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Pirfenidone reduces functional decline in patients with Idiopathic Pulmonary Fibrosis (IPF). However, response to treatment is highly heterogeneous. We sought to evaluate whether response to pirfenidone is influenced by the pretreatment rate of forced vital capacity (FVC) decline. Fifty-six IPF patients were categorized as rapid (RP) or slow progressors (SP) based on whether their FVC decline in the year preceding pirfenidone treatment was > or ≤ 10% predicted. Following pirfenidone treatment patients were followed-up every 6 months and up to 24 months. In the entire population, pirfenidone reduced significantly FVC decline from 231 to 49 ml/year at 6 months (T6) (p = 0.003) and this effect was maintained at the 12-, 18- and 24-month time points (p value for trend n.s.). In RP, the reduction of FVC decline was evident at 6 months (36 vs 706 ml/year pretreatment; p = 0.002) and maintained, though to a lesser degree, at 12 (106 ml/year), 18 (176 ml/year) and 24 months (162 ml/year; p value for trend n.s). Among SP, the reduction in FVC decline was not significant at any of the time points analyzed. In conclusion, pirfenidone reduces FVC decline in IPF patients. However, its beneficial effect is more pronounced in patients with rapidly progressive disease.

Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease characterized by progressive loss of lung function and poor prognosis. The so-called acute exacerbation of IPF (AE-IPF) may lead to severe hypoxemia requiring mechanical ventilation in the intensive care unit (ICU). AE-IPF shares several pathophysiological features with acute respiratory distress syndrome (ARDS), a very severe condition commonly treated in this setting. A review of the literature has been conducted to underline similarities and differences in the management of patients with AE-IPF and ARDS. During AE-IPF, diffuse alveolar damage and massive loss of aeration occurs, similar to what is observed in patients with ARDS. Differently from ARDS, no studies have yet concluded on the optimal ventilatory strategy and management in AE-IPF patients admitted to the ICU. Notwithstanding, a protective ventilation strategy with low tidal volume and low driving pressure could be recommended similarly to ARDS. The beneficial effect of high levels of positive end-expiratory pressure and prone positioning has still to be elucidated in AE-IPF patients, as well as the precise role of other types of respiratory assistance (e.g., extracorporeal membrane oxygenation) or innovative therapies (e.g., polymyxin-B direct hemoperfusion). The use of systemic drugs such as steroids or immunosuppressive agents in AE-IPF is controversial and potentially associated with an increased risk of serious adverse reactions. Common pathophysiological abnormalities and similar clinical needs suggest translating to AE-IPF the lessons learned from the management of ARDS patients. Studies focused on specific therapeutic strategies during AE-IPF are warranted.

Pulmonary rehabilitation in cystic fibrosis (CF) utilizes the same components as in other respiratory conditions. However, there are some specifics that should be beard in mind. For one, CF is a multi-organ disease with quite variable clinical characteristics and disease burden among individuals. Thus, pulmonary rehabilitation cannot focus solely on the lungs but has to take upper airways, nutritional aspects, the cardiovascular system, liver disease, and the musculoskeletal system into account. Furthermore, people with CF may host various pathogens, which may be (directly or indirectly) transmitted from one person to the other. Infection control measures are, thus, of utmost importance and often interfere with traditional rehabilitation settings such as group sessions. The chapter will focus on aspects of pulmonary rehabilitation which are particularly important in CF. For general information on components of pulmonary rehabilitation, we refer to the respective chapters at the beginning of this book.

Background: Recent evidences show that Pulmonary Rehabilitation (PR) is effective in patients with Interstitial Lung Disease (ILD). It is still unclear whether disease severity and/or etiology might impact on the reported benefits. We designed this prospective study 1) to confirm the efficacy of rehabilitation in a population of patients with ILDs and 2) to investigate whether baseline exercise capacity, disease severity or ILD etiology might affect outcomes. Methods: Forty-one patients (IPF 63%, age 66.9 ± 11 ys) were enrolled in a standard PR course in two centers. Lung function, incremental and endurance cyclo-ergometry, Six Minutes Walking Distance (6MWD), chronic dyspnea (Medical Research Council scale-MRC) and quality of life (St. George Respiratory Questionnaire-SGRQ) were recorded before and at the end of PR to measure any pre-to-post change. Correlation coefficients between the baseline level of Diffuse Lung Capacity for Carbon monoxide (DLCO), Forced Vital Capacity (FVC), 6MWD, power developed during incremental endurance test, GAP index (in IPF patients only) and etiology (IPF or non-IPF) with the functional improvement at the 6MWDT (meters), at the incremental and endurance cyclo-ergometry (endurance time) and the HRQoL were assessed. Results: Out of the 41 patients, 97% (n = 40) completed the PR course. Exercise performance (both at peak load and submaximal effort), symptoms (iso-time dyspnea and leg fatigue), SGRQ and MRC significantly improved after PR (p < .001). Patients with lower baseline 6MWD showed greater improvement in 6MWD (Spearman r score = -.359, p = .034) and symptoms relief at SGRQ (r = -.315, p = .025) regardless of underlying disease. Conclusion: Present study confirms that comprehensive rehabilitation is feasible and effective in patients with ILD of different severity and etiology. The baseline submaximal exercise capacity inversely correlates with both functional and symptom gains in this heterogeneous population.

