Abstract: Rationale: Bronchiectasis is a chronic debilitating disease with few evidence-based long-term treatments.Objectives: A randomized controlled trial assessing the efficacy of nebulized gentamicin therapy over 1 year in patients with non–cystic fibrosis bronchiectasis.Methods: Sixty-five patients were randomized to either twice-daily nebulized gentamicin, 80 mg, or nebulized 0.9% saline, for 12 months. All were reviewed at three-monthly intervals during treatment and at 3 months' follow-up.Measurements and Main Results: At each review the following were assessed: quantitative and qualitative sputum bacteriology; sputum purulence and 24-hour volume; FEV1, FVC, and forced expiratory flow, midexpiratory phase; exercise capacity; Leicester Cough Questionnaire and St. George's Respiratory Questionnaire; and exacerbation frequency. Fifty-seven patients completed the study. At the end of 12 months' treatment, compared with the saline group, in the gentamicin group there was reduced sputum bacterial density with 30....

Abstract: A 5-years multicenter prospective study on 201 patients with common variable immunodeficiencies and 101 patients with X-linked agammaglobulinemia over a cumulative follow-up period of 1,365 patient-years was conducted to identify prognostic markers and risk factors for associated clinical co-morbidities, the effects of long-term immunoglobulin treatment and the IgG trough level to be maintained over time required to minimise infection risk. Overall, 21% of the patients with common variable immunodeficiencies and 24% of patients with X-linked agammaglobulinemia remained infection free during the study. A reduction of pneumonia episodes has been observed after initiation of Ig replacement. During the observation time, pneumonia incidence remained low and constant over time. Patients with pneumonia did not have significant lower IgG trough levels than patients without pneumonia, with the exception of patients whose IgG trough levels were persistently <400 mg/dL. In X-linked agammaglobulinemia, the only co-morbidity risk factor identified for pneumonia by the final multivariable model was the presence of bronchiectasis. In common variable immunodeficiencies, our data allowed us to identify a clinical phenotype characterised by a high pneumonia risk: patients with low IgG and IgA levels at diagnosis; patients who had IgA level <7 mg/dL and who had bronchiectasis. The effect of therapy with immunoglobulins at replacement dosage for non-infectious co-morbidities (autoimmunity, lymphocytic hyperplasia and enteropathy) remains to be established. A unique general protective trough IgG level in antibody deficiency patients will remain undefined because of the major role played by the progression of lung disease in X-linked agammaglobulinemia and in a subset of patients with common variable immunodeficiencies.

Abstract: Rationale: Cystic fibrosis (CF) is characterized by bronchoalveolar neutrophilia and submucosal lymphocytosis. We hypothesized that Th17 lymphocytes are part of this submucosal infiltrate.Objectives: Quantification and phenotyping of the lymphocytic infiltrate in the bronchial submucosa of patients with CF (n = 53, of which 20 were newly diagnosed), non-CF bronchiectasis (n = 17), and healthy control subjects (n = 13).Methods: We measured IL-17 levels in bronchoalveolar lavage and CD4+, CD8+, and IL-17+ cell counts in endobronchial biopsies. Correlations were made with infection status and other inflammatory markers. Potential cellular sources of IL-17 were determined by double staining.Measurements and Main Results: IL-17+ cell counts (median [interquartile range] cells/mm2) were significantly higher in patients with established CF (205 [115–551]) and non-CF bronchiectasis (245 [183–436]) than in control subjects (53 [12–82]) (P < 0.01 for both). Patients with newly diagnosed CF had intermediate counts (...

