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Lung transplantation for interstitial lung diseases

transplantationPurpose of review : An overview over the role of lung transplantation in interstitial lung diseases will be given.

Recent findings : Lung transplantation is an established therapy option for patients with various end-stage lung diseases. Currently, the worldwide procedural frequency is about 3600 per year. Unfortunately, the shortage of donor organs leads to approximately every sixth patient in western countries dying before a donor organ is available. The most frequent underlying clinical indications are emphysema, pulmonary fibrosis, and cystic fibrosis. In a recent registry report worldwide, 23% of all lung transplant recipients had a diagnosis of idiopathic pulmonary fibrosis. In experienced centers, candidates for transplantation are chosen according to disease-specific factors after excluding contra-indications. However, there are several challenges for lung transplantation. The number of lung transplantations performed is limited by the supply of donor organs, and the long-term survival rates are still inferior compared with other forms of solid organ transplantation.

Summary : Lung transplantation offers a survival benefit in carefully selected patients with interstitial lung diseases.

Advanced pulmonary sarcoidosis

sarcoidosisTo present an update on the most recent contributions in advanced pulmonary sarcoidosis (APS).

Recent findings : Pathology is better described and the differences between fibrosing pulmonary sarcoidosis and usual interstitial pneumonia (UIP) are clarified. Serial spirometry is the most reliable tool for monitoring evolution. Survival may be predicted by an integrative algorithm based on pulmonary function and computed tomography (CT).

Summary : APS is characterized by significant fibrocystic pulmonary lesions at CT and pathology. There are two main patterns of APS, one with predominant central bronchovascular distortion, often associated with airflow limitation, and the other with predominant honeycombing with a different location than in UIP with severe restrictive impairment and very low diffusion capacity of the lung for carbon monoxide. APS may be burnt out but is most often still active as evidenced by several findings, including on 18F-fluorodeoxyglucose-PET. There is an increased mortality and morbidity with chronic respiratory insufficiency, pulmonary hypertension stemming from multiple mechanisms, chronic pulmonary aspergillosis and extra infections. Acute worsening episodes are frequent. Serial spirometry, particularly forced vital capacity, is the most reliable tool for monitoring evolution. A new elegant algorithm based on pulmonary function and CT may predict survival.

Despite important stakes, there is still a lack of therapeutic recommendations. However, the use of antisarcoidosis treatment is most often required at least as a temporary trial. Finally, the effect of pulmonary hypertension treatment has recently been the object of further evaluation.

Testing Spirometers: Are the Standard Curves of the American Thoracic Society Sufficient?

Related Articles

Testing Spirometers: Are the Standard Curves of the American Thoracic Society Sufficient?

Respir Care. 2014 Sep 2;

Authors: Lefebvre Q, Vandergoten T, Derom E, Marchandise E, Liistro G

Abstract
BACKGROUND: The performance of spirometers is often measured only under ideal conditions, with a mechanical simulator reproducing the expiratory standard American Thoracic Society (ATS) curves generated by a computer. Studies have questioned the value of these results in real-life conditions. The aim of this study was to evaluate the accuracy and precision of 5 office spirometers with a flow-volume simulator using the ATS curves and using flow-volume curves obtained from patients.
METHODS: We measured the FVC, peak expiratory flow, and FEV1 by simulating different dynamic waveforms applied by a computer-driven syringe, the Hans Rudolph flow-volume simulator. In addition to testing standard curves recommended by the ATS, we also tested curves obtained with subjects.
RESULTS: The precision of the office spirometers was good and comparable using the standard ATS curves. One device presented the best performances in terms of accuracy and precision according to the ATS recommendations, but we observed significant biases in all devices with Bland-Altman analysis, particularly with the curves obtained from subjects with severe COPD.
CONCLUSIONS: The global quality of most spirometers makes them acceptable for the detection of pulmonary diseases. However, we demonstrated accuracy issues not shown by the standard testing procedure. We propose to improve the testing of spirometers by implementing more realistic flow-volume curves and to refine the analysis of the results.

PMID: 25185146 [PubMed - as supplied by publisher]

Physical Activity and Quality of Life Improvements of Patients with Idiopathic Pulmonary Fibrosis Completing a Pulmonary Rehabilitation Program.

