scholarly journals DSP rs2076295 variants influence nintedanib and pirfenidone outcomes in idiopathic pulmonary fibrosis: a pilot study

2021 ◽  
Vol 15 ◽  
pp. 175346662110425
Author(s):  
Martina Doubkova ◽  
Eva Kriegova ◽  
Simona Littnerova ◽  
Petra Schneiderova ◽  
Martina Sterclova ◽  
...  
Keyword(s):  
Pilot Study ◽  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Vital Capacity ◽  
Cross Validation ◽  
Healthy Individuals ◽  
Lung Capacity ◽  
Diffuse Lung ◽  
Antifibrotic Drugs

Background: The antifibrotic drugs nintedanib and pirfenidone are used for the treatment of idiopathic pulmonary fibrosis (IPF). We analysed the association of common profibrotic polymorphisms in MUC5B (mucin 5B, rs35705950) and DSP (desmoplakin, rs2076295) on antifibrotic treatment outcomes in IPF. Methods: MUC5B rs35705950 and DSP rs2076295 were assessed in IPF patients ( n = 210, 139 men/71 women) from the Czech EMPIRE registry and age- or sex-matched healthy individuals ( n = 205, 125 men/80 women). Genetic data were collated with overall survival (OS), acute exacerbation episodes, worsening lung function and antifibrotic treatment. Results: We confirmed overexpression of the MUC5B rs35705950*T allele (55.2% versus 20.9%, p < 0.001) and the DSP rs2076295*G allele (80.4% versus 68.3%, p < 0.001) in IPF compared with controls. On antifibrotic drugs, lower mortability was observed in IPF patients with DSP G* allele ( p = 0.016) and MUC5B T* allele ( p = 0.079). Carriers of the DSP rs2076295*G allele benefitted from nintedanib treatment compared with TT genotype by a longer OS [hazard ratio (HR) = 7.99; 95% confidence interval (CI) = 1.56–40.90; p = 0.013] and a slower decline in lung function (HR = 8.51; 95% CI = 1.68–43.14; p = 0.010). Patients with a TT genotype (rs2076295) benefitted from treatment with pirfenidone by prolonged OS ( p = 0.040; HR = 0.35; 95% CI = 0.13–0.95) compared with nintedanib treatment. Both associations were confirmed by cross-validation analysis. After stratifying by MUC5B rs35705950*T allele carriage, no difference in treatment outcome was observed for nintedanib or pirfenidone ( p = 0.784). In the multivariate model, smoking, age, forced vital capacity (FVC) and DLCO (diffuse lung capacity) at the IPF diagnosis were associated with survival. Conclusion: Our real-world study showed that IPF patients with MUC5B T* allele or DSP G* allele profit from antifibrotic treatment by lower mortability. Moreover, carriers of the DSP rs2076295*G allele benefit from treatment with nintedanib, and TT genotype from treatment with pirfenidone. MUC5B rs35705950 did not impact the outcome of treatment with either nintedanib or pirfenidone. Our single-registry pilot study should be confirmed with an independent patient cohort.

scholarly journals Efficacy of early antifibrotic treatment for idiopathic pulmonary fibrosis

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Keishi Sugino ◽  
Hirotaka Ono ◽  
Natsumi Watanabe ◽  
Masahiro Ando ◽  
Eiyasu Tsuboi ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Disease Progression ◽  
Vital Capacity ◽  
Early Stage ◽  
Japanese Patients ◽  
Oxygen Desaturation ◽  
Stage I ◽  
Relative Decline ◽  
Antifibrotic Drugs

