A rare presentation of histologically proven sarcoidosis of the knee: A case report and brief review of the literature.

We here report a patient with histologically proven sarcoidosis of the knee, a rare localization of sarcoidosis, which usually presents itself as a pulmonary disease. Case reports of radiological images that suggest osseous sarcoidosis of the appendicular skeleton are not so rare, however few are histologically proven. Since in our patient MRI could not distinguish between sarcoidosis and another (possibly malignant) disease, histological proof was obtained through a CT-guided biopsy. Imaging and treatment guidelines for extrapulmonary sarcoidosis are inexistent, due to lack of randomized trials.

Inhaler technique: facts and fantasies. A view from the Aerosol Drug Management Improvement Team (ADMIT).

Health professionals tasked with advising patients with asthma and chronic obstructive pulmonary disease (COPD) how to use inhaler devices properly and what to do about unwanted effects will be aware of a variety of commonly held precepts. The evidence for many of these is, however, lacking or old and therefore in need of re-examination. Few would disagree that facilitating and encouraging regular and proper use of inhaler devices for the treatment of asthma and COPD is critical for successful outcomes. It seems logical that the abandonment of unnecessary or ill-founded practices forms an integral part of this process: the use of inhalers is bewildering enough, particularly with regular introduction of new drugs, devices and ancillary equipment, without unnecessary and pointless adages. We review the evidence, or lack thereof, underlying ten items of inhaler 'lore' commonly passed on by health professionals to each other and thence to patients. The exercise is intended as a pragmatic, evidence-informed review by a group of clinicians with appropriate experience. It is not intended to be an exhaustive review of the literature; rather, we aim to stimulate debate, and to encourage researchers to challenge some of these ideas and to provide new, updated evidence on which to base relevant, meaningful advice in the future. The discussion on each item is followed by a formal, expert opinion by members of the ADMIT Working Group.

Erratum: Inhaler technique: facts and fantasies. A view from the Aerosol Drug Management Improvement Team (ADMIT).

[This corrects the article DOI: 10.1038/npjpcrm.2016.17.].

Increase in ventilated air spaces after eradication of chronic methicillin-resistant Staphylococcus aureus infection in cystic fibrosis patients.

Effective microbiogical eradication of methicillin-resistant Staphylococcus aureus (MRSA) in patients with cystic fibrosis (CF) can be obtained, but its effect is not always clear-cut in terms of spirometric indices. The aim of this observational prospective cohort study was to study the potential effect of eradication of chronic MRSA infection on lung function including ventilation distribution. Six CF patients, chronically colonized with MRSA (median age: 21 years (range 14-46); median FEV1: 76 (95%CI 58-98)%pred) were successfully eradicated using oral rifampicin and fusidic acid in combination with topical decolonization measures. Lung function and multiple breath washout test were performed at the start and at the end of the eradication protocol and after an average follow-up period of 7·5±1·5(SD) months. One patient cultured MRSA again 4 months after successful eradication. All patients reported reduced sputum production and viscosity. By the end of the follow-up period, there was an increase in ventilated FRCMBW and no change in plethysmographic FRCPL. This resulted in a significant decrease of trapped air by half a litre (from 579 to 40 ml; P = 0·013). Lung clearance index (LCI) also showed a small but significant decrease (from 7·2 to 6·7; P = 0·014) after eradication of MRSA. We conclude that MRSA eradication can be successful, also in terms of recruitment of previously unventilated air spaces, potentially due to reduced sputum production and/or viscosity.

Chronic bronchitis in COPD patients is associated with increased risk of exacerbations: a cross-sectional multicentre study.

