COPD Journal of Chronic Obstructive Pulmonary Disease

Publisher: Informa Healthcare

Journal description

From pathophysiology and cell biology to pharmacology and psychosocial impact, COPD: Journal Of Chronic Obstructive Pulmonary Disease publishes a wide range of original research, reviews, case studies, and conference proceedings to promote advances in the pathophysiology, diagnosis, management, and control of lung and airway disease and inflammation - providing a unique forum for the discussion, design, and evaluation of more efficient and effective strategies in patient care

Current impact factor: 2.62

Impact Factor Rankings

2015 Impact Factor Available summer 2015
2013 / 2014 Impact Factor 2.62
2012 Impact Factor 2.31
2011 Impact Factor 1.794
2010 Impact Factor 2.25

Impact factor over time

Impact factor

Additional details

5-year impact 0.00
Cited half-life 4.20
Immediacy index 0.51
Eigenfactor 0.00
Article influence 0.00
Website Journal of Chronic Obstructive Pulmonary Disease website
Other titles COPD (Online), COPD, Journal of chronic obstructive pulmonary disease, Chronic obstructive pulmonary disease
ISSN 1541-2563
OCLC 50389096
Material type Document, Periodical, Internet resource
Document type Internet Resource, Computer File, Journal / Magazine / Newspaper

Publisher details

Informa Healthcare

  • Pre-print
    • Author can archive a pre-print version
  • Post-print
    • Author cannot archive a post-print version
  • Restrictions
    • 12 months embargo
  • Conditions
    • On author's personal website or institution website
    • Publisher copyright and source must be acknowledged
    • On a non-profit server
    • Must link to publisher version
    • Publisher's version/PDF cannot be used
    • NIH funded authors may post articles to PubMed Central for release 12 months after publication
    • Wellcome Trust authors may deposit in Europe PMC after 6 months
  • Classification
    ​ yellow