Abstract Background Although antifibrotic drugs, including nintedanib and pirfenidone, slow the progression of idiopathic pulmonary fibrosis (IPF), there is little data about the timing of start of antifibrotic treatment in real-world clinical practice. The present study aimed to clarify the efficacy of nintedanib and pirfenidone in patients with early-stage IPF. Methods We compared survival and disease progression between patients with IPF with Japanese Respiratory Society (JRS) disease severity system stage I with and without oxygen desaturation on the 6-min walk test (6MWT) and increased the gender–age–physiology (GAP) staging. We examined the efficacy of antifibrotic drugs in patients with early-stage IPF. Results The severity of stage I IPF (n = 179) according to the JRS criteria consisted of the following GAP staging criteria: stage I, 111 cases; stage II, 58 cases; stage III, 10 cases. The duration from the initial visit to disease progression and survival time was significantly shorter in JRS stage I patients with oxygen desaturation on the 6MWT or with increased GAP staging (unfavorable group) compared with patients without those factors. In the unfavorable group, the relative decline in percentage predicted forced vital capacity (%FVC) over 6 months was significantly lower in patients undergoing antifibrotic treatment compared with non-treated patients. Conclusion Antifibrotic drugs have a beneficial effect on the decline in %FVC in Japanese patients with early-stage IPF who have oxygen desaturation on the 6MWT or increased GAP staging.

Impact of Lung Biopsy on Lung Function in Idiopathic Pulmonary Fibrosis

Respiration ◽  
2020 ◽  
pp. 1-8
Author(s):  
Pierre-Henri Aussedat ◽  
Nader Chebib ◽  
Kais Ahmad ◽  
Jean-Charles Glerant ◽  
Gabrielle Drevet ◽  
...  
Keyword(s):  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Forced Vital Capacity ◽  
Lung Biopsy ◽  
Vital Capacity ◽  
Forced Expiratory Volume ◽  
Surgical Lung Biopsy ◽  
Before And After ◽  
The Impact

<b><i>Background:</i></b> Video-assisted surgical lung biopsy (SLB) is performed in 10–30% of cases to establish the diagnosis of idiopathic pulmonary fibrosis (IPF). <b><i>Objectives:</i></b> The aim of the study was to analyze the impact of SLB on lung function in patients eventually diagnosed with IPF. <b><i>Methods:</i></b> This is an observational, retrospective, monocentric study of all consecutive patients eventually diagnosed with IPF in multidisciplinary discussion who underwent SLB over 10 years in a specialized center. The primary end point was the variation in forced vital capacity (FVC) before and after the SLB. The secondary end points were the variations in forced expiratory volume in one second (FEV1), total lung capacity (TLC), carbon monoxide diffusion capacity (DLCO), and morbidity and mortality associated with the SLB. <b><i>Results:</i></b> In 118 patients who underwent SLB and were diagnosed with IPF, a relative decrease in FVC of 4.8% (<i>p</i> &#x3c; 0.001) was found between measurements performed before and after the procedure. The mean FVC decrease was 156 ± 386 mL in an average period of 185 days, representing an annualized decline of 363 ± 764 mL/year. A significant decrease was also observed after SLB in FEV1, TLC, and DLCO. Complications within 30 days of SLB occurred in 14.4% of patients. Two patients (1.7%) died within 30 days, where one of them had poor lung function. Survival at 1 year was significantly poorer in patients with FVC &#x3c;50% at baseline. <b><i>Conclusion:</i></b> In this uncontrolled study in patients ultimately diagnosed with IPF, SLB was followed by a significant decline in FVC, which appears to be numerically greater than the average decline in the absence of treatment in the literature. <b><i>Summary at a Glance:</i></b> This study evaluated the change in lung function in 118 consecutive patients diagnosed with idiopathic pulmonary fibrosis by surgical lung biopsy. Forced vital capacity decreased by 156 ± 386 mL in a mean of 185 days between the last measurement before and first measurement after biopsy, representing an annualized decline of 363 ± 764 mL/year.

scholarly journals Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage

2018 ◽  
Vol 2018 ◽  
pp. 1-7 ◽  
Author(s):  
Makoto Shioya ◽  
Mitsuo Otsuka ◽  
Gen Yamada ◽  
Yasuaki Umeda ◽  
Kimiyuki Ikeda ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Vital Capacity ◽  
Total Lung Capacity ◽  
Unknown Etiology ◽  
Advanced Stage ◽  
High Resolution Computed Tomography ◽  
Pleuroparenchymal Fibroelastosis ◽  
Radiological Criteria ◽  
Lung Capacity

