Dae-Wook Kim

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with a poor prognosis. Pirfenidone is approved and widely used for the treatment of IPF and reduces lung function decline. The aim of this study was to evaluate the efficacy of different doses of pirfenidone for the prevention of disease progression in patients with IPF. Methods: This was a prospective, observational, single-center cohort study conducted in Haeundae Paik Hospital, Republic of Korea, from April 2021 to March 2023. IPF patients were assigned to three groups according to the dose of pirfenidone (600 mg, 1200 mg, 1800 mg). Disease progression was defined as an absolute decline to ≥5% of forced vital capacity (FVC) (% predicted value) or an absolute decline to ≥10% of diffusing capacity of the lung for carbon monoxide (DLco) (% predicted value) over 12 months. The primary endpoint was to evaluate the clinical effects of pirfenidone of each dosage on disease progression in IPF patients by comparing the FVC (% predicted value) and DLco (% predicted value) values over 12 months. The secondary endpoint was to evaluate the prognostic value of Krebs von den Lungen-6 (KL-6) in the disease progression in IPF patients using the baseline KL-6 value and the change in KL-6 values between the baseline and 12 months. Results: A total of 44 patients were enrolled, of whom 39 completed the study, with 13 patients assigned to each of the three groups. The median age was 71.7 years, and 79.5% of patients were men. The baseline characteristics were similar across groups, except the 600 mg group was older (75.9 vs. 69.2 vs. 68.2 years, p = 0.016). The overall median change in FVC and DLco over 12 months was −2.7% (IQR: −9.1%, −1.2%) and −3.8% (IQR: −13.6%, −3.7%), respectively. There was no difference in the decline in FVC (change in FVC, % predicted value: −3.23 vs. −4.08 vs. −1.54, p = 0.621) and DLco (change in DLco, % predicted value: 0.00 vs. −3.62 vs. −3.15, p = 0.437) among the three groups. Fourteen patients (35.9%) suffered disease progression. The rate of disease progression did not differ according to the dose of pirfenidone (38.5 vs. 38.5 vs. 30.8%, p = 1.000). In multivariable logistic regression analysis, KL-6 was not a statistically significant predictor of disease progression. Conclusions: In our study, regardless of dose, consistent pirfenidone use for 12 months resulted in similar efficacy for the prevention of disease progression in patients with IPF. Large-scale, randomized, double-blind, placebo-controlled clinical trials are needed.

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with a poor prognosis. Pirfenidone is approved and widely used for the treatment of IPF and reduces lung function decline. The aim of this study was to evaluate the efficacy of different doses of pirfenidone for the prevention of disease progression in patients with IPF. Methods: This was a prospective, observational, single-center cohort study conducted in Haeundae Paik Hospital, Republic of Korea, from April 2021 to March 2023. IPF patients were assigned to three groups according to the dose of pirfenidone (600mg, 1,200mg, 1,800mg). The primary endpoint was the efficacy of pirfenidone according to dose for the prevention of disease progression over 12 months. The secondary endpoint was the role of Krebs von den Lungen-6 (KL-6) in predicting disease progression. Results: A total of 44 patients were enrolled, of whom 39 completed the study, with 13 patients assigned to each of the three groups. The median age was 71.7 years, and 79.5% of patients were men. The baseline characteristics were similar across groups, except the 600mg group was older (75.9 vs. 69.2 vs. 68.2 years, p = 0.016). Fourteen patients (35.9%) suffered disease progression. The rate of disease progression did not differ according to the dose of pirfenidone (38.5 vs. 38.5 vs. 30.8%, p = 1.000). In multivariable logistic regression analysis, KL-6 was not a statistically significant predictor of disease progression. Conclusions: In our study, regardless of dose, consistent pirfenidone use for 12 months resulted in similar efficacy for the prevention of disease progression in patients with IPF. Large-scale, randomized, double-blind, placebo-controlled clinical trials are needed.

Background: This study evaluated the efficacy and safety of pulmonary rehabilitation (PR) on functional performance, exercise-related oxygen saturation, and health-related quality of life among patients with idiopathic pulmonary fibrosis (IPF). Methods: A total of 25 patients with IPF (13 in the PR group and 12 in the non-PR group) were enrolled between August 2019 and October 2021 at Haeundae-Paik Hospital in the Republic of Korea. A cardiopulmonary exercise test (CPET), six-minute walk test (6MWT), pulmonary function test (PFT), Saint George’s Respiratory Questionnaire (SGRQ), muscle strength test, and bioelectrical impedance analysis were performed in each group at baseline and after eight weeks of PR. Results: The mean age was 68 years of age and most subjects were male. Baseline characteristics were similar between the two groups. The distance during 6MWT after PR was significantly improved in the PR group (inter-group p-value = 0.002). VO₂max and VE/VCO₂ slopes showed a significant difference after eight weeks only in the PR group, but the rate of change did not differ significantly from the non-PR group. Total skeletal muscle mass, PFT variables, and SGRQ scores did not differ significantly between the groups. Conclusions: PR improved exercise capacity, as measured using CPET and 6 MWT. Further studies in larger samples are needed to evaluate the long-term efficacy of PR in IPF patients.