Publications in this journal

  • [Show abstract] [Hide abstract]
    ABSTRACT: Alpha1-antitrypsin Deficiency (AATD) is a rare hereditary disorder with an estimated prevalence of about 1/5000 individuals in Italy. Deficient patients are at a higher risk of developing lung emphysema and chronic liver disease. The low estimated prevalence of AATD prompted the establishment of a registry with the aim of learning more about the natural history and the quality of care of these patients. The Italian registry for AATD was established in 1996. In this study, genetic and clinical findings of Italian AATD patients are presented. Moreover, we also evaluated the changes in health-related quality of life (HRQoL) in patients with COPD and AAT deficiency over a three-year period, in relation to augmentation therapy. In a period spanning 18 years (1996–2014) a total of 422 adult subjects with severe AATD were enrolled, namely 258 PI*ZZ, 74 PI*SZ, 4 PI*SS and 86 patients with at least one rare deficient allele. The 21.3% frequency for AATD patients with at least one deficient rare variant is the highest so far recorded in national registries of AATD. The registry data allow a detailed characterization of the natural course of the disease and the level of patient care, as well as confirm the usefulness of early AATD detection.
    COPD Journal of Chronic Obstructive Pulmonary Disease 05/2015; 12(S1):52-57. DOI:10.3109/15412555.2015.1023393
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    ABSTRACT: The study of rare diseases is compromised by its rarity. The establishment of national and international registries can overcome many of the problems and be used for many monogenetic conditions with relatively consistent outcomes and thus lead to a consistency of clinical management by centres of excellence. However, in Alpha-1 antitrypsin deficiency (AATD), the outcome is highly variable in terms of the organ(s) most affected and the diversity of disease penetration and progression. This creates the added difficulty of understanding the disease sufficiently to monitor and advise the patients to the standard required and importantly design and deliver clinical trials that address the many facets of the disease. The development of research registries and centres of excellence provides the necessary expertise and data to further the understanding and management of diseases like AATD though with significant cost implications. The ADAPT programme was established in 1996 with extensive core funding to enable patients to be seen from all regions of the United Kingdom as an addition to the National Health Service without appointment time constraints and with the purpose of collecting extensive state of the art demographics. The model has proven to be highly productive providing new insights especially into the lung disease, generating and delivering on clinical trials and importantly establishing active patient groups and participation.
    COPD Journal of Chronic Obstructive Pulmonary Disease 05/2015; 12(S1):63-68. DOI:10.3109/15412555.2015.1021911
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    ABSTRACT: Alpha-1 antitrypsin deficiency (AATD) is associated with an increased risk of pulmonary emphysema and liver disease. The growing interest in this deficiency in Spain led to the development of the Spanish Registry of Patients with Alpha-1 Antitrypsin Deficiency (REDAAT) in 1993. At present, the REDAAT is a network of more than 350 health care professionals and the database includes a total of 511 individuals. The adult population included consists of 469 individuals (91.8% of the total) and their phenotype distribution is: 348 Pi*ZZ (74.2%), 100 Pi*SZ (21.3%) and 21 carriers of rare variants (4.5%). The most frequent diagnosis is lung disease (74.6%). Patients with chronic obstructive pulmonary disease (COPD) registered in the REDAAT constitute approximately 15% of the expected cases of AATD-related COPD in Spain. Pi*ZZ showed more severe impairment in lung function and younger age at baseline compared with Pi*SZ. The mean decline in FEV1 in the Pi*ZZ subgroup was -23 ml/year (SD:142.8), being -18 ml/year (SD:108.8) in Pi*SZ. Forty-five percent of the Pi*ZZ individuals received augmentation therapy. A total of 61 deaths was recorded. The characteristics of the REDAAT population demonstrate some differential trends compared to other series: distribution of phenotypes, inclusion of children and patients treated with replacement therapy. Patients with the Pi*SZ phenotype were older and had milder lung function impairment. The most important challenge of this registry is to collect good quality long-term data that will allow better understanding of the natural history of the disease in real life.
    COPD Journal of Chronic Obstructive Pulmonary Disease 05/2015; 12(S1):27-31. DOI:10.3109/15412555.2015.1021912