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Physical Activity and Quality of Life Improvements of Patients with Idiopathic Pulmonary Fibrosis Completing a Pulmonary Rehabilitation Program.

Respir Care. 2014 Sep 2;

Authors: Gaunaurd IA, Gómez-Marín OW, Ramos CF, Sol CM, Cohen MI, Cahalin LP, Cardenas DD, Jackson RM

Abstract
INTRODUCTION: Pulmonary rehabilitation is effective for patients with COPD, but its benefit is less clearly established in idiopathic pulmonary fibrosis (IPF), especially in regard to levels of physical activity and health-related quality of life. The objectives were to determine whether pulmonary rehabilitation increased physical activity as assessed by the International Physical Activity Questionnaire (IPAQ), and improved quality of life and symptoms as assessed by the St George respiratory questionnaire for IPF (SGRQ-I) and the Borg dyspnea index (BDI).
METHODS: Subjects who met current criteria for IPF were randomized to a 3-month pulmonary rehabilitation program (n = 11) or to a control group (n = 10). The rehabilitation group participated in twice-weekly, 90-min exercise sessions (24 total sessions). The control group maintained its preceding, normal physical activity. All subjects underwent 6-min walk tests to assess the postexertion BDI. The SGRQ-I and a 5-point self-assessment of health were completed at baseline, after 3 months of intervention or observation, and after 3-month follow-up. All subjects completed the IPAQ weekly.
RESULTS: Subjects in the rehabilitation group maintained significantly higher levels of physical activity throughout the 3-month rehabilitation program (rehabilitation: 51,364 ± 57,713 [mean ± SD] metabolic equivalent of task-minutes; control: 20,832 ± 37,155, P = .027 by 2-tailed Mann-Whitney test). SGRQ-I symptom domain scores improved considerably by -9 ± 22 in the rehabilitation group, whereas in the control group they worsened (16 ± 12 rehabilitation compared with control, P = .013 by 2-tailed Mann-Whitney test). During the 3-month follow-up, self-reported physical activity levels in the rehabilitation group were 14,428 ± 8,884 metabolic equivalent of task-minutes and in the control group 16,923 ± 32,620 (P = .173 by 2-tailed Mann-Whitney test), demonstrating substantial reversal of activity in the rehabilitation group. BDI scores after 6-min walk tests did not change significantly.
CONCLUSIONS: A 3-month rehabilitation program significantly improved symptoms (SGRQ-I) and physical activity levels (IPAQ) in subjects with IPF while they participated actively in the program.

PMID: 25185149 [PubMed - as supplied by publisher]

Relationship Between Medication Adherence and Health-Related Quality of Life in Subjects With COPD: A Systematic Review.

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Relationship Between Medication Adherence and Health-Related Quality of Life in Subjects With COPD: A Systematic Review.

Respir Care. 2014 Sep 2;

Authors: Agh T, Dömötör P, Bártfai Z, Inotai A, Fujsz E, Mészáros A

Abstract
BACKGROUND: Various aspects of medication adherence and health-related quality of life (HRQOL) have been studied in subjects with COPD. Nevertheless, little is known about the association between these factors. The aim of this study was to undertake a systematic review of the published literature focusing on the relationship between medication adherence and HRQOL in COPD.
METHODS: A systematic literature search of English language articles was conducted in April 2013 using MEDLINE. No publication date limits were defined. All of the included studies were assessed for quality.
RESULTS: Seven studies were included in the review. Three of the assessed studies found no correlation, and 3 studies described positive and 2 studies reported negative associations between medication adherence and HRQOL. The results indicate that an improved HRQOL may be a trigger for non-adherence in patients with COPD.
CONCLUSIONS: The relationship between medication adherence and HRQOL may be dual. The effect of medication adherence on HRQOL might be a consequence of the effectiveness of therapy and the negative effects (ie, side effects, daily life limitation of therapy, social stigma) that it can generate. HRQOL might also influence the patterns of patients' drug use, as an increased HRQOL might trigger non-adherence. The dynamics between adherence and HRQOL might differ over time, as the negative effects of medication non-adherence might become dominant in the long term.

PMID: 25185152 [PubMed - as supplied by publisher]

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