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Interstitial lung diseases
Published in Claudio F. Donner, Nicolino Ambrosino, Roger S. Goldstein, Pulmonary Rehabilitation, 2020
In keeping with PR programmes for other diagnostic groups, studies have noted that the benefits decrease by 6 months, associated with non-adherence, lack of supervision and disease progression (31,32). A recent systematic review of PR in IPF noted only short-term improvements in exercise capacity and HRQL (33). An RCT in which the study group underwent 6 months of PR showed longstanding improvements in exercise capacity, health status and muscle force but not physical activity (34). In a study by Dowman and colleagues (35), exercise training increased exercise capacity and HRQL in people with ILD, with larger benefits occurring in asbestosis and IPF as compared with CTD. Lower baseline function was associated with greater benefits. Benefits from this 8-week programme declined at 6 months except in those with CTD. Those in whom the exercise prescription could be progressed according to protocol improved more. Those patients with better lung function and less pulmonary hypertension (less severe disease) were noted to have the more sustained improvements (35). Patients with fibrotic idiopathic interstitial pneumonia were reported by Wallaert and colleagues to show improvements in exercise capacity, anxiety, depression and HRQL following 8 weeks of home-based PR without any change to their PA (36). In a retrospective report, patients in whom IPF and emphysema were combined had lesser improvements after a 3-week PR programme compared with those with COPD only (37).
Pneumonitis In Bone Marrow Transplant Recipients
Published in Lourdes R. Laraya-Cuasay, Walter T. Hughes, Interstitial Lung Diseases in Children, 2019
F. Leonard Johnson, Otto Schofer
Unfortunately, effective treatment is not currently available for most patients, because most pneumonias are idiopathic or due to cytomegalovirus. There is little well-documented evidence that corticosteroids are effective in idiopathic interstitial pneumonia.18, 25 In one study of patients developing radiation pneumonitis following treatment for disseminated malignant disease, corticosteroids were found to be “helpful” in approximately 20% of treated patients.18 Most patients with idiopathic interstitial pneumonia following marrow transplantation receive corticosteroids, with anecdotal reports of efficacy in a small number of cases.15
Interstitial lung disease associated with connective tissue disorders
Published in Muhunthan Thillai, David R Moller, Keith C Meyer, Clinical Handbook of Interstitial Lung Disease, 2017
Deborah Assayag, Aryeh Fischer
Identifying occult CTD among patients diagnosed with a presumed ‘idiopathic’ interstitial pneumonia (IIP) is common: a recent study from a multidisciplinary ILD program evaluated 114 consecutive ILD patients, of which 17 (15%) were confirmed to have a new CTD diagnosis (36). There is no standardized approach to this evaluation, and current guidelines recommend performing a thorough history and physical examination and testing for a panel of autoantibodies (37–39). Many centres have also found that a multidisciplinary evaluation that includes rheumatologic consultation is useful (14,34,40,41). However, because it is both unrealistic and impractical to have rheumatologic evaluation for all cases of IIP, it is left up to the individual provider to decide when to engage rheumatology, and certain guidelines have been suggested (41) (Table 14.2).
Clinical characterization of patients with interstitial lung disease: Report from a single Canadian Center
Published in Canadian Journal of Respiratory, Critical Care, and Sleep Medicine, 2021
Charlene D. Fell, Gillian C. Goobie, Chelsea A. Ford-Sahibzada, Kerri A. Johannson
Patient diagnoses were established during bi-weekly ILD multidisciplinary team (MDT) meetings, during which cases were reviewed by respirologists, thoracic radiologists and pulmonary pathologists with expertise in ILD. Diagnoses were based on contemporaneous international 2011 IPF22 and 2002 idiopathic interstitial pneumonia (IIP)23 guidelines. Because patients were enrolled into the study prior to the publication of the updated IPF and IIP guideline statements; all clinical data and diagnoses were reviewed at the time of data analysis to ensure diagnostic classification consistent with the most recent 2018 IPF24 and 2013 IIP25 guidelines. If a consensus diagnosis could not be reached by the MDT, the patient’s ILD was labeled “unclassifiable” and a working diagnosis or differential diagnosis was noted. The date of diagnosis was established as the date on which all of the necessary information was available to arrive at a diagnosis; usually, this was the date of the first CT chest or surgical lung biopsy (if performed). In many cases, the date of diagnosis preceded the patient’s first visit to the ILD clinic by several months.
Preparation, optimization, and in vivo evaluation of an inhaled solution of total saponins of Panax notoginseng and its protective effect against idiopathic pulmonary fibrosis
Published in Drug Delivery, 2020
Mengjiao Liu, Tianyi Zhang, Chen Zang, Xiaolan Cui, Jianliang Li, Guohua Wang
Idiopathic pulmonary fibrosis (IPF) is a specific form of fibrosing interstitial lung disease (ILD) that is chronic and progressive. Although IPF is the most common form of idiopathic interstitial pneumonia and is associated with a poor prognosis, the precise mechanisms underlying this condition have yet to be elucidated (Hamer et al., 2020). The incidence of IPF increases annually and has been associated with living and occupational environments, the action of certain medications, inappropriate treatments, and a variety of other factors (Hutchinson et al., 2015). A number of pathological changes are known to occur in the lungs following pulmonary fibrosis: the lungs take on a bosselated and cobblestone appearance on the pleural surface, and the lung tissue becomes enlarged and fibrotic in localized areas (Wolters et al., 2014). There are significant pathological and abnormal changes when fibrotic lungs are compared with normal pulmonary fibroblasts and extracellular matrix (Richeldi et al., 2017). The diagnosis of IPF is complex but needs to consider clinical, imaging, and histological criteria (Lynch et al., 2018). Patients with IPF may have other complications and comorbidities that could affect the treatment during the clinical course of IPF. However, only a limited number of drugs are available for patients with mild to moderate forms of IPF; the final option for patients with severe IPF is pulmonary transplantation (Lin et al., 2017; Lederer & Martinez, 2018). At present, pirfenidone and nintedanib are usually administered, either orally or intravenously, for the treatment of pulmonary fibrosis (Rogliani et al., 2016; Varone et al., 2018). However, these drugs are expensive, side effects are common, are most patients report dissatisfaction with the clinical efficacy of these treatments (Mulhall et al., 2015; Lancaster et al., 2017). Consequently, there is an urgent need to develop new drugs for the treatment of IPF.
An update on emerging drugs for the treatment of idiopathic pulmonary fibrosis
Published in Expert Opinion on Emerging Drugs, 2018
Shambhu Aryal, Steven D. Nathan
IPF affects about 3 million people worldwide [48]. It is the most common idiopathic interstitial pneumonia with an incidence of 7–16 per 100,000 cases and a prevalence of 14–43 per 100,000 people [49]. It is a disease of older people and is more common in males[2]. It is a fatal lung disease with a median survival of 2.5–5 years after diagnosis [2]. Moreover, IPF and its comorbidities have a severe impact on the quality of life of afflicted patients.