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    ABSTRACT: The French registry of patients with alpha-1 antitrypsin deficiency (AATD)-associated emphysema was launched in 2006. Here, we aimed to report on the baseline characteristics of these patients, their health-related quality of life (HRQoL) and factors associated with HRQoL. Another goal was to survey the practices of French physicians regarding augmentation therapy. We included 273 patients with AATD, emphysema, obstructive-pattern [forced expiratory volume in 1 sec/forced volume capacity (FEV1/FVC) < 0.7], FEV1 ≤ 80% predicted. Mean (SD) age was 51.8 (11.1) years, 240 (87.9%) of patients were smokers or ex-smokers, mean (SD) FEV1 was 40.5% (15.7) predicted. Mean (SD) SGRQ score was 49.0 (20.0) and was higher for females than males (52.7 [20.7] vs 46.8 [18.2]; p = 0.01). Dyspnea showed the strongest association with SGRQ score (r = 0.65; p < 0.0001), followed by chronic bronchitis (r = 0.33; p < 0.0001) and wheezing (r = 0.32; p < 0.0001). Number of exacerbations in the year before inclusion was also significantly associated with SGRQ score (r = 0.36; p < 0.0001). The SGRQ score was associated with the 6-min walking distance (r = -0.53, p < 0.0001), FEV1 (% predicted, r = -0.53, p < 0.0001) and DLCO (% predicted, r = -0.52, p < 0.0001). It was also associated with the GOLD 2006 (r = 0.53; p < 0.0001) and GOLD 2011 (r = 0.63; p < 0.0001) classifications and with the BODE index (r = 0.37; p < 0.0001). Age, history of tobacco smoking or current smoking did not show any association with SGRQ total scores. On multivariate analysis, a model including age, chronic bronchitis, dyspnea (MRC scale), diffusing lung capacity and 6-min walking distance explained 57% of the variation in the score. The French registry provides important insights into the clinical characteristics of French patients with AATD-related emphysema.
    COPD Journal of Chronic Obstructive Pulmonary Disease 05/2015; 12(S1):46-51. DOI:10.3109/15412555.2015.1022645
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    ABSTRACT: Individuals with Alpha-1 antitrypsin deficiency (AATD) have mutations in the SERPINA1 gene causing genetic susceptibility to early onset lung and liver disease that may result in premature death. Environmental interactions have a significant impact in determining the disease phenotype and outcome in AATD. The aim of this study was to assess the impact of smoke exposure on the clinical phenotype of AATD in Ireland. Clinical demographics and available thoracic computerised tomography (CT) imaging were detected from 139 PiZZ individuals identified from the Irish National AATD Registry. Clinical information was collected by questionnaire. Data was analysed to assess AATD disease severity and evaluate predictors of clinical phenotype. Questionnaires were collected from 107/139 (77%) and thoracic CT evaluation was available in 72/107 (67.2%). 74% of respondents had severe Chronic Obstructive Pulmonary Disease (COPD) (GOLD stage C or D). Cigarette smoking was the greatest predictor of impairment in FEV1 and DLCO (%predicted) and the extent of emphysema correlated most significantly with DLCO. Interestingly the rate of FEV1 decline was similar in ex-smokers when compared to never-smokers. Passive smoke exposure in childhood resulted in a greater total pack-year smoking history. Radiological evidence of bronchiectasis was a common finding and associated with increasing age. The Irish National AATD Registry facilitates clinical and basic science research of this condition in Ireland. This study illustrates the detrimental effect of smoke exposure on the clinical phenotype of AATD in Ireland and the benefit of immediate smoking cessation at any stage of lung disease.
    COPD Journal of Chronic Obstructive Pulmonary Disease 05/2015; 12(S1):2-9. DOI:10.3109/15412555.2015.1021913
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    ABSTRACT: The Alpha-1 Foundation Research Registry has a long history of facilitating research studies in the United States. The current contact registry is used to invite participants to research studies. However, the next generation of individuals diagnosed with alpha-1 antitrypsin deficiency may look quite different from historical cohorts. This paper uses data from the Alpha Coded Testing (ACT) study, a home genetic testing program in which deficient individuals are invited to participate in the Registry, to demonstrate the impact that selection bias can introduce into registry data. Environmental tobacco smoke (ETS) exposure is rapidly declining in the United States. We queried whether consecutive non-smokers with or without childhood ETS in ACT (N = 801) had been diagnosed with COPD more often if deficiency genes were defined in subsequent testing. The prevalence of COPD was not different between cohorts with or without ETS exposure between normal (PiMM and PiMS), moderately deficient (PiMZ, PiMNull, and PiSS), and severely deficient (PiSZ, PiZZ, PiSNull, and PiZNull) genotypes. Surprisingly, age adjusted COPD Severity Scores in this cohort were higher for individuals with normal genotypes compared to moderately (P<0.001) and severely (P = 0.04) deficient genotypes. Ascertainment bias of testing within families (which yields the highest incidence of deficiency genotypes) also finds many family members without symptoms, even over the age of 40. We conclude that the future utility of registries will depend on accurate determination of testing mechanics. Larger database initiatives using the COPD Patient Powered Research Network are described.