BACKGROUND AND AIMS: Chronic bronchitis (CB) in chronic obstructive pulmonary disease (COPD) patients is associated with increased mortality, frequent exacerbations and faster disease progression. This study investigates the prevalence of CB in a large population of COPD patients to identify features associated with CB. METHODS: Cross-sectional multicentre study in patients with Global Initiative for Chronic Obstructive Lung Disease (GOLD) stages 2-4 from Belgium and Luxembourg. RESULTS: The 974 patients included were on average 67.8 ± 9.6 years old; 72% were male, FEV1 was 52.5 ± 15.8% of predicted. The prevalence of CB was 64% (622/974). In patients with CB, the number of pack-years smoked and the prevalence of chronic respiratory failure, cachexia and skeletal muscle wasting were significantly higher, whereas FEV1 and FEV1 /VC were lower. The prevalence of CB increased with GOLD stage and was higher in patients with emphysema and those exposed to occupational risk factors. The CB group had more exacerbations, a higher percentage of patients with frequent exacerbations (37.3% vs. 14.2% of patients; p < 0.0001), increased COPD-related, non-intensive care unit hospitalisations and all-cause hospitalisation rates. In multiple logistic regression analysis, frequent exacerbation was the most important independent variable associated with CB, followed by current smoking, chronic respiratory failure, COPD duration and age. CONCLUSIONS: CB prevalence in GOLD stage 2-4 COPD patients is high. CB is related to current tobacco smoking, and prevalence increases with COPD severity and duration, emphysema and age. CB could be the hallmark of a subtype of COPD easy to identify in clinical practice, associated with increased disease severity and increased risk of exacerbation.

Prescription of inhalers in asthma and COPD: towards a rational, rapid and effective approach.

Inhaled medication is the cornerstone of the pharmacological treatment of patients with asthma and COPD. The major two classes of inhaled medication include corticosteroids (ICS) and bronchodilators. There is a wide diversity in molecules in both classes. Moreover, there is a wide variation in delivery systems. The correct use of inhalers is not granted and patients often incur in many mistakes when using pMDIs and DPIs, despite repeated instructions. A better matching between patient and device could be accomplished if the physician is aware of: (1) the patient characteristics (disease, severity, fluctuation in airflow obstruction, etc); (2) what class of medication is indicated; (3) where in the lung the medication should be delivered; and, (4) how this can be best achieved by a given device in this specific patient. We focus on the prescription of pMDIs and DPIs at the GP office or at the outpatient clinic of the hospital, and we propose an evidence based approach enabling the caregiver to make a rational choice in only a few minutes by just considering the following four simple questions: Who?, What? Where? and How? (the so-called 3W-H approach).

Severe right heart failure in a patient with chronic obstructive lung disease: a diagnostic challenge.

A 55-year-old male was admitted for evaluation of severe dyspnoea and hypoxaemia. Physical examination upon admission showed elevated jugular venous pressure and an accentuated second heart sound. Chest radiograph showed cardiomegaly with increased bibasilar markings. Arterial blood gas analysis while breathing room air showed marked hypoxaemia. High resolution computed tomography angiography of the chest showed modestly enlarged mediastinal lymph nodes with discrete diffuse ground-glass attenuation especially at the lower lung zones. Positron emission tomography using 18F labelled 2-deoxy-D-glucose (FDG) demonstrated the mediastinal lymph nodes were FDG-avid. Transthoracic echocardiography showed dilated hypokinetic right heart chambers with bulging of the interventricular septum to the left, compatible with acute cor-pulmonale. From the tricuspid regurgitation jet measurement a systolic pulmonary artery pressure (PAP) of 48 mmHg was estimated. Patent foramen ovale was suspected on bubble test. Right heart catheterisation confirmed pulmonary arterial hypertension: mPAP 47 mmHg, pulmonary artery occlusion pressure 5 mmHg, cardiac index 1.1 L/min/m2, pulmonary vascular resistance (PVR) 959 dyne.sec.cm(-5). Pulmonary function tests showed a marked diffusing capacity for carbon monoxide (DLCO) decrease of 32% predicted but no obstructive lung deficit. Before an open lung biopsy could be scheduled the patient developed acute cardiogenic shock. At autopsy pulmonary veno-occlusive disease with marked pulmonary hypertension was diagnosed.

COPD awareness survey: do Belgian pulmonary physicians comply with the GOLD guidelines 2010?

