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: Dysphagia is one of the most serious complications of occipitocervical fusion (OCF). The previous studies have shown that postoperative cervical alignment, documented with occipito (O)-C2 angles, C2-C6 angles, and pharyngeal inlet angles (PIA), impacted the incidence of postoperative dysphagia in patients undergoing OCF. Here, we investigated the relationship of preoperative versus postoperative cervical alignment on the incidence of postoperative dysphagia after OCF. Methods: We retrospectively reviewed the clinical data/medical charts for 22 patients following OCF (2006– 2019). The O-C2 angles, C2-C6 angles, PIA, and narrowest pharyngeal airway spaces (nPAS) were assessed using plain lateral radiographs of the cervical spine before and after the surgery. The severity of dysphagia was assessed with the functional oral intake scale (FOIS) levels as documented in medical charts; based on this, patients were classified into the nondysphagia (FOIS: 7) versus dysphagia (FOIS: 1–6) groups. Results: Seven patients (35%) experienced dysphagia after OCF surgery. Preoperative PIA and nPAS were smaller in the dysphagia group. Spearman rank correlation showed a positive correlation between preoperative PIA and FOIS and between preoperative nPAS and FOIS. Conclusion: This study suggests that preoperative cervical alignment may best predict the incidence of postoperative dysphagia after OCF.

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.

間質性肺炎の診断・治療において要となるのが原因と病型を確定することであり，それには集学的検討が重要である．さらに，間質性肺炎の経過は症例ごとに進行速度やパターンが異なるため，治療や慢性安定期の段階においても個々の症例に合わせたリハビリテーション，栄養，生活指導，服薬指導などあらゆる観点からの集学的検討を行い，「呼吸困難→活動性低下→廃用→さらなる呼吸困難」の負のスパイラルを逆回転させる必要がある．さらに，逆回転の効果の維持には患者によるセルフマネジメントも重要であり，医療者と患者で協働する包括的呼吸リハビリテーションが重要である．