    COPD Journal of Chronic Obstructive Pulmonary Disease 05/2015; 12(S1):42-45. DOI:10.3109/15412555.2015.1021914
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    ABSTRACT: Alpha-1-antitrypsin deficiency (AATD) is a rare condition with clinical mani-festations of the lung and the liver. There is evidence that the gender affects the clinical presentation of non-AATD chronic obstructive lung disease (COPD). The aim of this study was to analyze gender-dependent disease pattern in AATD-based COPD. Data from 1066 individuals from the German AATD registry were analyzed by descriptive and analytical statistics. The AAT genotypes comprised 820 individuals with PiZZ (male 56%, female 45%), 109 with PI SZ (male 55%; female 45%), and others (n = 137). A subgroup of 422 patients with available post-bronchodilator FEV1% predicted was analyzed in detail after stratification in spirometric GOLD stages I-IV. The age of the registered individuals is 52.2 ± 13.4 years (male: 51.91 ± 13.86 years; female: 52.76 ± 13.39 years). Female patients with GOLD I-IV showed lower numbers of pack-years and lower BMI. The time between the first symptom and the establishment of the correct diagnosis was significantly longer in female (14.47 ± 16.46 years) as compared to male individuals (12.39 ± - 14.38 years, p = 0.04). In conclusion, the data of the registry allow to characterize the natural course of the disease and highlight differences in the clinical presentation of patients with AATD-dependent COPD.
    COPD Journal of Chronic Obstructive Pulmonary Disease 05/2015; 12(S1):58-62. DOI:10.3109/15412555.2015.1023785
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    ABSTRACT: The alpha-1 antitrypsin deficiency (AATD) targeted screening program, together with the National Registry, were established in Poland in 2010 soon after the AATD diagnostics became available. Between 2010 and 2014 a total of 2525 samples were collected from respiratory patients countrywide; 55 patients with severe AAT deficiency or rare mutations were identified and registered, including 36 PiZZ subjects (65%). The majority of AATD patients were diagnosed with COPD (40%) or emphysema (7%), but also with bronchial asthma (16%) and bronchiectasis (13%). Therefore, the registry has proved instrumental in setting-up the AATD-dedicated network of respiratory medical centres in Poland. Since augmentation therapy is not reimbursed in our country, the smoking cessation guidance, optimal pharmacotherapy of respiratory symptoms as well the early detection, and effective treatment of exacerbations is absolutely essential.
    COPD Journal of Chronic Obstructive Pulmonary Disease 05/2015; 12(S1):22-26. DOI:10.3109/15412555.2015.1021915
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    ABSTRACT: A Belgian alpha-1-antitrypsin (AAT) deficiency registry has been established in 2003. Currently 55 patients are included. At the same time, a working group has been set up for publishing national guidelines. In 2014, several Belgian patients founded Alpha-1 Global. We hope that the integrated activities of all the stakeholders involved in AAT deficiency will permit a high quality care for all patients suffering from this disabling disease.
    COPD Journal of Chronic Obstructive Pulmonary Disease 05/2015; 12(S1):10-14. DOI:10.3109/15412555.2015.1021916
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    ABSTRACT: The Swedish national register of severe alpha1-antitrypsin (AAT) deficiency was established in 1991. The main aims are to prospectively study the natural history of severe AAT deficiency, and to improve the knowledge of AAT deficiency. The inclusion criteria in the register are age ≥18 years, and the PiZ phenotype diagnosed by isoelectric focusing. The register is kept updated by means of repeated questionnaires providing data to allow analysis of the mode of identification, lung and liver function, smoking-habits, respiratory symptoms and diagnoses as reported by physicians. Until February 2014, a total of 1553 PiZZ individuals had been included in the register. The 1102 subjects still alive constituted about 20% of the adult PiZZ individuals in Sweden. Forty-three percent of the subjects had been identified during investigation of respiratory symptoms, 7% by an investigation of liver disease, 26% in an investigation of other pathological conditions, and 24% in a population or family screening. Forty five percent of the subjects had never smoked, 47% were ex-smokers, and 8% current smokers. Twenty-eight percent of the never-smokers, 72% of the ex-smokers, and 61% of the current smokers fulfilled the criteria for COPD with a FEV1/FVC ratio of <0.70. Among the 451 deceased, the most common cause of death was respiratory diseases (55%), followed by liver diseases (13%). We conclude that the detection rate of severe AAT deficiency is relatively high in Sweden. Large numbers of subjects are identified for other reasons than respiratory symptoms, and the majority of these have never smoked.
    COPD Journal of Chronic Obstructive Pulmonary Disease 05/2015; 12(S1):36-41. DOI:10.3109/15412555.2015.1021909