Abstract: The outcome and cause of death of each lung disease directly associated with rheumatoid arthritis (RA-LD) have been poorly investigated. A retrospective study was conducted of 144 patients with RA-LD, in whom the median follow-up period after the initial visit for a respiratory examination was 4.5 yrs. A total of 57 patients were identified with usual interstitial pneumonia (UIP), 31 with bronchiectasis, 16 with nonspecific interstitial pneumonia (NSIP), 11 with bronchiolitis, five with organising pneumonia (OP), five with diffuse alveolar damage (DAD) and 19 with combined disease. The 5-yr survival rates were 36.6% in the UIP group, 87.1% in the bronchiectasis group, 93.8% in the NSIP group, 88.9% in the bronchiolitis group, 60.0% in the OP group and 20.0% in the DAD group. Survival of patients with DAD was worse than that of patients with UIP. Overall, survival of patients with UIP was worse than that of patients with bronchiectasis, NSIP or bronchiolitis. Of the 144 patients, 71 (49.3%) died, of whom 58 (81.7%) died due to respiratory lesions. Of patients with RA-LD, patients with DAD experienced the highest mortality, and the survival of patients with UIP was worse than that of patients with NSIP.

Abstract: Rationale High resolution computed tomography (HRCT) is a more sensitive tool for detecting early cystic fibrosis (CF) lung disease than either spirometry or plain radiography, but its relationship to other measures of lung function has not been established in young children. Objectives 1) To assess whether the lung clearance index (LCI) derived from multiple breath inert-gas washout (MBW) is as effective as HRCT in identifying pulmonary abnormalities; and 2) explore the relationships between abnormalities detected by HRCT and by spirometry, plethysmography and MBW (collectively, LFTs) in young children with CF. Methods Children with CF underwent LFTs and volumetric HRCT on the same day. Healthy age-matched controls underwent identical LFTs without HRCT. Scans were anonymised, and scored using the Brody-II CT scoring system, to assess for presence and extent of bronchiectasis, airway wall thickening, mucus plugging, and parenchymal opacities. Results Assessments were undertaken in 60 children with CF (mean (SD) 7.8 (1.3) years) and 54 healthy controls (7.9 (1.2) y). Among children with CF, 84% (47/56) had abnormal LCI, 58% (27/47) abnormal plethysmographic lung volumes (FRCpleth or RV), 35% (21/60) abnormal sRaw and 47% (28/60) abnormal spirometry (FEV1 or FEF25–75); whereas HRCT scans were abnormal in 85% (51/60): median total Brody-II score: 9.5% (range 0–51%). Total CT score correlated more strongly with LCI (Spearman correlation=0.77) than with spirometry (R=−0.43) or any other marker of lung function. Of the nine children with normal LCI, five had abnormalities on HRCT, whereas five children with normal HRCT had raised LCI. Conclusions These results suggest that while LCI and HRCT have similar sensitivity to detect CF lung disease, complimentary information may be gained in individual patients.

Abstract: Allergic bronchopulmonary aspergillosis (ABPA) is a Th2 hypersensitivity lung disease in response to Aspergillus fumigatus that affects asthmatic and cystic fibrosis (CF) patients. Sensitization to A. fumigatus is common in both atopic asthmatic and CF patients, yet only 1-2% of asthmatic and 7–9% of CF patients develop ABPA. ABPA is characterized by wheezing and pulmonary infiltrates which may lead to pulmonary fibrosis and/or bronchiectasis. The inflammatory response is characterized by Th2 responses to Aspergillus allergens, increased serum IgE and eosinophilia. A number of genetic risks have recently been identified in the development of ABPA. These include HLA-DR and HLA-DQ, IL-4 receptor alpha chain (IL-4RA) polymorphisms, IL-10-1082GA promoter polymorphisms, surfactant protein A2 (SP-A2) polymorphisms, and cystic fibrosis transmembrane conductance regulator gene (CFTR) mutations. The studies indicate that ABPA patients are genetically at risk to develop skewed and heightened Th2 responses to A. fumigatus antigens. These genetic risk studies and their consequences of elevated biologic markers may aid in identifying asthmatic and CF patients who are at risk to the development of ABPA. Furthermore, these studies suggest that immune modulation with medications such as anti-IgE, anti-IL-4 and/or IL-13 monoclonal antibodies may be helpful in the treatment of ABPA.