Objective. Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease characterized by predominant upper lobe pulmonary fibrosis of unknown etiology. However, the prognosis of IPPFE has not been discussed. We investigated the clinical characteristics and prognostic factors of IPPFE and idiopathic pulmonary fibrosis (IPF). Methods. We performed a retrospective cohort study on 375 consecutive idiopathic interstitial pneumonia patients between April 2004 and December 2014. Among them, we diagnosed IPPFE and IPF patients using high-resolution computed tomography radiological criteria. Results. Twenty-nine IPPFE patients (9 males, 20 females) and 67 IPF patients (54 males, 13 females) were enrolled. IPPFE patients were significantly more likely to be females and nonsmokers and had lower body mass index, lower values of predicted percentage of forced vital capacity (%FVC), and a higher residual volume-to-total lung capacity ratio than IPF patients. Survival analysis revealed that they had significantly poorer prognosis than IPF patients in GAP (gender, age, and physiology) stages II + III. %FVC and GAP index independently predict mortality in patients with IPPFE. Conclusions. Patients with IPPFE showed poorer prognosis in the advanced stage than patients with IPF. %FVC and GAP index are independent predictors of survival in patients with IPPFE.

scholarly journals Automatic quantitative computed tomography measurement of longitudinal lung volume loss in interstitial lung diseases

2022 ◽  
Author(s):  
Salim A. Si-Mohamed ◽  
Mouhamad Nasser ◽  
Marion Colevray ◽  
Olivier Nempont ◽  
Pierre-Jean Lartaud ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Diseases ◽  
Vital Capacity ◽  
Pulmonary Function Tests ◽  
Interstitial Lung Diseases ◽  
Volume Loss ◽  
Lung Capacity ◽  
Lung Ct ◽  
Ct Volume

Abstract Objectives To compare the lung CT volume (CTvol) and pulmonary function tests in an interstitial lung disease (ILD) population. Then to evaluate the CTvol loss between idiopathic pulmonary fibrosis (IPF) and non-IPF and explore a prognostic value of annual CTvol loss in IPF. Methods We conducted in an expert center a retrospective study between 2005 and 2018 on consecutive patients with ILD. CTvol was measured automatically using commercial software based on a deep learning algorithm. In the first group, Spearman correlation coefficients (r) between forced vital capacity (FVC), total lung capacity (TLC), and CTvol were calculated. In a second group, annual CTvol loss was calculated using linear regression analysis and compared with the Mann–Whitney test. In a last group of IPF patients, annual CTvol loss was calculated between baseline and 1-year CTs for investigating with the Youden index a prognostic value of major adverse event at 3 years. Univariate and log-rank tests were calculated. Results In total, 560 patients (4610 CTs) were analyzed. For 1171 CTs, CTvol was correlated with FVC (r: 0.86) and TLC (r: 0.84) (p < 0.0001). In 408 patients (3332 CT), median annual CTvol loss was 155.7 mL in IPF versus 50.7 mL in non-IPF (p < 0.0001) over 5.03 years. In 73 IPF patients, a relative annual CTvol loss of 7.9% was associated with major adverse events (log-rank, p < 0.0001) in univariate analysis (p < 0.001). Conclusions Automated lung CT volume may be an alternative or a complementary biomarker to pulmonary function tests for the assessment of lung volume loss in ILD. Key Points • There is a good correlation between lung CT volume and forced vital capacity, as well as for with total lung capacity measurements (r of 0.86 and 0.84 respectively, p < 0.0001). • Median annual CT volume loss is significantly higher in patients with idiopathic pulmonary fibrosis than in patients with other fibrotic interstitial lung diseases (155.7 versus 50.7 mL, p < 0.0001). • In idiopathic pulmonary fibrosis, a relative annual CT volume loss higher than 9.4% is associated with a significantly reduced mean survival time at 2.0 years versus 2.8 years (log-rank, p < 0.0001).