Chronic Obstructive Pulmonary Disease (COPD) is underestimated, underdiagnosed and often under-treated in the general population. A survey of 17 structured questions, delivered to all Belgian pulmonary physicians (PPs) (116 responses), evaluated diagnosis and treatment strategies in accordance with the Global Initiative for Chronic Obstructive Lung Disease (GOLD) guidelines 2010 and assessed opinions about the importance of diurnal variation of COPD symptoms. All COPD diagnoses (37% new cases) were spirometry confirmed. Main diagnostic parameters were symptoms (99%), external risk factors (99%), clinical examination (97%), exacerbations (96%) and patient mobility (96%). FEV1 (forced expiratory volume in 1s) (97%) or FEV1/FVC (ratio of FEV1 to forced vital capacity) (93%) were used most to assess diagnosis and severity. The 3 most important therapeutic objectives were symptom relief, preventing exacerbations, and improving quality of life; if these were not reached, the preferred strategy (60% of PPs) was adding another medication. Treatment strategies varied with COPD stage: short-acting beta2-agonists (90%) and short-acting anti-cholinergics (59%) were used for GOLD I disease, whereas for higher stages long-acting beta2-agonists (36-48%) and long-acting anti-cholinergics (79%) were given with inhaled corticosteroids (21-67%). Symptoms were perceived to vary throughout the day, affecting quality of life (97%) and mobility (89%). In particular, respiratory symptoms were more severe in the morning (51-92%), leading PPs to adapt treatment (69%). This survey demonstrated that management of COPD by PPs in Belgium is generally in line with the GOLD guidelines 2010 and that they perceive morning symptoms as being frequent and having an impact on patient's life.

An incident case of primary acquired pulmonary alveolar proteinosis.

This is a case of a primary acquired pulmonary alveolar proteinosis (PAP) in an asymptomatic patient, on the waiting list for kidney transplantation, confirmed on lung biopsy and by identifying anti-GM-CSF antibodies.

Whole-lung lavage: a successful treatment for restoring acinar ventilation distribution in primary acquired pulmonary alveolar proteinosis.

A 51-year-old active smoker with primary acquired pulmonary alveolar proteinosis (PAP) diagnosed by biopsy and anti-GM-CSF antibodies was treated safely with whole-lung lavage (WLL). This resulted in a rapid improvement of symptoms and arterial blood oxygenation, but not of standard lung function parameters. However, we also performed the multiple-breath nitrogen washout (MBW) test to determine the lung clearance index (LCI) as well as indices of acinar ventilation heterogeneity (S(acin)) and conductive ventilation heterogeneity (S(cond)). At baseline, a distinct abnormality was seen for S(acin) and LCI, while S(cond) was at the upper limit of normal for this subject. S(acin), in particular, was in excess of the S(acin) abnormality corresponding to a 20-pack-year smoking history. Immediately after WLL, S(acin) and S(cond) both fell to within a normal range while LCI also decreased but remained abnormal. The S(acin) decrease was much greater than the S(cond) decrease, which was to be expected after 1 week of smoking cessation at the hospital (smoking was resumed after release from hospital). A follow-up visit 7 weeks after WLL revealed a spectacular improvement on CT scan and improvements in standard lung function. Another follow-up visit 14 weeks after WLL showed further improvements in standard lung function, and both S(acin) and S(cond) remained well within the normal range, and LCI was above the upper limit of normal. We conclude that in this patient, removal of excess surfactant by WLL resulted in a restored ventilation distribution in most of the distal air spaces.

Prevalence and impact on FEV(1) decline of chronic methicillin-resistant Staphylococcus aureus (MRSA) colonization in patients with cystic fibrosis. A single-center, case control study of 165 patients.