Abstract: BACKGROUND: Abnormalities, including bronchiectasis, that are detectable on high-resolution computed tomography (HRCT) have been associated with severe asthma. Bronchiectasis is associated with the diagnosis of allergic bronchopulmonary aspergillosis (ABPA), which also occurs in patients with severe asthma. We sought to determine the frequency and pattern of HRCT abnormality and the relationship with Aspergillus fumigatus sensitization in one severe asthma population. METHODS: We examined our database of patients attending a supraregional severe asthma service (Manchester, UK). Clinical, physiological and immunological characteristics were compared between those with HRCT evidence of airway disease (specifically bronchiectasis) and those with no radiological abnormality. RESULTS: Of 133 patients analysed, 111 (83.4%) had an abnormal HRCT with bronchial wall thickening (41.3%), bronchiectasis (35.3%), air trapping (20.3%) and bronchial dilatation (16.5%) occurring most frequently. Radiological evidence of airway disease was associated with more obstructive spirometry (postbronchodilator FEV?/FVC ratio 73.2%vs 64.8% [difference -8.5%, 95% CI -16.9 to -0.1, P = 0.048]). A. fumigatus sensitization was associated with a 2.01 increased hazard ratio of bronchiectasis (95% CI 1.26 to 3.22, P = 0.005), and more obstructive spirometry (postbronchodilator FEV?/FVC ratio 57.6 vs 70.3 [difference -12.8, 95% CI -19.8 to -5.7, P = 0.001]). Patients with A. fumigatus sensitization had variable clinical and radiological characteristics that frequently did not conform to the conventional diagnostic criteria for ABPA. CONCLUSION: Patients with severe asthma frequently have radiological abnormalities on HRCT. Sensitization to A. fumigatus is associated with bronchiectasis and greater airflow obstruction, even when diagnostic criteria for ABPA are not met.

Abstract: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is being recognized with increasing frequency. Diagnostic and treatment information is limited. A systematic review is presented, focusing on patient demographics, clinical presentation, diagnosis, treatment options, and outcomes. A systematic electronic literature search was conducted for adult DIPNECH cases reported in the English literature during the past 6 years. Twenty-four DIPNECH cases were identified. Another case from our institution is contributed. Women represent 92% (23 of 25). Mean age at diagnosis was 58 years (range, 36-76 yr). Most were nonsmokers (16 of 24). Symptoms included cough (71%), dyspnea (63%), and wheezing (25%) occurring days to years before diagnosis. Pulmonary function testing showed obstructive ventilatory disease in 54%. Lung nodules were seen in 15 patients (63%), ground-glass attenuation in 7 patients (29%), and bronchiectasis in 5 patients (21%). Histological confirmation required surgical lung biopsy for 88%; however, transbronchial biopsies alone were diagnostic in three patients. Treatments strategies included systemic and inhaled corticosteroids, bronchodilators, and lung resection. Available follow-up data in 17 patients showed 6 clinically improved, 7 who remained stable, and 4 clinically deteriorated. The majority of patients presenting with DIPNECH are middle-aged females with symptoms of cough and dyspnea; obstructive abnormalities on pulmonary function testing; and radiographic imaging showing pulmonary nodules, ground-glass attenuation, and bronchiectasis. In general, the clinical course remains stable; however, progression to respiratory failure does occur. Long-term follow-up and treatment remains incomplete. Establishment of a national multicenter DIPNECH registry would allow formulation of optimal evidence-based guidelines for management of these patients.

Abstract: • Consensus recommendations for managing chronic suppurative lung disease (CSLD) and bronchiectasis, based on systematic reviews, were developed for Australian and New Zealand children and adults during a multidisciplinary workshop.

