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Background During exercise, dynamic hyperinflation (DH), measured by a reduction in inspiratory capacity (IC), increases exertional dyspnea and reduces functional capacity in many patients with severe COPD. Although noninvasive ventilation (NIV) during exercise can improve exercise duration, the effect on DH is unclear. Research Questions In people with COPD, resting hyperinflation, and evidence of DH during exercise, does bilevel NIV during exercise reduce DH and increase endurance time compared with exercise with no NIV, and does NIV with an individually titrated expiratory positive airway pressure (T-EPAP) reduce DH and increase exercise endurance time more than does NIV with standardized EPAP (S-EPAP) of 5 cm H2O? Study Design and Methods A randomized crossover trial in which investigators and participants were blinded between NIV interventions was performed. Participants (N = 19; FEV1 of 1.02 ± 0.24 L (39% ± 6% predicted) completed three constant work rate endurance cycle tests in random order—no NIV, NIV with S-EPAP, and NIV with T-EPAP—during exercise. Primary outcomes were isotime IC and exercise endurance time. Outcome measures from each intervention were compared at isotime and at end exercise by using a linear mixed-model analysis. Results Compared with those with no NIV, isotime IC and endurance time were greater with both NIV with S-EPAP (mean difference: 95% CI, 0.19 L [0.10-0.28]; 95% CI, 153 s [24-280], respectively) and T-EPAP (95% CI, 0.22 L [0.13-0.32]; 95% CI, 145 s [28-259], respectively). There was no difference between NIV with S-EPAP and NIV with T-EPAP. Interpretation In people with COPD and DH during exercise, NIV during exercise reduced DH and increased cycle endurance time. An S-EPAP of 5 cm H2O was adequate to obtain these benefits. Trial Registry Australian New Zealand Clinical Trials Registry; No.: ACTRN12613000804785; URL: http://www.anzctr.org.au

PURPOSE Pulmonary rehabilitation (PR) has been shown to be an effective intervention in patients with very severe chronic obstructive pulmonary disease (COPD) awaiting lung transplantation (LTx). The objective of this study was to characterize the prevalence of acute exacerbations (AEs) during PR and their impact on the outcomes of pre-LTx PR. METHODS In this retrospective analysis, 559 patients with COPD awaiting LTx who were referred to a 4-wk inpatient PR program were evaluated. A total of 114 patients (20%) acquired an AE during PR and continued in an adapted fashion. Pulmonary function testing, 6-min walk test (6MWT), and a health-related quality-of-life questionnaire (SF-36) were administered on admission and on discharge of PR. RESULTS Following PR, both groups, patients with and without AE, increased their 6MWT significantly (P < .001) to a clinically relevant amount (58 ± 72 and 52 ± 64 m, respectively). The sum scores of the SF-36 also improved significantly without any between-group differences. No observed changes were different between the two groups. No relevant predictors for PR outcomes could be detected by logistic regression. CONCLUSIONS Our data show that patients with end-stage COPD listed for LTx can achieve clinically relevant improvements in functional exercise capacity and quality of life even if they develop an AE during PR.

The perceptions of using noninvasive ventilation (NIV) during exercise in patients with COPD who are naïve to NIV is unknown. The present study aimed to examine the perceptions of using NIV during exercise in people with COPD and to determine the relationship between patient perceptions with both baseline patient characteristics and exercise outcomes.During a trial examining the effect of NIV during exercise on dynamic hyperinflation in people with COPD who were naïve to NIV, participants completed a 5-point Likert scale questionnaire (scored strongly disagree -2 to strongly agree +2) before and after using NIV during exercise and a semi-structured interview after using NIV during exercise.Eighteen participants, mean age (SD) 69 (7) y, FEVIndividuals with COPD, naïve to NIV, and using NIV during exercise for the first time reported a positive effect of NIV on breathlessness and exercise performance. Participants' perceived benefit of NIV correlated moderately with increased endurance time and resting hyperinflation and with a reduction in dynamic hyperinflation during exercise, suggesting that patient reports could also aid selection of those who will benefit from NIV during exercise.

IntroductionBioelectrical 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.ObjectivesTo investigate the frequency of low FFMI and PhA and their associations with exercise capacity and HRQL in patients with IPF.MethodsPatients 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.Results98 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, pConclusionOne-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.