BACKGROUND: Risk factors for methicillin-resistant Staphylococcus aureus (MRSA) in Cystic Fibrosis (CF) and the impact on CF disease progression are still under debate. The objectives of this study were to determine clinical variables associated with MRSA colonization and examine impact on FEV(1) evolution in CF patients. METHODS: A retrospective case-control study using the University Hospital of Brussels CF clinic patient registry from 2002 to 2010, comparing clinical variables and decline of FEV(1) of MRSA positive patients with age and sex matched controls, chronically colonized with S. aureus. RESULTS: Thirty of the 165 CF patients, chronically colonized with S. aureus, had cultures positive for MRSA (18.2%). Excluding patients under 4 years, the prevalence became 15.2% (23/151). Chronic colonization (i.e., three or more consecutive positive cultures) was found in 19/151 (12.6%). The MRSA positive group showed a higher proportion of patients with genotype F508del, less pancreas sufficient patients, more bronchiectasis and more frequent hospitalization. The FEV(1) recorded one year prior to, and at the moment of MRSA acquisition, was lower but not significantly different from that obtained in controls (72.9%±26.6 vs 84.3±21.8 and 68.2%±27.1 vs 81.4%±24.3 respectively, p>0.1). However, FEV(1) decline over 2- and 6-year periods, were significantly greater in the chronic MRSA group than in the controls (-5%±5.5 vs -2.5±2.3 over 2 years (p=0.043) and -1.8%±4.6 vs -1.0%±1.9 over a 6-year period (p=0.026)). CONCLUSION: In our center the prevalence of MRSA in CF patients, chronically colonized with S. aureus and over the age of 4 years, was 15.2% (12.6% chronic infection). MRSA colonization was shown to be associated with a genotype F508del, presence of bronchiectasis and hospitalization. Our spirometric data also show that a MRSA episode entails an FEV(1) decline that is almost double that predicted for CF patients who can remain unaffected by MRSA.

Retail sales of inhalation devices in European countries: so much for a global policy.

OBJECTIVE: To evaluate the retail sales of pressurised metered-dose inhalers (pMDIs), dry-powder inhalers (DPIs) and liquids for nebulisation in 16 European countries. METHODS: Retail sales data relating to pMDIs, DPIs and liquids for nebulisation delivering short- and long-acting bronchodilators, corticosteroids and combinations between 2002 and 2008 were obtained from the IMS sales database. The IMS database ensured that wholesalers' stock sales accurately matched that of retail pharmacies and included purchases by panel pharmacies directly from pharmaceutical manufacturers, specialist wholesalers and distribution cooperatives. RESULTS: Mean inhaler retail sales (expressed as percentages of total sales) were 47.5% for pMDIs, 39.5% for DPIs and 13% for nebulisers. The distribution of inhaler sales differed significantly between the countries with pMDI sales greatest in the United Kingdom and Hungary compared to other countries, where DPI sales prevailed. Sales of nebulisation liquids were high in Italy. The pMDI was the most frequently prescribed inhaler for bronchodilators. In contrast, retail sales of DPIs were similar to those of pMDIs for inhaled corticosteroids, and higher in the case of inhalers with combined long-acting ß(2)-agonist and corticosteroid. CONCLUSION: We found a high degree of variability in inhaler prescription between European countries. Differing health policies, costs, health insurance issues, pharmaceutical/commercial aspects and prescribers' and patients' preferences may explain this variation. We suggest a need for more uniform, outcome-led inhaler prescribing practice across Europe to improve the efficacy and cost effectiveness of the treatment of obstructive airways diseases.

Spontaneous soccer-induced pneumomediastinum in a 39-year-old man.

We report on a healthy 39-year-old man who developed spontaneous pneumomediastinum during a soccer game without a traumatic impact. Chest radiography and CT demonstrated the extensive pneumomediastinum and subcutaneous emphysema. Treatment of this rare condition is conservative and prognosis is excellent.

Is there any relationship between nCPAP therapy and signs of sinus hyperpneumatization?

OBJECTIVES: Both nasal continuous positive airway pressure (nCPAP) therapy and nose blowing can generate high pressures in the nose and sinuses. Nose blowing generates higher pressures than nCPAP therapy, but the duration of nCPAP therapy is considerably longer than the duration of nose blowing. Therefore, nCPAP could cause bone deformation. The aim of this study was to document the influence of the pressure generated by nCPAP therapy on the structure and dimensions of the sinuses and on the nose-blowing patterns of the patients. METHODOLOGY: The study included nine patients, who had recently been diagnosed with obstructive sleep apnea syndrome (OSAS) and had not received any previous treatment for OSAS. Before nCPAP therapy was started, they all underwent computer tomography (CT) in the prone position with sequential coronal slices followed by pressure measurements during nose blowing. After the initial measurements, nCPAP therapy commenced. All of the patients were treated with a fixed-pressure device and nasal mask for 6 mo. nCPAP therapy compliance was checked after 6 mo. At the end of the 6 mo treatment with nCPAP, coronal CT scans of the sinuses and pressure measurements during nose blowing were repeated. RESULTS AND CONCLUSION: Although CPAP therapy provides continuous positive pressure for several hours at night, bone structure and sinus dimensions appeared to be unchanged after 6 mo of therapy. However, CPAP therapy seemed to have an effect on the nose-blowing pattern of the patients, with a significant decrease in nose blowing pressure after 6 mo of CPAP treatment.

