Appendicular Skeletal Muscle Mass Correlates with Patient-Reported Outcomes and Physical Performance in Patients with Idiopathic Pulmonary Fibrosis.

Idiopathic pulmonary fibrosis (IPF), an incurable lung disease of unknown cause, often presents with losses of skeletal muscle mass. IPF requires comprehensive care, but it has not been investigated which skeletal muscle mass index reflects holistic management factors: pulmonary function, patient-reported outcomes (PROs), and physical performance. We compared three representative indices of skeletal muscle mass with holistic management factors in IPF patients. Twenty-seven mild to severe IPF patients (21 male) with the mean age of 76.1 ± 5.9 years were enrolled. The three indices were appendicular skeletal muscle mass index (ASMI), cross-sectional area of pectoralis major (PMCSA), and cross-sectional area of erector spinae muscles (ESMCSA). ASMI is considered as a gold standard for sarcopenia assessment, while PMCSA and ESMCSA are frequently used in IPF. As PROs, we assessed breathlessness with the modified Medical Research Council dyspnea scale (mMRC), symptoms with the chronic obstructive pulmonary disease assessment test (CAT), and health-related quality of life with St. George's Respiratory Questionnaire (SGRQ). For physical performance, peripheral muscle strength and 6-min walk distance (6MWD) were investigated. In this cross-sectional study, ASMI showed the greatest number of significantly correlated indices, such as pulmonary function, peripheral muscle strength, 6MWD, mMRC, and SGRQ. PMCSA showed the next greatest number of correlations, with peripheral muscle strength, 6MWD, and mMRC, whereas ESMCSA showed no significant correlations with any index. Thus, ASMI correlated with both PROs and physical performance, and PMCSA correlated mainly with physical performance. In conclusion, assessing ASMI is helpful for the comprehensive care of patients with IPF.

L-Menthol Olfactory Stimulation Reduced Dyspnea Sensation during the 6 min Walk Test in Patients with Chronic Breathlessness Syndrome: A Pilot Study.

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.

Determinants of Six-Minute Walk Distance in Idiopathic Pulmonary Fibrosis and Idiopathic Pleuroparenchymal Fibroelastosis.

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.

Benefits of Pulmonary Rehabilitation in Patients with Idiopathic Pulmonary Fibrosis Receiving Antifibrotic Drug Treatment.

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.