Zusammenfassung Hintergrund Pulmonale Hypertonie (PH) ist definiert als ein Anstieg des mittleren pulmonal-arteriellen Drucks auf > 20 mmHg in Ruhe, der auf Dauer zu einer Rechtsherzinsuffizienz führen kann. Bis vor einigen Jahren wurde PH-Patienten wegen der Befürchtung einer möglichen Symptomverschlechterung und dem Auftreten unerwünschter, belastungsassoziierter klinischer Ereignisse von körperlichem Training abgeraten. Methoden Drei elektronische Datenbanken wurden mit folgenden Suchbegriffen nach randomisiert, kontrollierten Studien durchsucht, in denen eine Trainingsintervention bei PH-Patienten untersucht wurde: „pulmonary hypertension ODER „pulmonary arterial hypertension“ UND „exercise“ ODER „pulmonary rehabilitation“ UND „randomized“. Ergebnisse In dieser Metaanalyse konnten Daten von 5 Studien mit insgesamt 187 PH-Patienten eingeschlossen werden, welche 3- bis 12-wöchige Trainingsprogramme (z. B. 10 – 45 Minuten Ergometertraining; 60 – 80 % der maximalen Herzfrequenz) absolvierten. Patienten in den Trainingsgruppen wiesen im Vergleich zur Kontrollgruppe signifikante (p Schlussfolgerung Ein individuell angepasstes Trainingsprogramm bei stabilen PH-Patienten kann nach zuvor optimierter medikamentöser Therapie die körperliche Belastbarkeit und Lebensqualität ohne nachweisbare klinische Gefährdung verbessern. Diese Einschätzungen basieren bisher nur auf kleinen Fallzahlen und größere, randomisierte Studien mit unterschiedlichen PH-Schweregraden sind dringend notwendig.

Background Supplemental oxygen is a recommended therapy option in stable hypoxemic COPD patients. Often, supplemental oxygen is provided by continuous flow (CF). However, demand oxygen delivery systems (DODS) that provide an oxygen bolus only during inspiration have gained increasing use as they prolong oxygen cylinder life (beside battery life). However, there is a lack of evidence if different DODS and CF devices are equivalent. Methods Seventy hypoxemic COPD patients (FEV1 32 ± 9% predicted, PaO2 56±7 mmHg) on long-term oxygen therapy were included in this prospective single-blinded, randomized cross-over trial. Following an initial incremental shuttle walk test, patients performed 3 endurance shuttle walk tests (ESWT) at 85% of their maximum walking speed in random order with: (A) CF (ESWT-CF), (B) a DODS based on liquid oxygen (ESWT-DL) and (C) an DODS oxygen concentrator (ESWT-DC). The primary outcome was oxygen saturation (SpO2) at ESWT isotime. Secondary outcomes were total ESWT duration, heart rate (HR) and breathing frequency (BF) at isotime and dyspnea at end-exercise. Results SpO2 at ESWT isotime was not clinically different between devices: 90 ± 4% (CF), 89 ± 5% (DL) and 90 ± 5% (DC). However, 20% of the patients showed a ≥4% lower oxygen desaturation while using a DODS device. Secondary outcomes were similar under the three conditions. Conclusion Oxygen supplementation via DODS (based on liquid oxygen or as a concentrator) yielded comparable physiological effects during standardized walking in stable hypoxemic COPD patients like CF. However, 20% of patients showed a clinically relevant lower oxygen saturation while using a DODS device. Therefore, we suggest individual testing of oxygen saturation of DODS suitability.