Fibrosis pulmonar idiopática: Impacto de la biopsia de pulmón en la función pulmonar

2021 ◽  
Vol 3 (1) ◽  
pp. 18-19
Author(s):  
Ricardo Lemus-Rangel
Keyword(s):  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Forced Vital Capacity ◽  
Lung Biopsy ◽  
Vital Capacity ◽  
Forced Expiratory Volume ◽  
Surgical Lung Biopsy ◽  
Before And After ◽  
The Impact

<b>Background:</b> Video-assisted surgical lung biopsy (SLB) is performed in 10–30% of cases to establish the diagnosis of idiopathic pulmonary fibrosis (IPF). <b>Objectives:</b> The aim of the study was to analyze the impact of SLB on lung function in patients eventually diagnosed with IPF. <b>Methods:</b> This is an observational, retrospective, monocentric study of all consecutive patients eventually diagnosed with IPF in multidisciplinary discussion who underwent SLB over 10 years in a specialized center. The primary end point was the variation in forced vital capacity (FVC) before and after the SLB. The secondary end points were the variations in forced expiratory volume in one second (FEV<sub>1</sub>), total lung capacity (TLC), carbon monoxide diffusion capacity (DLCO), and morbidity and mortality associated with the SLB. <b>Results:</b> In 118 patients who underwent SLB and were diagnosed with IPF, a relative decrease in FVC of 4.8% (<i>p</i> &#x3c; 0.001) was found between measurements performed before and after the procedure. The mean FVC decrease was 156 ± 386 mL in an average period of 185 days, representing an annualized decline of 363 ± 764 mL/year. A significant decrease was also observed after SLB in FEV<sub>1</sub>, TLC, and DLCO. Complications within 30 days of SLB occurred in 14.4% of patients. Two patients (1.7%) died within 30 days, where one of them had poor lung function. Survival at 1 year was significantly poorer in patients with FVC &#x3c;50% at baseline. <b>Conclusion:</b> In this uncontrolled study in patients ultimately diagnosed with IPF, SLB was followed by a significant decline in FVC, which appears to be numerically greater than the average decline in the absence of treatment in the literature. Summary at a Glance: This study evaluated the change in lung function in 118 consecutive patients diagnosed with idiopathic pulmonary fibrosis by surgical lung biopsy. Forced vital capacity decreased by 156 ± 386 mL in a mean of 185 days between the last measurement before and first measurement after biopsy, representing an annualized decline of 363 ± 764 mL/year.

scholarly journals Co-trimoxazole to reduce mortality, transplant, or unplanned hospitalisation in people with moderate to very severe idiopathic pulmonary fibrosis: the EME-TIPAC RCT

2021 ◽  
Vol 8 (9) ◽  
pp. 1-110
Author(s):  
Andrew M Wilson ◽  
Allan B Clark ◽  
Anthony Cahn ◽  
Edwin R Chilvers ◽  
William Fraser ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Medical Research ◽  
Forced Vital Capacity ◽  
Research Council ◽  
Vital Capacity ◽  
Medical Research Council ◽  
Leicester Cough Questionnaire