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    ABSTRACT: The assessment of biomarkers in biological samples from the lung has long been employed. Upon cooling water vapor present in exhaled breath, variable amounts of droplets of condensate (EBC) containing volatile and non-volatile compounds may be easily and non-invasively obtained from patients of any age.Objective of the present study was to compare the level of EBC conductivity determined for cohorts of individuals with different inflammatory lung disorders with that of healthy never-smoking individuals.The conductivity in EBC of PiZZ-Alpha-1-antitrypsin deficiency patients with a diagnosis of emphysema (PiZZ-AATD) was 3 fold lower than in spouse controls (54.5 ± 11.6 vs 165.3 ± 10.7 μS/cm). Non-PiZZ emphysema patients had conductivity in EBC of 59.6 ± 5.8 μS/cm and patients with sarcoidosis without airflow obstruction had EBC conductivity of 178,8 ± 6,2 μS/cm, not significantly different (p = 0.5) from healthy controls. Conductivity in serial EBC samples from patients with PiZZ-AATD emphysema and healthy controls was stable in 6 different samples collected over a period of 14 months. We conclude that conductivity values in EBC can be used as a correction factor for dilution of non-volatile components in EBC.
    COPD Journal of Chronic Obstructive Pulmonary Disease 05/2015; 12(S1):32-35. DOI:10.3109/15412555.2015.1021910
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    ABSTRACT: Since 1999, as part of the Alpha1 International Registry (AIR), the Canadian Alpha-1 Antitrypsin Deficiency (AATD) Registry has maintained demographic and medical information volunteered by AATD individuals. We undertook a retrospective chart review to describe the characteristics of registry participants. Inclusion criteria were ZZ phenotype or other severe deficiency and written consent. We reviewed baseline medical records and annual follow-ups, conducted by mail. The number of registrants ranged from 8.7 per million in British Columbia and Ontario to 1.3 per million in Quebec. Similarly, the rate of augmentation therapy use ranged from 7.7 per million in British Columbia to 0.1 per million in Quebec. 290 patients (146 males), most PiZZ, were enrolled by 2013. Patients with lung disease reported symptoms onset at (mean ± SD) 40 ± 11 years but were diagnosed as AATD at 47 ± 10 years. Typical patients were ex-smokers with respiratory symptoms, severely reduced FEV1, an accelerated rate of FEV1 decline, and minimal bronchodilator response. A subgroup diagnosed by liver disease or familial screening was younger and had better preserved lung function but a similar rate of FEV1 decline. There were 63 deaths, of which 29 were lung-related and 6 were liver-related. Average age at death was 60.5 ± 11.2 years. Discussion: Most patients experience a diagnostic delay of seven years after symptom onset, a period during which lung health may deteriorate further. There is marked regional variation in the rate of diagnosis and specific therapy usage for AAT in Canada.
    COPD Journal of Chronic Obstructive Pulmonary Disease 05/2015; 12(S1):15-21. DOI:10.3109/15412555.2015.1021908
  • COPD Journal of Chronic Obstructive Pulmonary Disease 04/2015; 12(2):113-114. DOI:10.3109/15412555.2015.1018510
  • COPD Journal of Chronic Obstructive Pulmonary Disease 03/2015; DOI:10.3109/15412555.2014.995757
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    ABSTRACT: Diaphragmatic weakness in chronic obstructive pulmonary disease (COPD) is ascribed to hyperinflation-induced diaphragm shortening as well as impairment in cellular and subcellular structures. Although phrenic neuropathy is known to cause diaphragmatic weakness, phrenic neuropathy is rarely considered in COPD. This work aimed at assessing phrenic nerve conduction in COPD and its relation to radiographic hyperinflation and pulmonary function. Forty COPD patients were evaluated. Radiographic parameters of lung hyperinflation were measured on postero-anterior and lateral chest x-ray films. Flow volume loop parameters were obtained from all patients. Motor conduction study of the phrenic nerves was performed and potentials were recorded over the xiphoid process and the ipsilateral 7th intercostal space. Twenty-seven healthy subjects were enrolled as controls. Parameters of phrenic nerve conduction differed significantly in patients compared to controls. Phrenic nerve abnormalities were detected in 17 patients (42.5%). Electrophysiological measures correlated with diaphragmatic angle of depression on lateral view films and with lung height on postero-anterior films. They did not correlate with the flow volume loop data or disease severity score. Phrenic nerve conduction abnormality is an appreciated finding in COPD. Nerve stretching associated with diaphragmatic descent can be a suggested mechanism for nerve lesion. The presence of phrenic neuropathy may be an additional contributing factor to diaphragmatic dysfunction in COPD patients.
    COPD Journal of Chronic Obstructive Pulmonary Disease 03/2015; DOI:10.3109/15412555.2014.993465
  • COPD Journal of Chronic Obstructive Pulmonary Disease 03/2015; DOI:10.3109/15412555.2014.995756