Background:\ud Cognitive impairment might interfere with the efficacy of Pulmonary Rehabilitation (PR) in Chronic Obstructive Pulmonary Disease (COPD). We aimed to identify differential responses to PR between cognitively impaired (CI) and cognitively normal (CN) COPD patients by assessing health status and exercise capacity.\ud \ud Methods:\ud Sixty patients (FEV1: 47 ± 15%) were classified as CI or CN according to the Montreal Cognitive Assessment (MoCA ≤25points) and completed a 3-week inpatient PR program. Cognitive function (neuropsychological battery), health-status (36-Item Short Form Survey [SF-36]), and exercise capacity (6-min walk test [6MWT], cycle-endurance test [CET]) were assessed before and after PR. Responsiveness to PR was estimated by mean change (delta-value [Δ]) and the d-Effect Size (ES).\ud \ud Results:\ud Twenty-five COPD patients (42%) presented evidence of mild CI prior to PR. Both, CI and CN patients significantly improved global cognitive function, health status (the majority of SF-36 components), and exercise capacity (6MWT and cycle endurance) in response to PR. Compared to CN, CI patients did not improve SF-36 subdomains of “role emotional” and “bodily pain”, and demonstrated a lower magnitude of improvement in 6MWT ([Δ]: 25 m; ES: 0.21) compared to CN ([Δ]: 46 m; ES: 0.54).\ud \ud Conclusions:\ud PR has favorable effects on global cognitive function, health status, and exercise capacity in both CI and CN COPD patients. There was no concrete evidence to indicate interference of cognitive impairment to PR effectiveness.

RationaleIn patients with COPD, oxygen (O2)-supplementation via a constant flow oxygen system (CFOS) can result in insufficient oxygen saturation (SpO22-system (ATOS) has been shown to be beneficial compared with an untitrated CFOS, however, it is unknown if ATOS is superior to CFOS, titrated during exercise as stipulated by guidelines. The aim was to investigate the effects of ATOS compared with titrated CFOS on walking capacity in people with hypoxaemic COPD.MethodsFifty participants completed this prospective randomised controlled, double-blind, crossover trial. Participants performed two endurance shuttle walk tests (ESWTs) with: (1) exercise titrated CFOS (ESWTCFOS) and (2) ATOS targeting an SpO2of 92% (ESWTATOS). Primary outcome measure was walking time. Secondary measures were SpO2, transcutaneous-PCO2(TcPCO2), respiratory rate (RR), heart rate (HR) at isotime (end of shortest ESWT) with blood gases and dyspnoea at rest and end exercise.ResultsParticipants (median (IQR): age 66 (59, 70) years, FEV128.8 (24.8, 35.1) % predicted, PO254.7 (51.0, 57.7) mm Hg, PCO244.2 (38.2, 47.8) mm Hg) walked significantly longer with ESWTATOSin comparison to ESWTCFOS(median effect (95% CI) +144.5 (54 to 241.5) s, p2was significantly higher (+3 (95% CI 1 to 4) %, p2, RR and HR were comparable. End exercise, PO2(+8.85 (95% CI 6.35 to 11.9) mm Hg) and dyspnoea (−0.5 (95% CI −1.0 to −0.5) points) differed significantly in favour of ATOS (each p2was comparable.ConclusionIn patients with hypoxaemia with severe COPD the use of ATOS leads to significant, clinically relevant improvements in walking endurance time, SpO2, PO2and dyspnoea with no impact on PCO2.Trial registration numberNCT03803384.

Background: Frailty is prevalent in patients awaiting lung transplant (LTx) and is associated with delisting, death while on the waiting list and death after LTx. Decreasing frailty of LTx-candidates could therefore be important to improve pre- and post-LTx-status. Objective: Primary aim was to investigate the effects of a comprehensive PR-program on physical frailty in LTx-candidates. Methods: 32 participants from an inpatient PR-program with a confirmed diagnosis of COPD or interstitial lung disease who were listed or were in the process to be listed for a LTx were consecutively included in this trial. Primary outcome was the change in physical frailty measured by short physical performance battery test (SPPB; score 0-12) performed at PR-admission and discharge. SPPB results can be categorized into frail (SPPB≤7), pre-frail (SPPB=8-9) and not-frail (SPPB≥10). Participants with an SPPB score≤11 were considered for final analyses. Results: 28 participants (60±5years; FEV1 49±26%pred., SPPB-baseline: frail: n=5 [17.9%], pre-frail: n=10 [35.7%]; not-frail: n=13 [46.4%]) were analysed. Baseline SPPB-score (8.8±2.2) significantly (p Conclusion: Our findings show that a comprehensive PR-program can significantly and to a clinically meaningful extent, reduce frailty in LTx-candidates.

