Pulmonary rehabilitation and noninvasive ventilation in patients with hypercapnic interstitial lung disease.

BACKGROUND: Pulmonary rehabilitation (PR) has a positive impact on functional status and quality of life in patients with interstitial lung disease (ILD). OBJECTIVES: This study investigated the effects of PR in hypercapnic ILD patients receiving nighttime noninvasive positive pressure ventilation (NPPV). METHODS: Consecutive ILD patients referred to a specialized inpatient PR center were included. All participated in a PR program. Those with hypercapnia received NPPV (NPPV group; n = 29); the remaining patients served as comparison group (n = 319). RESULTS: PR improved the 6-min walk distance by 64.4 ± 67.1 m versus baseline (p < 0.0001) in NPPV patients and by 43.2 ± 55.1 m (p < 0.0001) in the comparison group (difference 21.1 m, 95% confidence interval 0.5-41.8; p = 0.045). There was no change in total lung capacity during PR in NPPV recipients or the comparison group. Forced vital capacity significantly increased from baseline in the comparison, but not the NPPV group. NPPV recipients were significantly more likely than the comparison group to have improved dyspnea during PR (p = 0.049). There was no improvement in the 36-item Short Form (SF-36) physical component score in the NPPV group after PR, but there was in the comparison group. PR improved the SF-36 mental component score versus baseline in both groups. CONCLUSION: An individually tailored PR plus nighttime NPPV appears feasible in hypercapnic ILD patients and significantly improves exercise capacity and quality of life.

Effects of inpatient pulmonary rehabilitation in patients with interstitial lung disease.

Pulmonary rehabilitation is recommended for patients with chronic lung diseases including idiopathic pulmonary fibrosis according to international guidelines. However, data for patients with interstitial lung disease (ILD) are limited. We examined the effect of an inpatient pulmonary rehabilitation on functional status and quality of life in ILD patients. We evaluated 402 consecutive ILD patients who were admitted to a specialised pulmonary rehabilitation centre (1999-2010). All patients performed a standardised pulmonary rehabilitation programme including pulmonary function tests, blood-gas analysis, 6-min walk test (6MWT), dyspnoea rating and health-related quality of life questionnaire (the 36-item short-form health survey; SF-36) on admission and discharge. Mean duration of pulmonary rehabilitation was 30±1 days. 6MWT distance improved by 46±3 m (308±6 m versus 354±6 m; p<0.001). Dyspnoea rating did not change. Lung function testing showed marginal improvement of vital capacity (+1±0%; p = 0.002). The SF-36 questionnaire demonstrated an increase in all eight sub-scores as well as in the physical and mental health summary scores (physical 6±1 points, p<0.001; mental health 10±1 points, p<0.001). Moreover, patients with signs of pulmonary hypertension also benefited from pulmonary rehabilitation. In a large cohort of patients with ILD, pulmonary rehabilitation had a positive impact on functional status and quality of life. Considering the limited treatment options in this patient population pulmonary rehabilitation appears to be a valuable adjunct therapy.

Newly detected rapid eye movement associated sleep apnea after coronavirus disease 2019 as a possible cause for chronic fatigue: two case reports.

BACKGROUND: Coronavirus disease 2019 has become a health problem spreading worldwide with pandemic characteristics since March 2020. Post coronavirus disease 2019 symptoms are more frequent than initially expected, with fatigue as an often-mentioned issue. CASE PRESENTATIONS: We describe a 32-year-old white male and a 55-year-old white female who suffered from post coronavirus disease 2019 fatigue syndrome. On polysomnography, rapid eye movement associated sleep apnea with an increased hypopnea index during rapid eye movement phases of 36.8 and 19.5 events per hour was found. Based on the patients' burdensome fatigue symptoms, we initiated automatic positive airway pressure therapy, which diminished sleep apnea (rapid eye movement index: 0.0 in both patients) and, consequently, also the fatigue symptoms. CONCLUSIONS: Since sleep apnea and coronavirus disease 2019 are both associated with fatigue, a screening for sleep apnea might be considered in coronavirus disease 2019 patients with fatigue syndrome.

Relationship between body composition, exercise capacity and health-related quality of life in idiopathic pulmonary fibrosis.