Background Idiopathic pulmonary fibrosis is an irreversible fibrosing lung disorder with a poor prognosis. Current treatments slow the rate of decline in lung function and may influence survival, but they have a significant side-effect profile and so additional therapeutic options are required. People with idiopathic pulmonary fibrosis have altered innate immunity and altered lung microbiota, with the bacterial burden relating to mortality. Two randomised controlled trials have demonstrated beneficial effects with co-trimoxazole (SEPTRIN®; Essential Generics Ltd, Egham, UK; Chemidex Generics Ltd, Egham, UK), with the suggestion of an improvement in rates of survival. Objectives To determine the clinical efficacy of co-trimoxazole in people with moderate to severe idiopathic pulmonary fibrosis. Design A Phase II, double-blind, placebo-controlled, parallel-group, randomised multicentre study. Setting UK specialist interstitial lung disease centres. Participants Patients who were randomised had idiopathic pulmonary fibrosis diagnosed by a multidisciplinary team. In addition, patients had significant breathlessness (i.e. a Medical Research Council Dyspnoea Scale score of > 1) and impaired lung function (i.e. a forced vital capacity of < 75% predicted). Patients could be taking licensed medication for idiopathic pulmonary fibrosis, but were excluded if they had significant comorbidities, including airflow obstruction. Intervention Oral co-trimoxazole, 960 mg twice per day (two 480-mg tablets twice per day), compared with placebo tablets (two tablets twice per day) for a median of 27 months (range 12–42 months). Otherwise, both trial groups had standard care. Main outcome measures The primary outcome was the time to death (all causes), transplant or first non-elective hospital admission. Secondary outcomes were the individual components of the primary end point and the number of respiratory-related events. Questionnaires (the King’s Brief Interstitial Lung Disease questionnaire; the Medical Research Council Dyspnoea Scale; EuroQol-5 Dimensions, five-level version; the Leicester Cough Questionnaire; and the Cough Symptom Score) and lung function tests (forced vital capacity and diffusing capacity for carbon monoxide) were undertaken at baseline and at 12 months. Results The trial randomised a total of 342 (295 male) patients (active treatment group, n = 170; placebo group, n = 172), using minimisation for hospital and receipt of licensed antifibrotic medication, from 39 UK hospitals. The patients had a mean (standard deviation) age of 71.3 years (7.47 years) and a mean forced vital capacity of 2.25 l (0.56 l). A total of 137 (40%) patients were taking pirfenidone (Esbriet, Roche Holding AG, Basel, Switzerland) and 116 (34%) were taking nintedanib (Ofev®, Boehringer Ingelheim, Brackness, UK). There was one post-randomisation exclusion from the co-trimoxazole group, but no withdrawals. There was no difference in the time to event for the composite primary end point (co-trimoxazole: hazard ratio 1.2, 95% confidence interval 0.9 to 1.6; p = 0.319). Likewise, there was no difference in other event outcomes, lung function measurements or patient-reported outcomes, other than a beneficial effect on the total Leicester Cough Questionnaire score, the social domain of the Leicester Cough Questionnaire score and the chest domain of the King’s Brief Interstitial Lung Disease questionnaire in the adjusted analysis. The repeated-measures analysis showed a significant overall difference in Cough Symptom Score. There were significantly more reports of nausea, but fewer reports of diarrhoea, with co-trimoxazole; however, differences in frequency of hyperkalaemia, rash and headache were not significant. The limitations of the trial were that it was not possible to evaluate the lung microbiota, there were missing data for secondary end points and there was no health economic analysis. Conclusion These results suggest that co-trimoxazole does not reduce the likelihood of death or number of hospitalisations among people with idiopathic pulmonary fibrosis with moderate to severe idiopathic pulmonary fibrosis. Further work is required to evaluate the effect in subgroups of individuals with idiopathic pulmonary fibrosis or the effect of antibiotics with different antibacterial properties. Trial registration Current Controlled Trials ISRCTN17464641. Funding This project was funded by the Efficacy and Mechanism Evaluation programme, a Medical Research Council and National Institute for Health Research (NIHR) partnership. This will be published in full in Efficacy and Mechanism Evaluation; Vol. 8, No. 9. See the NIHR Journals Library for further project information.

scholarly journals Benefit and clinical characteristics after the use of nintedanib in idiopathic pulmonary fibrosis

2019 ◽  
Vol 6 (2) ◽  
pp. 29-30
Author(s):  
Herrera-García José Carlos ◽  
Arellano Montellano Ek Ixel ◽  
Jaramillo Arellano Luis Enrique ◽  
Espinosa Arellano Andrea
Keyword(s):  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Adverse Effects ◽  
Clinical Characteristics ◽  
Vital Capacity ◽  
University Hospital ◽  
Antifibrotic Therapy ◽  
Successful Case ◽  
Mexican Patient

Introduction: Nintedanib is an approved antifibrotic agent for the treatment of idiopathic pulmonary fibrosis. (FPI) as monotherapy. To date, the evidence supports its effectiveness in this type of patients. Methodology: We present the case of a Mexican patient of 69 years with Idiopathic Pulmonary Fibrosis (IPF) treated with nintedanib for 52 weeks as monotherapy in a university hospital. Results: Before the 52 week period. There was a clear decrease in the patient's forced vital capacity (FVC) from 70% (2.14L) to 60% (1.83L). The treatment with nintedanib was initiated for a period of 12 months at a dose of 150 mg VO every 12 hrs. Lung function stabilized increasing from 60% (1.83L) to 70% (2.14L), the treatment was well tolerated. Only with presence of mild adverse effects without repercussions. Conclusion: We describe the successful case of a patient with Idiopathic Pulmonary Fibrosis after 52 weeks of treatment with Nintedanib, well tolerated with improved lung function, until now the antifibrotic therapy represents a safe and therapeutically option as monotherapy.