Background: Whole-body vibration training (WBVT) performed on a vibration platform can significantly improve physical performance in COPD patients. It has been suggested that an important mechanism of this improvement is based on an improvement in balance. Aim of this study was to investigate the effects of WBVT versus conventional balance training on exercise performance. Methods: 48 patients with severe COPD (FEV1: 37±7 %pred.) and low exercise performance (6-minute walk distance (6MWD): 55±10 %pred.) were included in a randomized controlled trial during a 3-week inpatient pulmonary rehabilitation. All patients completed a standardized endurance and strength training program. Additionally, patients performed 4 different balance exercises 3x/week for 2x1 minute each either on a vibration platform (Galileo) at varying frequencies (5 - 26 Hz) (WBVT) or on a conventional therapy tool inducing an instable surface (CON). Results: 6MWD improved significantly in both groups (WBVT: 48±46m, p Conclusion: WBVT can improve balance and muscular performance significantly more compared to conventional balance training.

Abstract Lymphangioleiomyomatosis (LAM) is a rare and progressive cystic lung disease with limited therapeutic options. We retrospectively analyzed the effects of a comprehensive 4-week inpatient pulmonary rehabilitation (PR) program in 58 patients with advanced LAM (FEV1: 45 ± 34%predicted, 6-min walk distance (6MWD): 338 ± 167 m). Exercise performance (6MWD: + 49 ± 50 m; p p = 0.049 and mental component: + 5.2 ± 12.1 points; p Trial registration Clinical-Trials registration number: NCT04184193; date of registration: December 3, 2019.

Patients with idiopathic pulmonary fibrosis (IPF) and concurrent hypoxaemia should be treated with oxygen therapy [1]. Oxygen therapy is commonly given by conventional nasal cannulas (CNC), but can also be delivered by other less conventional cannulas such as the Oxymizer. The Oxymizer is a nasal cannula with an internal pendant reservoir incorporated in the lumen at the patient end which has the potential to provide increased oxygenation while using the same oxygen setting as one would with CNC [2]., Something simple, like changing a nasal cannula for a technologically superior one, can improve exercise capacity and oxygenation in patients with IPF. There is a need to develop improved cannulas for comfort and patient acceptance. https://bit.ly/2NelacE

Insufficient cerebral blood flow regulation to meet increasing metabolic demand during physical exertion could be associated with cognitive impairment. We compared cerebral oxygen availability during exercise in cognitively impaired (CI) to cognitively normal (CN) COPD patients. Fifty-two patients (FEV1: 51 ± 16%) were classified as CN or CI according to the Montreal Cognitive Assessment. Patients performed cycle-ergometry at 75% peak capacity with continuous measurement of Near-Infrared Spectroscopy frontal-cortex Tissue oxygen Saturation Index (TSI), cerebral haemoglobin indices (oxy/deoxy/total- Hb), transcutaneous carbon-dioxide partial pressure (TcPCO2), and arterial oxygen saturation (SpO2). Twenty-one patients (40%) presented evidences of CI. During exercise, CN and CI patients exhibited mild to moderate SpO2decline (nadir[Δ]≥ −3 ± 2% and −5 ± 3%, respectively) but preserved baseline frontal-cortex TSI levels, whilst presenting small TcPCO2 perturbations and increased cerebral total-Hb (post [Δ]≥ 2.0 ± 3 μM sec−1). CI patients preserve the capacity to adequately maintain cerebral oxygen availability during submaximal exercise. Therefore, rehabilitative exercise training in CI patients with COPD exhibiting mild to moderate exercise-induced SpO2 decline does not appear to lead to reduced cerebral oxygen availability.

Pneumologische Rehabilitation ist eine evidenzbasierte, multidisziplinare Behandlung fur Patienten mit chronischen Erkrankungen der Atmungsorgane. Die Beantragung und Durchfuhrung von Rehabilitation ist an klare Vorgaben geknupft. Dieser Artikel gibt praktische Tipps in der Beantragung der Rehabilitation, im Umgang mit Ablehnung und in der Formulierung von begrundeten Widerspruchen. Auch der Lungensport, die Beantragung und das Suchen von Lungensportgruppen werden adressiert.

ZusammenfassungDas vorliegende Dokument ist eine Neufassung und Aktualisierung der Leitlinie zur Diagnostik und Therapie von Patienten mit COPD, die die bisherige Version aus dem Jahr 2007 ablöst. Die Fülle an neuen Erkenntnissen zu Risikofaktoren, Diagnostik, Schweregradeinschätzung, Prävention und medikamentösen sowie nicht medikamentösen Therapiemaßnahmen machten eine umfassende Überarbeitung erforderlich. Die neue Leitlinie baut auf das GOLD-Dokument unter Berücksichtigung von Besonderheiten in Deutschland und Österreich auf.

