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Interstitial lung diseases are heterogeneous entities with diverse clinical presentations. Among them, idiopathic pulmonary fibrosis and connective tissue disease-associated interstitial lung disease are specific categories that pulmonologists are most likely to encounter in the clinical field. Despite the accumulated data from extensive clinical trial and observations, we continue to have many issues which need to be resolved in this field.

In this update, we present the review of several articles regarding the clinical presentation, prognosis and treatment of patients with idiopathic pulmonary fibrosis or connective tissue disease-associated interstitial lung disease.

Current status of bronchoscopic lung volume reduction with endobronchial valves.

Thorax. 2013 Sep 5;

Authors: Shah PL, Herth FJ

Abstract
INTRODUCTION: Chronic obstructive pulmonary disease (COPD) is a major cause of morbidity and mortality worldwide. Emphysema is a component of COPD characterised by hyperinflation resulting in reduced gas exchange and interference with breathing mechanics. Endoscopic lung volume reduction using one-way valves to induce atelectasis of the hyperinflated lobe has been developed and studied in clinical trials over the last decade.
METHODS: Searches for appropriate studies were undertaken on PubMed and Clinical Trials Databases using the search terms COPD, emphysema, lung volume reduction and endobronchial valves.
RESULTS: The evidence from the randomised clinical trials suggests that complete lobar occlusion in the absence of collateral ventilation or where there is an intact lobar fissure are the key predictors for clinical success. Other indicators are greater heterogeneity in disease distribution between upper and lower lobes. The proportion of patients that respond to treatment improves from 20% in the unselected population to 75% with appropriate patient selection. The safety profile for endobronchial valves in this severely affected group of patients with emphysema was acceptable and the main adverse events observed were an excess of pneumothoraces.
CONCLUSION: Selected patients have the potential of significant benefit in terms of lung function, exercise capacity and possibly even survival. These considerations are essential in-order to maximise patient benefit in a resource-limited environment and also to ensure that beneficial treatments are available for the appropriate patient.

PMID: 24008689 [PubMed - as supplied by publisher]

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No association of 25-hydroxyvitamin D with exacerbations in primary care patients with COPD.

Chest. 2013 Sep 5;

Authors: Puhan MA, Siebeling L, Frei A, Zoller M, Bischoff-Ferrari H, Ter Riet G

Abstract
ABSTRACT BACKGROUND: Cross-sectional studies suggest an association of 25-hydroxyvitamin D with exacerbations in patients with COPD but longitudinal evidence from cohort studies is scarce. Our aim was to assess the association of serum 25-hydroxyvitamin D with exacerbations and mortality in primary care patients with COPD.
METHODS: We included 356 COPD patients (GOLD stages II-IV, free of exacerbations for ≥4 weeks) from a prospective cohort study in Dutch and Swiss primary care settings in the main analysis where we assessed the association of 25-hydroxyvitamin D with (centrally adjudicated) exacerbations and mortality using negative binomial and Cox regression, respectively.
RESULTS: Baseline mean serum 25-hydroxyvitamin D concentration was 15.5 ng/dl (SD 8.9) and 274 patients (77.0%) had 25-hydroxyvitamin D deficiency (<20 ng/dl). Compared to patients with severe 25-hydroxyvitamin D deficiency (<10 ng/dl, n=106 [29.8%]), patients with moderately deficient (10-19.99 ng/dl, n=168 [47.2%]) and insufficient (20-29.99 ng/dl, n=58 [16.3%]) concentrations had the same risk for exacerbations (incidence rate ratios of 1.01 [95% CI 0.77-1.57] and 1.00 [0.62-1.61], respectively). In patients with desirable concentrations (>30 ng/dl, n=24 [6.7%]) the risk was lower, although not statistically significantly (0.72 [0.37-1.42]). Including patients with vitamin D supplements, using different cut-offs for 25-hydroxyvitamin D or competing risk models did not materially change the results. We did not find a statistically significant association of 25-hydroxyvitamin D concentration with mortality.
CONCLUSION: This longitudinal study in a real-world COPD population that carefully minimized misclassification of exacerbations and the influence of confounding did not show an association of 25-hydroxyvitamin D with exacerbations and mortality. STUDY REGISTRATION ClinicalTrials.gov (NCT00706602).

PMID: 24008868 [PubMed - as supplied by publisher]

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Spirometry is underused in the diagnosis and monitoring of patients with chronic obstructive pulmonary disease (COPD).

Int J Chron Obstruct Pulmon Dis. 2013;8:389-95

Authors: Yu WC, Fu SN, Tai EL, Yeung YC, Kwong KC, Chang Y, Tam CM, Yiu YK

Abstract
Spirometry is important in the diagnosis and management of chronic obstructive pulmonary disease (COPD), yet it is a common clinical observation that it is underused though the extent is unclear. This survey aims to examine the use of spirometry in the diagnosis and management of COPD patients in a district in Hong Kong. It is a cross-sectional survey involving four clinic settings: hospital-based respiratory specialist clinic, hospital-based mixed medical specialist clinic, general outpatient clinic (primary care), and tuberculosis and chest clinic. Thirty physician-diagnosed COPD patients were randomly selected from each of the four clinic groups. All of them had a forced expiratory volume in 1 second (FEV1) to forced vital capacity ratio less than 0.70 and had been followed up at the participating clinic for at least 6 months for COPD treatment. Of 126 patients who underwent spirometry, six (4.8%) did not have COPD. Of the 120 COPD patients, there were 111 males and mean post-bronchodilator FEV1 was 46.2% predicted. Only 22 patients (18.3%) had spirometry done during diagnostic workup, and 64 patients (53.3%) had spirometry done ever. The only independent factor predicting spirometry done ever was absence of old pulmonary tuberculosis and follow-up at respiratory specialist clinic. Age, sex, smoking status, comorbidities, duration of COPD, percentage predicted FEV1, body mass index, 6-minute walking distance, and Medical Research Council dyspnea score were not predictive. We conclude that spirometry is underused in general but especially by nonrespiratory physicians and family physicians in the management of COPD patients. More effort at educating the medical community is urgently needed.

PMID: 24009418 [PubMed - in process]