clinical phase I–III trials are focusing on novel therapeutic targets. Furthermore, new approaches in nonpharmacological treatments in palliative care, pulmonary rehabilitation, lung transplantation, management of comorbidities and acute exacerbations aim to improve symptom control and quality of life. Here we summarise new therapeutic attempts and potential future approaches to treat this devastating disease.

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

European Respiratory Review ◽

10.1183/16000617.0076-2017 ◽

2018 ◽

Vol 27 (147) ◽

pp. 170076 ◽

Cited By ~ 15

Author(s):

Giovanni Ferrara ◽

Fabrizio Luppi ◽

Surinder S. Birring ◽

Stefania Cerri ◽

Antonella Caminati ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Supportive Care ◽

Symptom Control ◽

Best Supportive Care ◽

Current Evidence ◽

Future Directions ◽

Improve Symptom ◽

History Of

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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Initial therapeutic dose of corticosteroid for an acute exacerbation of IPF is associated with subsequent early recurrence of another exacerbation

Scientific Reports ◽

10.1038/s41598-021-85234-1 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Ryo Yamazaki ◽

Osamu Nishiyama ◽

Sho Saeki ◽

Hiroyuki Sano ◽

Takashi Iwanaga ◽

...

Keyword(s):

Risk Factors ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Therapeutic Dose ◽

Early Recurrence ◽

Time To Recurrence ◽

Acute Exacerbations ◽

Patient Groups ◽

The Mean ◽

Mean Time

AbstractSome patients with idiopathic pulmonary fibrosis (IPF) undergo recurrent acute exacerbations (AEs). This study aimed to elucidate the risk factors for recurrent AEs of IPF (AE-IPF). Consecutive patients with IPF admitted for their first AE-IPF between January 2008 and December 2018 were retrospectively recruited. Of 63 patients admitted for an AE-IPF and discharged alive, 9 (14.3%) developed a recurrence of AE within 1 year. The mean time to recurrence was 233 ± 103 days. Total doses (mg/month and mg/kg/month) of corticosteroids administered over day 1 to 30 after the AE were significantly higher in patients without recurrences of AE-IPF (5185 ± 2414 mg/month, 93.5 ± 44.0 mg/kg/month) than the doses in patients with recurrences (3133 ± 1990 mg/month, 57.2 ± 37.7 mg/kg/month) (p = 0.02 and p = 0.03, respectively). However, no differences were observed between the total doses of corticosteroids administered over days 31 to 60, 61 to 90, 91 to 120, and 151 to 180 after the AE. Furthermore, differences between the administration rates of immunosuppressive and antifibrotic treatments administered to the 2 patient groups were not significant. An increased total dose of corticosteroid administered over day 1 to 30 after an AE-IPF was associated with a decreased risk of subsequent recurrence of AE-IPF within 1 year after the first AE.

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Reducing lung function decline in patients with idiopathic pulmonary fibrosis: potential of nintedanib

Drug Design Development and Therapy ◽

10.2147/dddt.s38833 ◽

2013 ◽

pp. 503 ◽

Cited By ~ 1

Author(s):

Toby Maher ◽

Philip Molyneaux ◽

Woodcock

Keyword(s):

Lung Function ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Lung Function Decline

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The effect of additional antimicrobial therapy on the outcomes of patients with idiopathic pulmonary fibrosis: a systematic review and meta-analysis

Respiratory Research ◽

10.1186/s12931-021-01839-0 ◽

2021 ◽

Vol 22 (1) ◽

Author(s):

Ching-Yi Chen ◽

Chao-Hsien Chen ◽

Cheng-Yi Wang ◽

Chih-Cheng Lai ◽

Chien-Ming Chao ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Usual Care ◽

Antimicrobial Therapy ◽

Antimicrobial Agents ◽

Meta Analysis ◽

Lung Function Decline ◽

Increased Risk ◽

Significant Difference ◽

All Cause Mortality

Abstract Background The effect of additional antimicrobial agents on the clinical outcomes of patients with idiopathic pulmonary fibrosis (IPF) is unclear. Methods We performed comprehensive searches of randomized control trials (RCTs) that compared the clinical efficacy of additional antimicrobial agents to those of placebo or usual care in the treatment of IPF patients. The primary outcome was all-cause mortality, and the secondary outcomes were changes in forced vital capacity (FVC), diffusing capacity of the lung for carbon monoxide (DLCO), and the risk of adverse events (AEs). Results Four RCTs including a total of 1055 patients (528 receiving additional antibiotics and 527 receiving placebo or usual care) were included in this meta-analysis. Among the study group, 402 and 126 patients received co-trimoxazole and doxycycline, respectively. The all-cause mortality rates were 15.0% (79/528) and 14.0% (74/527) in the patients who did and did not receive additional antibiotics, respectively (odds ratio [OR] 1.07; 95% confidence interval [CI] 0.76 to 1.51; p = 0.71). No significant difference was observed in the changes in FVC (mean difference [MD], 0.01; 95% CI − 0.03 to 0.05; p = 0.56) and DLCO (MD, 0.05; 95% CI − 0.17 to 0.28; p = 0.65). Additional use of antimicrobial agents was also associated with an increased risk of AEs (OR 1.65; 95% CI 1.19 to 2.27; p = 0.002), especially gastrointestinal disorders (OR 1.54; 95% CI 1.10 to 2.15; p = 0.001). Conclusions In patients with IPF, adding antimicrobial therapy to usual care did not improve mortality or lung function decline but increased gastrointestinal toxicity.

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Effect of low dose pirfenidone on survival and lung function decline in patients with idiopathic pulmonary fibrosis (IPF): results from a real life study

10.1183/13993003.congress-2021.pa478 ◽

2021 ◽

Author(s):

Eung Gu Lee ◽

Tae-Hee Lee ◽

Yujin Hong ◽

Jiwon Ryoo ◽

Jung Won Heo ◽

...

Keyword(s):

Lung Function ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Low Dose ◽

Real Life ◽

Lung Function Decline ◽

Life Study ◽

Real Life Study

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Acute exacerbations of idiopathic pulmonary fibrosis and the role of corticosteroids

Breathe ◽

10.1183/20734735.0086-2020 ◽

2020 ◽

Vol 16 (3) ◽

pp. 200086

Author(s):

Christopher J. Brereton ◽

Helen E. Jo

Keyword(s):

Disease Management ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Supportive Care ◽

Disease Process ◽

Evidence Base ◽

Fatal Complication ◽

Acute Exacerbations ◽

International Awareness

An acute exacerbation of idiopathic pulmonary fibrosis (AEIPF) is a potentially fatal complication of an already debilitating disease. Management is currently centred on delivering excellent supportive care and identifying reversible triggers. Despite growing international awareness and collaboration, no effective therapies have been identified. Corticosteroids are often the mainstay of treatment; however, the evidence base for their use is poor. Here, we review our current understanding of the disease process and how to manage it, with a focus on the role of corticosteroid therapy.

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