Abstract: Objective:LadyWindermere syndromeis a well-knownbut poorly understood femalepredominant phenotypeof isolatedright middlelobeand lingular bronchiectasis associated with non-tuberculous mycobacterial (NTM) infection. Despite lengthy multidrug antibiotic treatment, the presence of damaged parenchymal tissue leads to symptomatic disease recurrence, often with resistant organisms. The use of surgical resection as an adjunct to medical therapy may alter this cycle, although little is known about the use of thoracoscopic lung resection in this patient population. Methods: This is a retrospective review of a prospectively collected database of patients with pulmonary NTM disease from July 2004 to December 2009. All patients had focal bronchiectasis of the right middle lobe and lingula, treated with targeted antimicrobial therapy for several months prior to resection. Results: A total of 134 patients underwent 172 operations, with 38 patients having staged bilateral resections. The cohort was predominately female (96%) and Caucasian (95%), with a mean age of 59 years (range 34—81 years). Using a thoracoscopic approach in all patients, 102 middle lobectomies and 70 lingulectomies were performed. Conversion to open thoracotomy occurred in five cases (3%). Secondary procedures were performed in 20 cases (12%). There was no operative mortality. Postoperative morbidity was noted following 12 operations (7%), primarily consisting of prolonged air leak. The mean length of stay was 3.3 days (range 1—15 days). Conclusions: Although medical therapy remains the primary treatment modality for patients with pulmonary NTM disease, the selective use of pulmonary resection may reduce the incidence of symptomatic disease recurrence. The addition of thoracoscopic resection to treatment regimens for patients with Lady Windermere syndrome can be accomplished with minimal morbidity and mortality. # 2011 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Abstract: Purpose of review Cystic fibrosis (CF) is a complex genetic disease affecting many organs, although 85% of the mortality is a result of lung disease. The natural history of the lung disease consists of early and persistent infection, an exaggerated inflammatory response, structural airway changes (i.e. bronchiectasis), and progressive airways obstruction, ultimately resulting in respiratory failure. As airways disease worsens, there is an increased likelihood of respiratory complications, such as pneumothorax, that may be serious. This review describes our current understanding of the pathogenesis of pneumothorax in CF and its treatment. Recent findings The CF Foundation Pulmonary Therapies Committee recently published their recommendations for the treatment of hemoptysis and pneumothorax. As insufficient data exist from which a systematic review of the literature could be used to develop guidelines, the recommendations were derived from a formalized expert panel consensus process. Summary We now have recommendations on specific care of the patient with CF who has a pneumothorax.

Abstract: Background: Bronchiectasis continues to be one of the major causes of morbidity and mortality in developing countries, with a probably underestimated higher prevalence than in developed countries. Objective: To assess the clinical profile of adult patients with bronchiectasis. Methods: We retrospectively reviewed the clinical, radiologic, and physiologic findings of 304 patients with bronchiectasis confirmed by high-resolution computed tomography. Results: Mean age of participants (45.7% males, 54.3% females) was 56 ± 25 years and 65.8% of them were lifetime non-smokers. Most common identified causes of bronchiectasis were childhood disease (22.7%), tuberculosis (15.5%), and pneumonia (11.5%). The predominant symptoms were productive cough (83.6%), dyspnea (72%), and hemoptysis (21.1%). The most common findings on chest examination were crackles (71.1%) and rhonchi (28.3%). Types of bronchiectasis were cylindrical in 47%, varicose in 9.9%, cystic in 45.1%, and multiple types in 24.3%. Involvement was multilobar in 75.3% and bilateral in 62.5%. Of 274 patients, 20.8% displayed normal pulmonary function test results, whereas 47.4%, 8% and 23.7% showed obstructive, restrictive, and mixed pattern, respectively. Patients with cystic disease had a higher frequency of hemoptysis (42%) and a greater degree of functional impairment, compared to other types. Conclusion: In patients with bronchiectasis from southern Turkey, generally presenting with recurrent productive cough, hemoptysis, dyspnea, and persistent bibasilar rales, the etiology remains mainly idiopathic. Post-infectious bronchial destruction is one of the major identified underlying pathological processes. The clinical picture and the deterioration of the pulmonary function test might be more severe in patients with cystic type bronchiectasis.

