Prognostic significance of forced vital capacity decline prior to and following antifibrotic therapy in idiopathic pulmonary fibrosis

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal interstitial lung disease (ILD). Currently, two antifibrotic drugs are available for reducing forced vital capacity (FVC) decline in IPF. However, many pulmonologists wait before initiating treatment, especially when IPF patients have stable disease. This study aimed to investigate the impact on survival outcome of FVC decline and a slow rate of FVC decline prior to and following treatment with these two antifibrotic drugs. Methods: Out of the 235 IPF patients treated with antifibrotic therapy that were screened, 105 cases were eligible, who then underwent physiological evaluation at 6 months prior to and following antifibrotic therapy. Clinical characteristics and prognostic outcomes were compared among groups, and prognostic factors were evaluated using a Cox proportional hazards analysis. Results: In terms of %FVC decline prior to the therapy and a slow rate of FVC decline, there was no significant difference between stable and worsened groups and responder and non-responder groups, respectively. On the other hand, in terms of %FVC decline (decline >5%) following antifibrotic therapy, the stable/improved group had significantly better prognosis than the worsened group. Prognostic analysis revealed that a stable/improved status following antifibrotic therapy [HR: 0.35 (0.15–0.87)] was significantly associated with a better prognosis. Conclusions: Concerning the FVC decline prior to and following antifibrotic therapy and a slow rate of FVC decline, only the FVC decline following the therapy is associated with a greater survival outcome. An early treatment decision may thus be beneficial for IPF. The reviews of this paper are available via the supplemental material section.

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Impact of Lung Biopsy on Lung Function in Idiopathic Pulmonary Fibrosis

Respiration ◽

10.1159/000509557 ◽

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

Background: Video-assisted surgical lung biopsy (SLB) is performed in 10–30% of cases to establish the diagnosis of idiopathic pulmonary fibrosis (IPF). Objectives: The aim of the study was to analyze the impact of SLB on lung function in patients eventually diagnosed with IPF. Methods: 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. Results: In 118 patients who underwent SLB and were diagnosed with IPF, a relative decrease in FVC of 4.8% (p < 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 <50% at baseline. Conclusion: 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.

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Effect of continued antifibrotic therapy after forced vital capacity decline in patients with idiopathic pulmonary fibrosis; a real world multicenter cohort study

Respiratory Medicine ◽

10.1016/j.rmed.2021.106722 ◽

2021 ◽

pp. 106722

Author(s):

Colin J. Adams ◽

Shane Shapera ◽

Christopher J. Ryerson ◽

Deborah Assayag ◽

Kerri A. Johannson ◽

...

Keyword(s):

Cohort Study ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Real World ◽

Forced Vital Capacity ◽

Vital Capacity ◽

Multicenter Cohort Study ◽

Antifibrotic Therapy ◽

Multicenter Cohort

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

Karger Kompass Neumología ◽

10.1159/000514300 ◽

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

Background: Video-assisted surgical lung biopsy (SLB) is performed in 10–30% of cases to establish the diagnosis of idiopathic pulmonary fibrosis (IPF). Objectives: The aim of the study was to analyze the impact of SLB on lung function in patients eventually diagnosed with IPF. Methods: 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. Results: In 118 patients who underwent SLB and were diagnosed with IPF, a relative decrease in FVC of 4.8% (p < 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 <50% at baseline. Conclusion: 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.

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Effect of antifibrotic agents on survival in idiopathic pulmonary fibrosis patients according to forced vital capacity: a real-world retrospective cohort study in Japan

10.21203/rs.3.rs-34466/v1 ◽

2020 ◽

Author(s):

Sho Saeki ◽

Osamu Nishiyama ◽

Ryo Yamazaki ◽

Kazuya Yoshikawa ◽

Ken Shirahase ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Forced Vital Capacity ◽

Retrospective Cohort ◽

Vital Capacity ◽

Survival Prognosis ◽

Antifibrotic Therapy ◽

Severe Group ◽

The Mean ◽

Antifibrotic Agents

Abstract Background Antifibrotic agents suppress the decline in forced vital capacity (FVC) and disease progression in idiopathic pulmonary fibrosis (IPF) patients. However, their effect on survival prognosis and differences in this effect according to baseline lung function have been unexplored. Therefore, this study aimed to examine the effect of antifibrotics on survival prognosis and whether this effect differed according to baseline FVC. Methods Consecutive IPF patients from January 2008 to May 2019 were examined retrospectively. FVC and effect of pirfenidone or nintedanib therapy were assessed. FVC at registration was used to categorize the patients into mild: FVC % predicted ≥ 80%, moderate: FVC % predicted 50–80%, and severe: FVC % predicted < 50% IPF groups. Results In total, 172 IPF patients were included. The mean FVC % predicted was 77.4 ± 22.2%. The median survival periods of patients in the mild, moderate, and severe IPF groups were 1,452, 1,305, and 481 days, respectively. Significant differences were observed in survival between the mild and severe groups and the moderate and severe groups (p < 0.0001), but not between the mild and moderate groups (p = 0.20). The survival was longer in patients on antifibrotic therapy in the mild (p = 0.18) and moderate groups (p = 0.04), but not in the severe group (p = 0.93). Conclusions Antifibrotics extended the survival of IPF patients. The effect was obvious in patients with FVC % predicted of 50–80%, a tendency was observed in patients with FVC % predicted ≥ 80%, while no effect was observed in patients with FVC % predicted < 50%.

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Effect of nintedanib on decline in forced vital capacity (FVC) over time in the INPULSIS trials in patients with idiopathic pulmonary fibrosis (IPF)

Pneumologie ◽

10.1055/s-0037-1598505 ◽

2017 ◽

Vol 71 (S 01) ◽

pp. S1-S125

Author(s):

S Gläser ◽

TM Maher ◽

KR Flaherty ◽

A Azuma ◽

V Cottin ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Forced Vital Capacity ◽

Vital Capacity ◽

Over Time

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Analysis of patients with idiopathic pulmonary fibrosis (IPF) with percent predicted forced vital capacity (FVC) < 50% treated with pirfenidone in RECAP

Pneumologie ◽

10.1055/s-0037-1598500 ◽

2017 ◽

Vol 71 (S 01) ◽

pp. S1-S125

Author(s):

U Costabel ◽

C Albera ◽

KU Kirchgaessler ◽

F Gilberg ◽

U Petzinger ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Forced Vital Capacity ◽

Vital Capacity

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Efficacy of early antifibrotic treatment for idiopathic pulmonary fibrosis

BMC Pulmonary Medicine ◽

10.1186/s12890-021-01595-3 ◽

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.

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