Pulmonary Rehabilitation in Patients with Pulmonary Sarcoidosis: Impact on Exercise Capacity and Fatigue.

BACKGROUND: There is limited evidence regarding the impact of multidisciplinary pulmonary rehabilitation (PR) on exercise capacity and fatigue in patients with pulmonary sarcoidosis. The aim of this study was to evaluate the impact on exercise capacity and fatigue following PR, and to examine whether baseline fatigue was related to change in peak oxygen uptake (ΔV̇O2peak). METHODS: Forty-one patients with pulmonary sarcoidosis attending a 4-week inpatient PR program were recruited to this pre-post study. Both maximal exercise capacity, defined as V̇O2peak and measured with a cardiopulmonary exercise test, and fatigue, assessed with the Fatigue Assessment Scale (score 10-50 points), were measured before and after PR. RESULTS: There was a statistically significant improvement in V̇O2peak (1.2 ± 2.3 mL/kg/min, p = 0.002), and fatigue decreased significantly (-1.7 ± 3.9 points, p = 0.009) following PR. Unadjusted linear regression analyses demonstrated that age (B = -0.076, p = 0.017) and baseline fatigue (B = 0.196, p = 0.001) were predictors for change in V̇O2peak, while in adjusted analyses (age, sex, baseline V̇O2peak, baseline fatigue, and diffusion capacity of the lung for carbon monoxide), only baseline fatigue predicted change in V̇O2peak following PR (B = 0.165, p = 0.026). CONCLUSION: A 4-week multidisciplinary PR program improves maximal exercise capacity and reduces fatigue in patients with pulmonary sarcoidosis. Baseline fatigue only partly predicted change in V̇O2peak following PR.

Short-Term Effects of a Multimodal 3-Week Inpatient Pulmonary Rehabilitation Programme for Patients with Sarcoidosis: The ProKaSaRe Study.

BACKGROUND: Real-world data on the effects of a multicomponent pulmonary rehabilitation (PR) for patients with sarcoidosis are scarce. OBJECTIVE: To describe characteristics of patients with sarcoidosis referred for a 3-week inpatient PR, to assess the effects of PR on their quality of life (QoL) and clinical outcomes, and to investigate whether there are specific subgroups who particularly benefit from PR. METHODS: Using a prospective multicentre study design, data regarding 6-min walking distance (6MWD), QoL (Saint George's Respiratory Questionnaire, SGRQ), and the secondary outcomes of dyspnoea and psychological burden (fatigue, anxiety, and depression) were collected. RESULTS: We included 296 patients in the study (average age 49.1 ± 9.7 years, 47% female, average vital capacity 3.5 ± 1.0 L [87.0 ± 20.6 predicted]). The 6MWD improved by the end of the rehabilitation by 39.8 m on average (p < 0.0001; standardised response mean, SRM = 0.61), SGRQ showed significant improvements in all 3 domains, and the total score (p < 0.001) improved by 5.69-8.28 points (SRM 0.46-0.62). For the secondary outcomes, significant improvement (p < 0.001) was seen for all measured parameters, e.g., dyspnoea (modified Medical Research Council Scale, mMRC), fatigue (Fatigue Assessment Scale [FAS]; SRM = -0.71), anxiety and depression (Hospital Anxiety and Depression Scale [HADS]; SRM -0.58/-0.38), and generic QoL (measured by the SF-36 scales of physical and mental health; SRM 0.31/0.55). CONCLUSIONS: Our results provide the first documented evidence that PR is a promising complementary therapy option for sarcoidosis patients who remain subjectively symptomatic despite optimised outpatient medical treatment.

Principles of rehabilitation and reactivation: interstitial lung disease, sarcoidosis and rheumatoid disease with respiratory involvement.

The interstitial lung diseases (ILDs) are characterised by dyspnoea on exertion, exercise-induced hypoxaemia, reduced skeletal muscle function and exercise intolerance. Evidence from nine randomised controlled trials shows that pulmonary rehabilitation improves exercise capacity, dyspnoea and quality of life in ILD, with moderately large effect sizes from 0.59 to 0.68. Participants with idiopathic pulmonary fibrosis, the most common and most progressive of the ILDs, achieve benefits in exercise capacity and quality of life that are of equal magnitude to those seen in other ILDs, with effect sizes from 0.59 to 0.75. Whole body exercise training is a core component of pulmonary rehabilitation for ILD. The standard exercise prescription used for other chronic lung diseases is effective in ILD, including 8 weeks of training with at least two supervised sessions per week and at least 30 min of aerobic training per session. However, the unique presentation and underlying pathophysiology of ILD may require modifications of the exercise prescription for individual patients. Those with connective tissue disease may present with joint pain and stiffness that require modification of the standard exercise prescription, including reduction in weight-bearing exercise. Some patients with severe disease may present with distressing dyspnoea that limits the intensity or progression of training. Because exercise-induced hypoxaemia is common in ILD and more severe than seen in other chronic lung diseases, pulmonary rehabilitation should be provided in a setting where supplemental oxygen therapy is available. Pulmonary rehabilitation programs offer the opportunity to address other critical aspects of ILD care, including management of comorbidities, symptoms and mood.