INTRODUCTION: Bioelectrical impedance analysis (BIA) can be used to estimate Fat-Free Mass Index (FFMI). However, the use of directly measured BIA variables, such as phase angle (PhA), has gained attention. The frequency of low FFMI and PhA and its associations with exercise capacity and health-related quality of life (HRQL) in patients with idiopathic pulmonary fibrosis (IPF) have been scarcely studied. OBJECTIVES: To investigate the frequency of low FFMI and PhA and their associations with exercise capacity and HRQL in patients with IPF. METHODS: Patients underwent assessment of lung function, body composition, exercise capacity by the 6 min walk distance (6MWD), and HRQL by the Medical Outcomes Study Short-Form 36-item Questionnaire (SF-36). Patients were classified as presenting normal or low PhA or FFMI, accordingly to the 10th percentiles of age-sex-body mass index (BMI)-specific reference values. RESULTS: 98 patients (84 males, age: 68±8 years, forced vital capacity: 64%±18%predicted) were included. 24 patients presented low PhA. They were characterised by worse lung function, exercise capacity and HRQL compared with patients with normal PhA. 10 patients presented low FFMI, but despite differences in body composition, no differences were found between these patients and patients with normal FFMI. In a single regression analysis, age, lung function and body composition variables (except FFMI) were related to 6MWD and SF-36 Physical Summary Score (R²=0.06-0.36, p<0.05). None of the variables were related to SF-36 Mental Summary Score. CONCLUSION: One-fourth of the patients with IPF with normal to obese BMI present abnormally low PhA. Patients classified as low PhA presented worse lung function, exercise capacity and HRQL.

Protocol for an observational study to identify potential predictors of an acute exacerbation in patients with chronic obstructive pulmonary disease (the PACE Study).

INTRODUCTION: Acute exacerbations of chronic obstructive pulmonary disease (AECOPD) are the most critical events for patients with COPD that have a negative impact on patients' quality of life, accelerate disease progression, and can result in hospital admissions and death. Although there is no distinct definition or detailed knowledge about AECOPD, it is commonly used as primary outcome in clinical studies. Furthermore, it may be difficult in clinical practice to differentiate the worsening of symptoms due to an AECOPD or to the development of heart failure. Therefore, it is of major clinical importance to investigate the underlying pathophysiology, and if possible, predictors of an AECOPD and thus to identify patients who are at high risk for developing an acute exacerbation. METHODS AND ANALYSIS: In total, 355 patients with COPD will be included prospectively to this study during a 3-week inpatient pulmonary rehabilitation programme at the Schoen Klinik Berchtesgadener Land, Schoenau am Koenigssee (Germany). All patients will be closely monitored from admission to discharge. Lung function, exercise tests, clinical parameters, quality of life, physical activity and symptoms will be recorded, and blood samples and exhaled air will be collected. If a patient develops an AECOPD, there will be additional comprehensive diagnostic assessments to differentiate between cardiac, pulmonary or cardiopulmonary causes of worsening. Follow-up measures will be performed at 6, 12 and 24 months.Exploratory data analyses methods will be used for the primary research question (screening and identification of possible factors to predict an AECOPD). Regression analyses and a generalised linear model with a binomial outcome (AECOPD) will be applied to test if predictors are significant. ETHICS AND DISSEMINATION: This study has been approved by the Ethical Committee of the Philipps University Marburg, Germany (No. 61/19). The results will be presented in conferences and published in a peer-reviewed journal. TRIAL REGISTRATION NUMBER: NCT04140097.

Cardiopulmonary response during whole-body vibration training in patients with severe COPD.

Several studies in patients with chronic obstructive pulmonary disease (COPD) have shown that whole-body vibration training (WBVT) has beneficial effects on exercise capacity. However, the acute cardiopulmonary demand during WBVT remains unknown and was therefore investigated in this study. Ten patients with severe COPD (forced expiratory volume in 1 s: 38±8% predicted) were examined on two consecutive days. On day one, symptom-limited cardiopulmonary exercise testing was performed on a cycle ergometer. The next day, six bouts of repeated squat exercises were performed in random order for one, two or three minutes either with or without WBVT while metabolic demands were simultaneously measured. Squat exercises with or without WBVT induced comparable ventilatory efficiency (minute ventilation (VE)/carbon dioxide production (V'CO2 ): 38.0±4.4 with WBVT versus 37.4±4.1 without, p=0.236). Oxygen uptake after 3 min of squat exercises increased from 339±40 mL·min-1 to 1060±160 mL·min-1 with WBVT and 988±124 mL min-1 without WBV (p=0.093). However, there were no significant differences between squat exercises with and without WBVT in oxygen saturation (90±4% versus 90±4%, p=0.068), heart rate (109±13 bpm versus 110±15 bpm, p=0.513) or dyspnoea (Borg scale 5±2 versus 5±2, p=0.279). Combining squat exercises with WBVT induced a similar cardiopulmonary response in patients with severe COPD compared to squat exercises without WBVT. Bearing in mind the small sample size, WBVT might be a feasible and safe exercise modality even in patients with severe COPD.

Predictors of success for pulmonary rehabilitation in patients awaiting lung transplantation.