ZusammenfassungEine pneumologische Rehabilitationsmaßnahme kann bei COPD-Patienten, insbesondere nach Exazerbationen, die Zahl der Rehospitalisierungen reduzieren und sogar die Mortalität verringern. Diese Effekte stehen jedoch in einem direkten Zusammenhang mit der Qualität der Rehabilitation, was diese komplex macht. Neben der Erfassung des medizinischen Status, der Leistungsfähigkeit sowie relevanter Komorbiditäten ist eine spezifische Diagnostik und zielgerichtete Therapie notwendig. Das schließt neben der medikamentösen Therapie, inklusive Training der Inhalationstechniken, unter Umständen auch endoskopische Therapien (z. B. Bronchialtoilette) ein. Auch die nicht-invasive Beatmung erhält eine wachsende Bedeutung im rehabilitativen Setting. Dies setzt neben der notwendigen fachlichen Expertise die Erfahrung und den Mut voraus, diese Patienten auch konkret in die Reha-Trainingsprogramme einzuschließen, die mit NIV durchgeführt werden können – wie Fahrradergometer-Therapien oder Laufbandtraining – mit dem Ziel, die Patienten einem höheren Trainingsstimulus zuzuführen, welcher die weitere Prognose des COPD-Patienten beeinflusst. Ein wichtiger Punkt in der Arzt-Patienten-Interaktion ist die Festlegung realistischer Patientenziele, um letztendlich zu einer dauerhaft aktiveren gesundheitsorientierteren Lebensweise zu gelangen.

Background: Emphysematous destruction of lung parenchyma visible in computed tomography (CT) can be attributed to chronic obstructive pulmonary disease (COPD) or to α1-antitrypsin deficiency (AATD). Objectives: We evaluated if visual semiquantitative phenotyping of CT data helps identifying individuals with AATD in a group of smokers with severe emphysema and airflow limitation. Method: n = 14 patients with AATD and n = 15 with COPD and a minimum of 10 pack years underwent CT, clinical assessment, and full-body plethysmography. The extent and type of emphysema as well as large and small airway changes were rated semiquantitatively for each lobe using a standardized previously published scoring system. Lastly, a final diagnosis for each patient was proposed. Results: AATD had a significantly lower mean emphysema score than COPD, with 8.9 ± 3.4 versus 11.9 ± 3.2 (p < 0.001), respectively. Within both groups, there was significantly more emphysema in the lower lobes (p < 0.05–0.001). The COPD group showed an upper- and middle-lobe predominance of emphysema distribution when compared to the AATD group (p < 0.001). Centrilobular (CLE) and panlobular (PLE) emphysema patterns showed a uniform distribution within both groups, with a CLE predominance in the upper lung and a PLE predominance in the lower lung regions. AATD and COPD both showed significantly more airway changes in lower lobes compared to upper lobes (p = 0.05–0.001), without significant differences between both groups. Conclusion: The typical emphysema distribution patterns seen on CT traditionally assigned to AATD and COPD were of little use in discriminating both entities. Also, airway changes could not contribute to a more precise differentiation. We conclude that a concise standardized phenotyping-driven approach to chest CT in emphysema is not sufficient to identify patients with AATD in a cohort of smokers with advanced emphysema.

The European Respiratory Society (ERS) and American Thoracic Society (ATS) guideline on management of chronic obstructive pulmonary disease (COPD) exacerbations was published in the March 2017 issue of the European Respiratory Journal [1]. Based on evidence syntheses, including meta-analyses, relevant evidence up to September 2015 was summarised and clinical recommendations for treatment of COPD exacerbations were formulated. These guidelines were endorsed by the ERS Executive Committee and approved by the ATS Board of Directors in December 2016.