Long-term effects of pulmonary rehabilitation on daily life physical activity of patients with stage IV sarcoidosis: A randomized controlled trial.

INTRODUCTION: Pulmonary rehabilitation (PR) is known to improve exercise tolerance, mood, and quality of life in patients with chronic respiratory diseases. The aim of this work was to determine whether PR provides long-term benefits in increasing daily life physical activity in patients with chronic sarcoidosis. METHODS: This randomized prospective study (registered ClinicalTrials.gov NCT02044939) of 38 patients with stage IV chronic sarcoidosis was performed between 2012 and 2016. Patients were assigned to participate in a 2-month PR program (n=20) or receive counseling (n=18). Assessments were performed at baseline, 2 months (end of the PR program), 6months, and 12months, and included daily life physical activity parameters (measured for 5 consecutive days), exercise tolerance, dyspnea, anxiety, depression, fatigue, and quality of life. The primary outcome was the 12-month change in time spent in activities above an estimated energy expenditure of 2.5metabolic equivalents (METs). Secondary daily life physical activity outcomes included number of steps per day, total daily energy expenditure, and total energy expenditure above 2.5METs. RESULTS: The primary outcome did not differ between the two groups; mean between-group differences were -13.2min (95% confidence interval [CI]: -76.3 to 49.8) at 6 months and -18.1min (95% CI: -55.7 to 19.4) at 12months. Although PR had no effect on secondary daily life physical activity outcomes, it did significantly increase exercise tolerance at 6 and 12 months and decrease the dyspnea score at 6 months and the fatigue score at 12months. CONCLUSION: This trial failed to demonstrate a beneficial effect of PR on daily life physical activity in sarcoidosis patients, suggesting that long-term behavioral programs may be necessary to complement PR.

[Exercise testing by spiroergometry in a patient with sarcoidosis stage II. An unusual combination of functional impairments]. / Spiroergometrie bei einem Patienten mit Sarkoidose II. Eine aussergewöhnliche Kombination von Funktionsstörungen.

Sarcoidosis is a multisystem disease of unknown origin characterised by epitheloid cell granulomas in the lung and many other organs. The measurement of the alveolo-arterial O(2)-gradient (AaDO(2)) during exercise is the most sensitive method to detect the involvement of the lung with impairment of gas exchange. The method is suitable for follow-up observations to make the indication for a steroid therapy in the case of deterioration. In this case presentation using the 9-panel display proposed by Wasserman we diagnosed besides a slight impairment of the gas exchange a cardiocirculatory dysfunction with a reduction of the oxygen pulse and a reduced minute ventilation with a decrease of the alveolar ventilation. The hypoventilation could be explained by a weakness of the respiratory muscles or a reduction of the central respiratory drive. The functional disturbances could be diagnosed by spiroergometry without expensive or invasive methods. The findings should be examined more specifically with regard to therapeutic consequences.

Childhood sarcoidosis: long-term follow-up.

The aim of the present study was to describe clinical features and long-term survival in childhood sarcoidosis. In total, 46 ethnic Caucasian Danish children (aged <16 yrs, 24 males) with sarcoidosis were identified in 1979-1994. In 33 (72%) children, diagnosis was verified by histology and, in the remaining 13, by clinical and radiological findings. In total, 37 subjects had a follow-up examination. Median (range) age at onset of disease was 14 (0.7-15.8) yrs and median (range) clinical follow-up was 15 (3-23) yrs after onset of disease. The median (range) age at clinical follow-up was 28 (17-30) yrs. At follow-up: 36 (78%) children recovered completely; 30 (65%) showed complete clinical regression at a median (range) 0.7 (0.6-5.9) yrs after onset of disease; two (4%) recovered with organ damage (unilateral loss of vision, abnormal chest radiograph); five (11%) still had chronic active disease with multiorgan involvement and impaired lung function; and three (7%) were deceased, due to central nervous system sarcoidosis and acute myeloid leukaemia probably caused by cytostatics. In Danish children, sarcoidosis had a favourable prognosis; the majority recovered <6 yrs after onset of disease. Some developed chronic active disease and impairment of pulmonary function, demanding continuous medical treatment. Prognosis was not related to the age at onset of disease. Erythema nodosum was associated with a good prognosis, and central nervous system involvement with a poor prognosis.

Benefits of physical training in patients with idiopathic or end-stage sarcoidosis-related pulmonary fibrosis: a pilot study.

