Background: Although patients with idiopathic pulmonary fibrosis (IPF) often receive treatment with antifibrotic drugs (AFDs) and pulmonary rehabilitation (PR) concurrently, there are no reports on the effect of PR on patients with IPF receiving AFDs. Therefore, we investigated the effect of PR on patients with IPF receiving AFDs. Methods: Eighty-seven eligible patients with IPF (61 male; 72.0 ± 8.1 years; GAP severity stage I/II/III: 26/32/12) were recruited for the study. Patients who completed a 3-month outpatient PR program and those who did not participate were classified into four groups according to use of AFDs: PR group (n = 29), PR+AFD group (n = 11), treatment-free observational group (control group; n = 26), and AFD group (n = 21). There was no significant difference in age, sex, or severity among the groups. Patients were evaluated for physical functions such as 6-min walk distance (6MWD) and muscle strength, dyspnea, and health-related quality of life (HRQOL) at baseline and at 3 months. Results: In the PR group, dyspnea and 6MWD showed significant improvement after the 3-month PR program (p < 0.05 and p < 0.01, respectively). HRQOL was significantly worse at 3 months (p < 0.05) in the AFD group, but not in the other groups. The change in 6MWD from baseline to the 3-month time point was significantly higher in the PR+AFD group than in the AFD groups (p < 0.01). Conclusions: It was suggested that AFD treatment reduced exercise tolerance and HRQOL at 3 months; however, the concurrent use of PR may prevent or mitigate these effects.

Background: There are only a few reports on the nutritional status and mortality of patients with idiopathic pulmonary fibrosis (IPF). As such, this study aims to investigate the relationship between controlling nutritional status (CONUT) and the mortality of elderly patients with IPF. Methods: A total of 170 IPF patients aged ≥65 years old who visited the rehabilitation department of our hospital between July 2014 and July 2021 (mean age: 75.7 ± 6.3 years, sex (male/female): 138/32, %FVC: 78.3 ± 18.3%) were retrospectively analyzed. The Kaplan–Meier method and log-rank test were applied. Furthermore, using a Cox proportional hazards model with multivariate analysis, we analyzed the relationship between all-cause mortality and baseline characteristics including CONUT. Results: Based on the CONUT score, the normal group included 101 cases, the mild group included 58 cases, the moderate group included 11 cases, and the severe group had 0 cases. There were 49 cases of all-cause mortality events, suggesting that the mortality of the moderate group was significantly poorer than that of the normal and mild groups (p < 0.05). Furthermore, multivariate analysis identified GAP stage (HR: 5.972, 95%CI: 2.901~12.291, p < 0.0001), mMRC scale (HR: 0.615, 95%CI: 0.389~0.971, p = 0.009), and CONUT (HR: 2.012, 95%CI: 1.192~3.395, p = 0.037) as factors significantly influencing mortality. Conclusions: Severe malnutrition was not observed in elderly patients with IPF. Moderate malnutrition was associated with a significantly higher risk of all-cause mortality, suggesting that CONUT is an important indicator for predicting mortality.

Background: In idiopathic pulmonary fibrosis (IPF), 6-minute walking distance (6MWD) is an independent factor for mortality. Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease with physical features such as emaciation, but the relationship between IPPFE and 6MWD is unclear. In this study, we investigated the factors that cause a decrease in the percent of the predicted value of a 6-minute walk distance (%6MWD), including the disease entities, IPF and IPPFE. Methods: This study included 100 patients (IPF: 75 patients, IPPFE: 25 patients, age: 73.5 ± 7.2 years, sex: 73 males) who visited the rehabilitation department. Patients with a %6MWD ≥ 80% were assigned to the normal group (n = 54), and patients with a %6MWD < 80% were assigned to the decreased group (n = 46). The items showing a significant difference between groups were used as independent variables, after the consideration of multicollinearity, for a logistic analysis where %6MWD < 80% was used as the dependent variable. Results: The 6MWD results show that there was no significant difference between IPF and IPPFE in the absolute value of 6MWD and in the number of people with 6MWD ≥ 250 m, but when 6MWD was compared with %6MWD, the IPPFE group showed a significantly lower value than the IPF group (p = 0.013). Logistic regression analysis showed that only BMI (p = 0.032), GAP index (p = 0.043), and mMRC (p = 0.026) were factors that caused a decrease in %6MWD in 100 patients. Conclusion: The results suggest that leanness, shortness of breath and severity of illness are the most important factors that determine exercise tolerance, regardless of disease entity in IPF and IPPFE.

There are very limited methods of relieving dyspnea that are independent of the causative disease. L-menthol olfactory stimulation is reported to be effective for dyspnea during exercise and inspiratory resistance. Therefore, we examined the effects of L-menthol olfactory stimulation on exertional dyspnea during the 6 min walking distance test (6MWT) in patients with chronic breathlessness syndrome. The subjects who consented to the study were divided into two groups. In Group A, the first 6MWT was performed as usual (placebo) while wearing a surgical mask, and the second 6MWT was performed under the L-menthol condition. In Group B, the first 6MWT was performed under the L-menthol condition, and the second 6MWT was performed as a placebo. A total of 16 subjects (70.8 ± 9.5 years) were included in the analysis. As for the effect of reducing dyspnea, a significant difference was observed in Group A patients who underwent the L-menthol condition in the second 6MWT (p = 0.034). In the comparison of the 6 min walking test under the L-menthol condition and the placebo, the modified Borg scale gain was significantly different between the L-menthol condition and the placebo (p = 0.007). Our results suggested that the L-menthol olfactory stimulation reduced dyspnea on exertion in patients with chronic breathlessness syndrome.