ZusammenfassungDie vorliegende Leitlinie ist eine Neufassung und Aktualisierung der Leitlinie zur Diagnostik und Therapie von Patienten mit Asthma, welche die bisher für den deutschen Sprachraum gültige Version aus dem Jahr 2006 ablöst. Die Fülle an neuen Erkenntnissen zur Pathophysiologie und zu den Phänotypen von Asthma und das erweiterte Spektrum an diagnostischen und therapeutischen Möglichkeiten bei dieser Erkrankung machte eine Neufassung und Aktualisierung notwendig. Es werden sowohl für Kinder und Jugendliche als auch für Erwachsene mit Asthma die aktuellen, Evidenz-basierten diagnostischen und therapeutischen Empfehlungen dargelegt.

ZusammenfassungDie Lebensqualität von COPD-Patienten ist häufig deutlich beeinträchtigt. Neben den körperlichen Limitationen der Betroffenen treten häufig psychische Komorbiditäten auf. Damit gehen häufig auch dysfunktionale Annahmen der Betroffenen in Bezug auf ihre Erkrankung und deren Behandlung einher. Diese können nicht nur die Lebensqualität der Betroffenen weiter beeinträchtigen, sondern auch Auswirkungen auf die Kommunikation zwischen Arzt und Patient sowie das Krankheitsverhalten haben. Aus diesem Grund ist es wichtig, dass Behandler sie im Dialog mit dem Patienten berücksichtigen. Der folgende Beitrag thematisiert, welches Gewicht dysfunktionalen Krankheitsannahmen und psychischen Komorbiditäten bei COPD zukommt, und geht auf die Möglichkeiten ein, diese im Arzt-Patient-Kontakt positiv zu berücksichtigen.

Background The acute effect of supplemental oxygen during exercise has been shown to differ largely among patients with COPD. It is unknown what factors influence oxygen response. Methods In a randomized, single-blind fashion, 124 patients with COPD underwent one 6-min walk test on supplemental oxygen (6MWTO2) and one 6-min walk test on room air after a practice 6-min walk test. Both gases were delivered via standard nasal prongs (2 L/min). For analyses, patients were stratified on the basis of PaO2 values and compared: (1) 34 patients with resting hypoxemia (HYX); (2) 43 patients with exercise-induced hypoxemia (EIH); and (3) 31 patients with normoxemia (NOX). Results Oxygen supplementation resulted in an increase in the 6-min walk distance in the total cohort (27 ± 42 meters; P Conclusions In contrast to patients with NOX, patients with HYX and EIH generally benefit from supplemental oxygen by increasing exercise capacity. However, less than one-half of patients reached the threshold of clinically relevant improvements. These oxygen responders were characterized by significantly lower exercise capacity levels. Trial Registry ClinicalTrials.gov; No.: NCT00886639; URL: www.clinicaltrials.gov.

Background: Numerous studies have reported positive effects of exercise training in patients with interstitial lung disease (ILD) on physical capacity and quality of life. However, evidence is rare on the effects of specific forms of training and further pathophysiological mechanisms in these patients. Objectives: In this multicenter study we aimed to explore the clinical effects of whole-body vibration training (WBVT) in patients with ILD on various outcome measures, including proinflammatory cytokines and myostatin. Methods: We randomly assigned 26 patients with different forms of multidisciplinary confirmed fibrotic ILDs either to the WBVT group (n = 11; 55% male, 61 ± 14 years old, forced vital capacity 83.2 ± 29.3% predicted, 6-min walking distance [6MWD] 478 ± 79 m) performing 3 months of a standardized training (3 times per week), or to a control training group (CTG, n = 15; 60% male, 63 ± 9 years old, FVC 74.6 ± 20.5% predicted, 6MWD 455 ± 85 m) performing sham WBV training. Training in the two groups was performed on a GalileoTM vibration plate (6–20 vs. 5 Hz). The functional assessments before and after the intervention period included pulmonary function, 6MWD test, chair rise test, ultrasonographic measurement of quadriceps muscle thickness (cross-sectional area), quality of life questionnaires, and serum samples. Results: We observed a significant increase in 6MWD (∆Training = 30 m [12–67], p = 0.024) and a decrease of myostatin (∆Training = –465 pg/mL [–713 to –166], p = 0.008) in the WBVT group. In contrast, no significant differences were observed in the CTG. Conclusions: The present study demonstrates that WBVT is able to significantly increase 6MWD and decrease myostatin in patients with fibrotic ILDs. Therefore, WBVT seems to be a beneficial and feasible training modality in ILD patients. Clinical Trial Registry: German Clinical Trials Registry (DRKS00012930).