BACKGROUND: Numerous studies have shown the effectiveness of comprehensive pulmonary rehabilitation (PR) programs in chronic obstructive pulmonary disease (COPD). At present, it is uncertain whether PR is also effective in the management of patients with various diseases awaiting lung transplantation (LTx). METHODS: In a retrospective clinical preanalysis and postanalysis, we investigated the effects of a 5-week inpatient PR in LTx candidates. We compared data of patients with COPD, α-1-antitrypsin deficiency, interstitial lung disease, or cystic fibrosis before and after PR with regard to exercise capacity (6-min walking distance [6MWD]) and health-related quality of life (HRQL; SF36). Multivariate stepwise regression analysis was performed to detect predictors of PR outcome. RESULTS: Eight hundred eleven data sets from consecutive patients referred to our PR center before LTx could be analysed (COPD, 360; α-1-antitrypsin deficiency, 127; interstitial lung disease, 195; cystic fibrosis, 69; other, 60). After PR 6MWD increased by 56 ± 58 m (P < 0.001), the physical summary component of SF36 (0-100) improved by 1.9 ± 8.5 points (P < 0.001), the mental summary component of SF36 by 8.7 ± 13.5 points (P < 0.001). Stepwise regression analysis yielded no relevant predictors of success or nonsuccess of PR with regard to age, sex, disease, body mass index, 6MWD, and HRQL on admission. CONCLUSION: Short-term comprehensive PR can significantly improve exercise capacity and HRQL in LTx candidates to a clinically relevant extent independent of the underlying lung disease. No relevant predictor influencing PR outcome could be detected. Further research is needed to evaluate the relevance of PR before LTx and its impact on clinical outcome after transplantation.

Immunoglobulin E production in chronic pancreatitis.

OBJECTIVES: Serum immunoglobulin E (IgE) was investigated in patients with chronic pancreatitis in order to elucidate possible disease mechanisms linking pancreatitis, adverse reaction to foods and allergy. DESIGN AND METHODS: Serum IgE was analysed in 86 non-atopic patients with advanced chronic pancreatitis and 54 non-atopic controls. IgE detection was performed by enzyme-linked immunofluorescence. RESULTS: Mean IgE levels (+/- standard error of mean) in chronic pancreatitis patients (286.1+/-49 kU/l) were found to be significantly elevated compared to controls (67.7+/-11 kU/l; P<0.0001). Normal IgE levels (<100 kU/l) were present in 40/54 control patients (74.1%), but only 39/86 pancreatitis patients (45.3%). Of the patients with chronic pancreatitis, 47/86 (54.6%) had clearly elevated IgE levels of >100 kU/l and their IgE values did not show a Gaussian distribution. However, nine-fold higher IgE levels were found in chronic pancreatitis patients with alcohol consumption of >25 g/day and exocrine insufficiency (915.5+/-240 kU/l) than in pancreatitis patients with normal exocrine function and no alcohol consumption (103.4+/-43 kU/l; P<0.001). Moreover, acute episodes of chronic pancreatitis were found to increase serum IgE levels. CONCLUSIONS: In patients with chronic pancreatitis, serum IgE production is markedly enhanced, especially during acute inflammatory episodes or when alcohol is consumed. Since abstinence from alcohol and pancreatic enzyme substitution are associated with clearly lower IgE levels, it may be concluded that pancreatic insufficiency with reduced nutrient digestion and alcohol consumption stimulate IgE production. This finding gives rise to the speculation that, apart from pancreatic inflammation, cross-linking of IgE with alimentary or other antigens might be involved in the pathophysiology of a sub-population of patients with chronic pancreatitis and manifest pancreatic insufficiency.

Noninvasive ventilation in pulmonary rehabilitation of COPD patients.

Noninvasive positive pressure ventilation (NIPPV) has been shown to improve exercise tolerance and health-related quality of life in patients with advanced COPD. This study tested the feasibility of nocturnal NIPPV as an additional tool in a hospital-based pulmonary rehabilitation program. This prospective observational trial included forty COPD patients in GOLD stage IV. NIPPV was successfully introduced and accepted during sleep by all patients. All patients received pressure support ventilation for 7.9+/-0.5h per day with an inspiratory support of 17.5+/-4.4 cmH(2)O, and an expiratory pressure of 4.5+/-0.9 cmH(2)O. The outcome of pulmonary rehabilitation in patients receiving nocturnal NIPPV was compared with the results of forty matched control patients who underwent the same program. Rehabilitation with nocturnal NIPPV resulted in the 6-minute walk test and in the longest non-stop walk distance in improvements of 82 and 89 m, respectively, while patients without nocturnal ventilatory support improved by 50 and 51 m (p<0.04 and p<0.03 between groups, respectively). Further significant improvements were found for FEV(1), lung hyperinflation, and blood gases in the NIPPV treated, but not in the control subjects. Health-related quality of life, assessed by the SF-36 questionnaire, improved moderately or largely in patients receiving NIPPV in the categories role-physical, vitality, social function, and mental health. Control subjects improved moderately in vitality only. In conclusion, nocturnal NIPPV is feasible and enhances the effects of pulmonary rehabilitation in advanced stage COPD.