Despite lack of knowledge in the field, several studies have underlined the role of endothelium dysfunction and platelet activation as significant players in the development and progression of chronic obstructive pulmonary disease (COPD). Indeed, endothelium plays a crucial role in vascular homeostasis and impairment, due to the inflammation process enhanced by smoking. Chronic inflammation and endothelial dysfunction have been proved to drive platelet activity. Consequently, thrombotic risk is enhanced in COPD, and might explain the higher percentage of cardiovascular death in such patients. Areas covered: This review aims to clarify the role of endothelium function and platelet hyper-activity as the pathophysiological mechanisms of the increased thrombotic risk in COPD. Expert commentary: In COPD patients, chronic inflammation does not impact only on lung parenchyma, but potentially involves all systems, including the endothelium of blood vessels. Impaired endothelium has several consequences, such as reduced vasodilatation capacity, enhanced blood coagulation, and increased platelet activation resulting in higher risk of thrombosis in COPD patients. Endothelium dysfunction and platelet activation are potential targets of therapy in patients with COPD aiming to reduce their risk of cardiovascular events

A growing body of literature suggests that comprehensive Pulmonary Rehabilitation (PR) improves symptoms and functional capacity also in patients with Interstitial Lung Disease. Aim of this study was to investigate whether the baseline level of functional capacity or lung derangement, and ILD etiology may predict and affect outcomes’ response to PR in these patients. MATERIALS AND METHODS Patients with ILD of different etiology were referred and prospectively admitted to PR, delivered according to a standardized protocol. Spirometry, Diffuse Lung Capacity for Carbon Monoxide [DLCO], incremental cyclo-ergometry test, Six Minutes Walking Distance Test [6MWDT], questionnaires on dyspnea and quality of life (St. George Respiratory Questionnaire-SGRQ, 5-point Medical Research Council scale-MRC) were assessed pre- and post- rehabilitation course; change from baseline of any measured variables was considered to assess the impact of PR on functional capacity, perceived symptoms and quality of life, respectively. Patients were stratified according to their level of DLCO, Forced Vital Capacity (FVC), 6MWTD, etiology (IPF or non-IPF), and GAP index (in IPF patients only). Analyses of changes from baseline and correlation test were conducted as appropriate. RESULTS Thirty-nine patients (mean age 66.87 ± 10.9 ys, IPF 62.5%) were enrolled and completed the PR course. 6MWDT (+54.3m, 95%CI 34.9-73.7, p < .0001), cycling time (+ 70.0%, p = .0009) and power (+60.4%, p= .008), iso-time dyspnea (-33.1%, p < .0001) and limb fatigue (-40.8%, p < .0001), SGRQ, MRC (p < .0001) significantly improved over time. Patients with lower baseline 6MWD showed greater change in 6MWD (Pearson r score = - .359, p = .034) and symptoms relief at SGRQ (r = -.229, p = .038). Different levels of FVC, DLCO, GAP index and etiology did not correlate with functional and symptoms outcomes. CONCLUSION Present study confirms that comprehensive rehabilitation is effective in patients with ILDs of different severit

Copyright © 2015 by the American Thoracic Society. Rationale: Pulmonary rehabilitation (PR) has demonstrated physiological, symptom-reducing, psychosocial, and health economic benefits for patients with chronic respiratory diseases, yet it is underutilized worldwide. Insufficient funding, resources, and reimbursement; lack of healthcare professional, payer, and patient awareness and knowledge; and additional patient-related barriers all contribute to the gap betweentheknowledge of the science andbenefits of PR and the actual delivery of PR services to suitable patients. Objectives: The objectives of this document are to enhance implementation, use, and delivery of pulmonary rehabilitation to suitable individuals worldwide. Methods: Members of the American Thoracic Society (ATS) Pulmonary Rehabilitation Assembly and the European Respiratory Society (ERS) Rehabilitation and Chronic Care Group established a Task Force and writing committee to develop a policy statement on PR. The document was modified based on feedback from expert peer reviewers. After cycles of review and revisions, the statement was reviewed and formally approved by the Board of Directors of the ATS and the Science Council and Executive Committee of the ERS. Main Results: This document articulates policy recommendations for advancing healthcare professional, payer, and patient awareness and knowledge of PR, increasing patient access to PR, and ensuring quality of PR programs. It also recommends areas of future research to establish evidence to support the development of an updated funding and reimbursement policy regarding PR. Conclusions: TheATS and ERS commit to undertake actions thatwill improve access to and delivery of PR services for suitable patients. They call on their members and other health professional societies, payers, patients, and patient advocacy groups to join in this commitment.