scholarly journals Delay and inequalities in the treatment of idiopathic pulmonary fibrosis: the case of two Nordic countries

2019 ◽  
Vol 13 ◽  
Author(s):  
Ida Pesonen ◽  
Lisa Carlson ◽  
Nicola Murgia ◽  
Riitta Kaarteenaho ◽  
Carl Magnus Sköld ◽  
...  
Keyword(s):  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Transplantation ◽  
Study Data ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Significant Difference ◽  
Antifibrotic Drugs

Background: Idiopathic pulmonary fibrosis (IPF) is characterized by progressive loss of lung function with high mortality within the first 5 years from diagnosis. In 2011–2014, two drugs, pirfenidone and nintedanib, have been approved worldwide for prevention of IPF progression. National IPF-registries have been established in both Finland and Sweden. Our study explored potential differences in the care of IPF in these two countries. Methods: Patients included consecutively in the Finnish and Swedish IPF-registries from January 1, 2014 through December 31, 2016 were included in the study. Data on demographics and lung function at the time of inclusion were collected. Access to antifibrotic drugs and data on disease outcomes, mortality and the proportion of patients who underwent lung transplantation, was collected during a 3-year follow up. Results: One-hundred and fifty-two patients from the Finnish and 160 patients from the Swedish IPF-cohorts were included in the study. At inclusion, Finnish patients were significantly older than the Swedish patients (74.6 years vs 72.5 years, p = 0.017). The proportion of non-smokers was significantly higher in the Finnish cohort (41.7% vs 26.9%, p = 0.007). Forced vital capacity (FVC), % of predicted (78.2 vs 71.7 for Finnish and Swedish patients, respectively, p = 0.01) and diffusion capacity for carbon monoxide (DLCO), % of predicted (53.3 vs 48.2 for Finnish and Swedish patients, respectively, p = 0.002) were significantly higher in the Finnish cohort compared to the Swedish cohort at the time of inclusion. During the 3-year follow up period, 45 (29.6%) Finnish and 111 (69.4%) Swedish patients, respectively, were initiated on treatment with an antifibrotic drug (pirfenidone or nintedanib) (p < 0.001). When comparing possible determinants of treatment, patients with higher FVC % were less likely to start antifibrotic drugs (OR 0.96, 95%CI 0.93–1.00, p < 0.024). To be resident in Sweden was the main determinant for receiving antifibrotic drugs (OR 5.48, 95%CI 2.65–11.33, p < 0.0001). No significant difference in number of deaths and lung transplantation during the follow up period was found. Conclusions: This study highlights differences concerning how IPF patients are treated in Finland and Sweden. How these differences will influence the long-term outcome of these patients is unknown.

scholarly journals Current and future treatment for idiopathic pulmonary fibrosis

2021 ◽  
Vol 64 (4) ◽  
pp. 256-263
Author(s):  
Won-Il Choi
Keyword(s):  
Survival Rate ◽  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Dose Adjustment ◽  
Vital Capacity ◽  
New Drugs ◽  
Lung Function Decline ◽  
Normal Limits ◽  
Near Future

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and fibrosing interstitial lung disease, which is associated with a short survival rate. The decline in forced vital capacity in patients with IPF appears to be almost the same rate regardless of baseline lung function status. This suggests that early treatment would be necessary to prevent further deterioration even lung function is maintained within normal limits. Both pirfenidone and nintedanib significantly slow the decline in lung function, reduce the risk of acute exacerbation, and improve survival rate. However, many individuals with IPF remain untreated. Most IPF patients can tolerate antifibrotic drug therapy, and the dose adjustment has been shown to effectively reduce side effects without modifying efficacy. Although the recent introduction of pirfenidone and nintedanib has led to the slowing of lung function decline, there is no evidence of fibrosis reversal. In the near future, several new drugs are expected to be prescribed to patients with IPF. We are anticipating that some drugs may reverse fibrosis. Fibrosis inhibiting drugs have different pharmacological actions and there are various mechanisms causing fibrosis in the lesion. Therefore, it is imperative to launch efforts to optimize antifibrotic effects through a combination therapy of several drugs. These efforts will hold out hope for patients with IPF.

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