BACKGROUND: The natural history of disease in patients with stage IV (fibrotic) sarcoidosis may mirror that of patients with idiopathic pulmonary fibrosis (IPF). Both are bothered by progressive dyspnea, exercise limitation and fatigue. OBJECTIVE: To establish whether patients suffering from pulmonary fibrosis might benefit from a physical training program. STUDY DESIGN: Twenty-four eligible patients referred to the out-patient clinic of the ild care expertise team of Hospital Gelderse Vallei, Ede, The Netherlands between November 2012 and November 2013 were included in this observational pilot study of a 12-week physical training program. Outcomes, including exercise capacity, skeletal muscle strength, lung function and fatigue were assessed at two time points: 1) baseline; and 2) after completion of a 12-week physical training program. RESULTS: At baseline, the percentage predicted DLCO, FVC, FEV1 and exercise capacity (assessed by six-minute walking distance (6MWD) or maximal oxygen uptake) was reduced in both groups. After program completion, exercise capacity improved (>10% improvement 6MWD) in 13 subjects (54.2%): 7 with IPF and 6 with sarcoidosis subjects. Other secondary endpoints, including pulmonary function tests and patient-reported outcome measures improved in some subjects. CONCLUSION: A 12-week physical training program improved or maintained exercise capacity in patients with IPF (despite disease progression) or fibrotic sarcoidosis. The results from this pilot study could be used to design prospective studies aimed at answering lingering questions about exercise training in patients with these progressive, incurable conditions.

Does physical training reduce fatigue in sarcoidosis?

BACKGROUND: Sarcoidosis patients frequently experience fatigue, exercise intolerance and muscle weakness, resulting in reduced quality of life (QOL). Scientific studies on the benefits of physical training in sarcoidosis have been scarce, so the aim of this pilot study was to examine the impact of a 13-week physical training program on fatigue, physical functions and QOL in fatigued sarcoidosis patients and/or patients with exercise intolerance. METHODS: 18 sarcoidosis patients participated in a 13-week physical training program. The Fatigue Assessment Scale (FAS), World Health Organization Quality of Life-BREF assessment instrument (WHOQOL-BREF),Medical Research Council (MRC) dyspnea scale, Visual Analogue Scale (VAS), six-minute walk test (6MWT), submaximal bicycle test and muscle strength of the quadriceps and elbow flexors were assessed at baseline and after the program. RESULTS: FAS scores had decreased (mean difference -2.7 points, 95% CI -4.4 to -1.1) after completion of the training program, along with improvements in WHOQOLBREF psychological health domain (mean difference 0.9 points, 95% CI 0.2 to 1.7) and MRC dyspnea score (mean difference -0.4 points, 95% CI -0.8 to -0.1). 6MWD improved by 34.6 m (95% CI 20.3 to 49.0) and mean heart rate on the bicycle test improved (mean difference 8.4 beats/minute, 95% CI -13.8 to -3.0), as did quadriceps strength (mean difference 10.7 kg, 95% CI 5.5 to 15.9). CONCLUSION: Fatigue reduced after a period of physical training in sarcoidosis patients. Moreover, psychological health and physical functions improved. Future studies are warranted to assess the benefits of physical training in sarcoidosis.

[Respiratory rehabilitation in patients with pulmonary sarcoidosis].

INTRODUCTION: In clinical practice, physical therapy is more often applied in patients with obstructive pulmonary disease than in other pulmonary diseases. One of them is pulmonary sarcoidosis. Depending on the stage, there are a few different types of pulmonary dysfunctions. With early disease progression pulmonary obstruction is expected and later interstitial fibrosis may lead to restrictive syndrome and development of bronchiectasias. RESPIRATORY REHABILITATION: Respiratory rehabilitation may improve pulmonary ventilation and the choice depends on lesion features. The objectives of respiratory rehabilitation are. to establish the optimal respiratory rate, sustain opened airways, achieve automatic diaphragm breathing and improve ventilation. For these purposes different techniques are used: aerosol therapy, breathing exercises, forced expiration, postural drainage, kinesitherapy. Using more than one of these techniques in the same patient we can prevent or delay consequences of respiratory failure and early disability. Early respiratory rehabilitation in these patients is an opportunity to reduce disability and improve quality of life.

[Follow-up of sarcoidosis outpatients]. / Dispansernoe nabliudenie bol'nykh sarkoidozom.

The results of long-term follow-ups of 300 patients with sarcoidosis registered at the Central Research Institute of Tuberculosis, Russian Academy of Medical Sciences, were studied. The paper provides a full characterization of the patients by their social status, clinical forms, detection modes, diagnostic methods. The efficiency of treatment was evaluated, as evidenced by the "movement" from group to group (from active group to inactive one). A follow-up makes it possible to correct treatment.

[X-ray criteria for assessing the activity of lung sarcoidosis at follow-up]. / Rentgenologicheskie kritetii otsenki aktivnosti sarkoidoza legkikh pri dispansernom nabliudenii.

The results of comprehensive X-ray findings in 300 patients with different clinical and X-ray forms of respiratory sarcoidosis who had been registered to be followed up at the Consulting Outpatient Department, Central Research Institute of Tuberculosis Russian Academy of Medical Sciences, were analyzed. The follow-up lasted 3 to 20 years. X-ray study heads the list of the most informative techniques for assessing the activity of sarcoidosis. The time of following up patients with sarcoidosis in dispensary registration groups is recommended in accordance with the recommendations of the sarcoidosis Consensus Commission. X-ray criteria for stabilization of the disease and the optimum methodological complex of radiation diagnosis are defined.