The impact of concomitant rhinitis on asthma-related quality of life and asthma control.

BACKGROUND: Characterizing the interactions between the upper and lower airways is important for the management of asthma. This study aimed at assessing the specific impact of concomitant rhinitis on asthma-related quality of life (QOL) and asthma control. METHODS: A cross-sectional, observational survey was conducted among 1173 patients with asthma (aged 12-45) recruited by general practitioners and chest physicians. AR was defined by self-reported rhinitis symptoms and previously documented sensitization to inhalant allergens. The primary outcomes were (1) asthma control assessed by the Asthma Control Questionnaire (ACQ) and (2) asthma-specific QOL evaluated through the Mini Asthma Quality of Life Questionnaire (mAQLQ). RESULTS: AR was present in 73.9% of the population with asthma and nonallergic rhinitis (NAR) in 13.6%. AR and NAR were associated with an increased risk of uncontrolled asthma (i.e. ACQ score > 1.5) with adjusted odds ratios (OR) of 2.00 (95% confidence interval [CI]: 1.35-2.97) and 1.77 (95%CI: 1.09-2.89), respectively. Multivariate linear regression analysis showed that AR and NAR had a modest, although significant, negative impact on the global mAQLQ score (beta coefficient: -0.293, standard error [SE]: 0.063 and beta coefficient: -0.221, SE: 0.080, P < 0.001, respectively), even after adjustment for the level of asthma control and demographic characteristics. CONCLUSION: This survey provides direct evidence that AR and NAR are associated with an incremental adverse impact on the disease-specific QOL of patients with asthma and the level of asthma control. Further investigations are required to determine whether appropriate treatment of rhinitis would efficiently reduce asthma morbidity.

Nocardia asiatica visiting Belgium: nocardiosis in a immunocompetent patient.

We report the case of a 57-year-old lady presenting with a Nocardia asiatica infection in Belgium. No predisposing conditions were found except for an underlying asthma. We reviewed the literature and discussed the role of linezolid in the treatment of nocardiosis.

A not so healthy muesli: a case report.

We present a patient with severe anaphylaxis, angioedema, hypotension and shock. The near fatal allergic reaction was caused by eating yogurt with muesli containing pine nuts. The patient developed an acute infero-posterolateral myocardial infarction due to systemic hypotension and shock. Food allergy to pine nut was demonstrated by dosage of specific IgE to pine nut.

Forced vital capacity and forced expiratory volume in six seconds as predictors of reduced total lung capacity.

The present study aims to derive guidelines that identify patients for whom spirometry can reliably predict a reduced total lung capacity (TLC). A total of 12,693 lung function tests were analysed on Caucasian subjects, aged 18-70 yrs. Restriction was defined as a reduced TLC. Lower limits of normal (LLN) for TLC were obtained from the European Respiratory Society recommended reference equations. Reference equations from the National Health and Nutrition Examination Survey III were used for forced vital capacity (FVC) and forced expiratory volume in six seconds (FEV(6)). The performance of FVC and FEV(6) to predict the presence of restriction was studied as follows: 1) using two-by-two (2x2) tables; and 2) by logistic regression analysis. Both analyses were performed in obstructive (defined as forced expiratory volume in one second (FEV(1))/FVC or FEV(1)/FEV(6) 100% pred (males) or >85% pred (females). In obstructive patients, spirometry cannot reliably diagnose a concomitant restrictive defect, but it can rule out restriction for patients with forced vital capacity or forced expiratory volume in six seconds >85% pred (males) or >70% pred (females).