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Idiopathic Pulmonary Fibrosis: Diagnosis and Prognostic Evaluation.

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Idiopathic pulmonary fibrosis (IPF) is the most common type of idiopathic interstitial pneumonia and has a dismal prognosis.

Median age at IPF onset is 60-70 years and it is mainly related to cigarette smoke exposure. Its clinical profile is heterogeneous and different clinical phenotypes are now better defined: familial IPF, slow and rapid progressors, combined pulmonary fibrosis and emphysema, anti-neutrophil cytoplasmic antibodies/microscopic polyangiitis and IPF, and IPF associated with lung cancer.

Acute exacerbation associated with rapid functional decline is an event that does not happen infrequently and affects survival. Diagnosis requires a typical usual interstitial pneumonia (UIP) pattern on computed tomography in the appropriate clinical setting or morphological confirmation of the UIP pattern when imaging findings are not characteristic enough. Surgical lung biopsy is the gold standard to obtain valuable information for histological analysis. However, less invasive procedures (transbronchial lung biopsy or even improved transbronchial lung biopsy by cryoprobes) are now under consideration.

Prognostic indicators are mainly derived by pulmonary function tests. Recently, staging systems have been proposed. © 2013 S. Karger AG, Basel.

Transbronchial Needle Aspiration: A Systematic Review on Predictors of a Successful Aspirate.

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Transbronchial needle aspiration (TBNA) is a safe and useful sampling technique for the diagnosis of mediastinal adenopathies/masses, but its accuracy seems to be influenced by selected clinical and procedural aspects.

Objectives: We performed a systematic review to identify the main predictors of a successful transbronchial aspirate according to different clinical settings.

Methods: We searched Medline and Embase for all studies evaluating predictors of TBNA diagnostic yield, published up to February 2012. Two authors reviewed all titles/abstracts and retrieved the full text of articles that are potentially relevant to identify studies according to predefined selection criteria. The methodological quality of studies was assessed through the revised Quality Assessment of Diagnostic Accuracy Studies tool. Evidence synthesis was graded according to overall number of studies, patients involved and methodological features.

Results: Fifty-three studies, involving more than 8,000 patients and evaluating 23 potential predictive factors, were included. Major predictors in an unselected population, as well as in patients with suspected/known lung cancer, included lymph node size (short axis length ≥2 cm), presence of abnormal endoscopic findings, subcarinal and right paratracheal location, and the use of histological needle by an experienced bronchoscopist. Stage I and sampling of more than one lymph node stations were the only predictors of a successful TBNA result in patients with suspected sarcoidosis.

Conclusions: The diagnostic yield of TBNA depends on selected clinical and procedural features. Knowledge of factors that predict a positive TBNA result may help optimize the diagnostic success of the procedure in different clinical settings.

Smoking-related emphysema is associated with idiopathic pulmonary fibrosis and rheumatoid lung.

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Smoking-related emphysema is associated with idiopathic pulmonary fibrosis and rheumatoid lung.

Respirology. 2013 Jul 2;

Authors: Antoniou KM, Walsh SL, Hansell DM, Rubens MR, Marten K, Tennant R, Hansel T, Desai SR, Siafakas NM, du Bois RM, Wells AU

Abstract
BACKGROUND AND OBJECTIVE: A combined pulmonary fibrosis/emphysema syndrome (CPFE) has been proposed, but the basis for this syndrome is currently uncertain. The aim was to evaluate the prevalence of emphysema in idiopathic pulmonary fibrosis (IPF) and rheumatoid lung (RA-ILD) and to compare the morphologic features of lung fibrosis between smokers and non-smokers.
METHODS: Using high resolution computed tomography (HRCT), the prevalence of emphysema and the pack-year smoking histories associated with emphysema were compared between current/ex-smokers with IPF (n=186) or RA-ILD (n= 46) and non-COPD controls (n=103) and COPD controls (n=34). The coarseness of fibrosis was compared between smokers and non-smokers.
RESULTS: Emphysema, present in 66/186 (35%) patients with IPF and 22/46 (48%) smokers with RA-ILD, was associated with lower pack-year smoking histories than in control groups (p<0.05 for all comparisons). The presence of emphysema in IPF was positively linked to the pack-year smoking history (odds ratio = 1.04, 95% CI=1.02, 1.06, p<0.0005). In IPF, fibrosis was coarser in smokers than in non-smokers, on univariate and multivariate analysis (p<0.01 for all comparisons). In RA-ILD, fibrosis was coarser in patients with emphysema but did not differ significantly between smokers and non-smokers.
CONCLUSIONS: In IPF and RA-ILD, a high prevalence of concurrent emphysema, in association with low pack-year smoking histories, and an association between coarser pulmonary fibrosis and a history of smoking in IPF, together provide support for possible pathogenetic linkage to smoking in both diseases.