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    ABSTRACT: Anxiety and depression are common comorbidities in people with chronic obstructive pulmonary disease (COPD). While these comorbidities could potentially lead to a higher motivation to learn about self-management, they could also inhibit patients from translating this knowledge into appropriate self-management behaviours. This paper explores the moderating effects of anxiety and depression on a health-mentoring intervention, focusing on mechanisms of change (mediation). 182 COPD patients participated in an RCT, with anxiety and depression assessed by the Hospital Anxiety and Depression Scale (HADS), self-management knowledge by the Partners in Health Scale, and spontaneous physical activity using accelerometers, all measured at baseline, 6 and 12 months. The moderated mediation model tested the intervention's effect on physical activity, mediated via changes in self-management knowledge, at different levels of anxiety and depression. Knowledge mediated the effect of the intervention on changes in physical activity only for participants reporting low levels of anxiety or depression. Both acted as moderators: Increased knowledge led to more physical activity among participants reporting low anxiety or depression and to less activity among highly anxious or depressed participants. Although health-mentoring interventions can be an effective tool to increase knowledge and physical activity among COPD patients, it is essential to take anxiety and depression into account, as increased knowledge may have detrimental effects in highly anxious or depressed participants. This suggests that patients with elevated anxiety or depression may need to be treated appropriately before engaging in chronic disease self-management interventions.
    COPD Journal of Chronic Obstructive Pulmonary Disease 03/2015; DOI:10.3109/15412555.2014.995289
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    ABSTRACT: Utility measures that summarize the health-related quality of life of an individual using a single number usually between 0 (death) and 1 (full health) are useful to quantify the benefits of health care interventions in terms of quality-adjusted life years (QALYs) and to help prioritizing limited health resources from cost-effectiveness analyses among patients with different health conditions. To determine utility scores in patients with oxygen-dependent chronic obstructive pulmonary disease (COPD). Patients with oxygen-dependent COPD (the cases) were matched, on a 1:2 basis, to COPD controls according to gender, age (± 5 years) and FEV1 (±5% predicted). Utility scores were obtained from the SF-6D, a measure derived from the SF-36. From a cohort of 102 patients with oxygen-dependent COPD, 68 (42 men; mean age: 71 years; mean FEV1: 35% predicted) were successfully matched with 136 controls. We found clinically and statistically significant differences in mean utility scores between cases (0.588 ± 0.071) and controls (0.627 ± 0.085; p = 0.001). The same differences were observed in men and women. Oxygen-dependence adds to the burden of disease in terms of quality of life. These utility scores may be useful in cost-utility analyses involving patients with oxygen-dependent COPD.
    COPD Journal of Chronic Obstructive Pulmonary Disease 03/2015; DOI:10.3109/15412555.2014.995290
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    ABSTRACT: Chronic obstructive pulmonary disease (COPD) guidelines suggest using inhaled corticosteroids (ICS) in patients with severe airflow limitation or those at high risk of exacerbations. This recommendation is based on evidence demonstrating that ICS, especially when prescribed in fixed-dose combinations (FDC) with long-acting β2 agonists (LABA), improve quality of life (QoL), decrease exacerbations and hospitalisations, and have been associated with a trend towards a reduction in all-cause mortality. Audit shows that routine prescribing practice frequently uses inhaler therapies outside current guidelines recommendations; severe to very severe disease constitutes about 20% of all COPD patients, but up to 75% of COPD patients are prescribed an ICS, with significant numbers given ICS/LABA as first-line maintenance therapy. The role of ICS in the treatment paradigm for COPD is changing, driven by the growing evidence of increased risk of pneumonia, and the introduction of a new class of FDC; LABA and long-acting muscarinic antagonists (LAMA), which simplify dual bronchodilation and present a plausible alternative therapy. As the evidence base for dual therapy bronchodilation expands, it is likely that maximal bronchodilation will move up the treatment algorithm and ICS reserved for those with more severe disease who are not controlled on dual therapy. This change has already manifested in local COPD algorithms, such as those at Tayside, and represents a significant change in recommended prescribing practice. This review reassesses the role of ICS in the shifting treatment paradigm, in the context of alternative treatment options that provide maximal bronchodilation.
    COPD Journal of Chronic Obstructive Pulmonary Disease 03/2015; DOI:10.3109/15412555.2014.995288