PMID: 23819865 [PubMed - as supplied by publisher]

Smoking Cessation Treatment and Outcomes Patterns Simulation: A New Framework for Evaluating the Potential Health and Economic Impact of Smoking Cessation Interventions.

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Smoking Cessation Treatment and Outcomes Patterns Simulation: A New Framework for Evaluating the Potential Health and Economic Impact of Smoking Cessation Interventions.

Pharmacoeconomics. 2013 Jul 3;

Authors: Getsios D, Marton JP, Revankar N, Ward AJ, Willke RJ, Rublee D, Ishak KJ, Xenakis JG

Abstract
BACKGROUND: Most existing models of smoking cessation treatments have considered a single quit attempt when modelling long-term outcomes.
OBJECTIVE: To develop a model to simulate smokers over their lifetimes accounting for multiple quit attempts and relapses which will allow for prediction of the long-term health and economic impact of smoking cessation strategies.
METHODS: A discrete event simulation (DES) that models individuals' life course of smoking behaviours, attempts to quit, and the cumulative impact on health and economic outcomes was developed. Each individual is assigned one of the available strategies used to support each quit attempt; the outcome of each attempt, time to relapses if abstinence is achieved, and time between quit attempts is tracked. Based on each individual's smoking or abstinence patterns, the risk of developing diseases associated with smoking (chronic obstructive pulmonary disease, lung cancer, myocardial infarction and stroke) is determined and the corresponding costs, changes to mortality, and quality of life assigned. Direct costs are assessed from the perspective of a comprehensive US healthcare payer ($US, 2012 values). Quit attempt strategies that can be evaluated in the current simulation include unassisted quit attempts, brief counselling, behavioural modification therapy, nicotine replacement therapy, bupropion, and varenicline, with the selection of strategies and time between quit attempts based on equations derived from survey data. Equations predicting the success of quit attempts as well as the short-term probability of relapse were derived from five varenicline clinical trials.
RESULTS: Concordance between the five trials and predictions from the simulation on abstinence at 12 months was high, indicating that the equations predicting success and relapse in the first year following a quit attempt were reliable. Predictions allowing for only a single quit attempt versus unrestricted attempts demonstrate important differences, with the single quit attempt simulation predicting 19 % more smoking-related diseases and 10 % higher costs associated with smoking-related diseases. Differences are most prominent in predictions of the time that individuals abstain from smoking: 13.2 years on average over a lifetime allowing for multiple quit attempts, versus only 1.2 years with single quit attempts. Differences in abstinence time estimates become substantial only 5 years into the simulation. In the multiple quit attempt simulations, younger individuals survived longer, yet had lower lifetime smoking-related disease and total costs, while the opposite was true for those with high levels of nicotine dependence.
CONCLUSION: By allowing for multiple quit attempts over the course of individuals' lives, the simulation can provide more reliable estimates on the health and economic impact of interventions designed to increase abstinence from smoking. Furthermore, the individual nature of the simulation allows for evaluation of outcomes in populations with different baseline profiles. DES provides a framework for comprehensive and appropriate predictions when applied to smoking cessation over smoker lifetimes.

PMID: 23821436 [PubMed - as supplied by publisher]

Lung transplantation for interstitial lung diseases and pulmonary hypertension.

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Lung transplantation for interstitial lung diseases and pulmonary hypertension.

Semin Respir Crit Care Med. 2013 Jun;34(3):281-7

Authors: Gottlieb J

Abstract
Lung transplantation (LTx) is an established therapeutic option for patients with various end-stage lung diseases. Presently the worldwide procedural frequency is ∼ 3,200 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 for LTx are emphysema, pulmonary fibrosis, and cystic fibrosis. Worldwide, a recent analysis of all recipients reported that 23% had idiopathic pulmonary fibrosis (IPF) and 3% had pulmonary artery hypertension. In experienced centers, candidates for transplantation are chosen according to disease-specific factors after the exclusion of contraindications. 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. Nevertheless, LTx offers a survival benefit in carefully selected patients with interstitial lung diseases and pulmonary hypertension.

PMID: 23821503 [PubMed - in process]

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