Abstract: Allergic bronchopulmonary aspergillosis (ABPA) is the best-known allergic manifestation of Aspergillus-related hypersensitivity pulmonary disorders. Most patients present with poorly controlled asthma, and the diagnosis can be made on the basis of a combination of clinical, immunological, and radiological findings. The chest radiographic findings are generally nonspecific, although the manifestations of mucoid impaction of the bronchi suggest a diagnosis of ABPA. High-resolution CT scan (HRCT) of the chest has replaced bronchography as the initial investigation of choice in ABPA. HRCT of the chest can be normal in almost one-third of the patients, and at this stage it is referred to as serologic ABPA (ABPA-S). The importance of central bronchiectasis (CB) as a specific finding in ABPA is debatable, as almost 40% of the lobes are involved by peripheral bronchiectasis. High-attenuation mucus (HAM), encountered in 20% of patients with ABPA, is pathognomonic of ABPA. ABPA should be classified based on the presence or absence of HAM as ABPA-S (mild), ABPA-CB (moderate), and ABPA-CB-HAM (severe), as this classification not only reflects immunological severity but also predicts the risk of recurrent relapses.

Abstract: While allergic bronchopulmonary aspergillosis (ABPA) is well recognized as a fungal complication of asthma, severe asthma with fungal sensitization (SAFS) is not. In ABPA the total immunoglobulin E (IgE) is usually >1,000 IU/mL, whereas in SAFS it is <1,000 IU/mL, and either skin prick tests or fungus-specific IgE tests are positive. ABPA may present with any severity of asthma, and occasionally with no asthma or cystic fibrosis, the other common underlying disease. SAFS is a problem in patients with poorly controlled asthma and occasionally presents in the intensive care unit (ICU). Production of mucous plugs and coughing paroxysms is more common in ABPA. Certain underlying genetic defects seem to underpin these remarkable phenotypic differences. From a management perspective both ABPA and SAFS respond to both high doses of corticosteroids and oral antifungal agents, with ∼60% response rate in both ABPA and SAFS with itraconazole. In 50% of patients itraconazole boosts inhaled corticosteroid exposure, sometimes leading to cushingoid features. Second-line therapy data are scant, but we have shown that 70 to 80% of patients who tolerate either voriconazole or posaconazole also respond. Other useful therapies include nebulized hypertonic saline to aid expectoration of thick sputum and long-term azithromycin for its anti-inflammatory effect on the airways. Omaluzimab is useful in some patients with SAFS and occasionally in ABPA. Complications of ABPA include bronchiectasis, typically central in distribution, and chronic pulmonary aspergillosis. Most patients with ABPA and SAFS can be stabilized for long periods with inhaled corticosteroids and itraconazole or another antifungal agent. Novel immunotherapies are on the horizon.

Abstract: Background Bronchopulmonary hygiene physical therapy is a form of chest physical therapy including chest percussion and postural drainage to remove lung secretions. These are applied commonly to patients with both acute and chronic airway diseases. Despite controversies in the literature regarding its efficacy, it remains in use in a variety of clinical settings. The various forms of this therapy are labour intensive and need to be evaluated. Objectives The objective of this review was to assess the effects of bronchial hygiene physical therapy in people with chronic obstructive pulmonary disease and bronchiectasis. Search strategy We searched the Cochrane Airways Group trials register and reference lists of articles up to January 2007. We also wrote to study authors. Selection criteria Randomised trials in which postural drainage, chest percussion, vibration, chest shaking, directed coughing or forced exhalation technique was compared to other drainage or breathing techniques, placebo or no treatment. Data collection and analysis Two reviewers applied the inclusion and exclusion criteria on masked publications independently. They assessed the trial quality independently. Only data from the first arm of crossover trials were included. Main results The seven included trials involved six comparisons and a total of 126 people. The trials were small and not generally of high quality. The results could not be combined as trials addressed different patient groups and outcomes. In most comparisons, bronchial hygiene physical therapy produced no significant effects on pulmonary function, apart from clearing sputum in chronic obstructive pulmonary disease and in bronchiectasis. An update search carried out in January 2007 did not identify any new studies for inclusion. Authors' conclusions There is not enough evidence to support or refute the use of bronchial hygiene physical therapy in people with chronic obstructive pulmonary disease and bronchiectasis.

Abstract: Background There is minimal literature available on the long-term outcome of pediatric non-cystic fibrosis (CF) bronchiectasis Aim To document 5-year outcomes of children with chest computerized tomography (CT) scan diagnosed bronchiectasis from a tertiary New Zealand (NZ) respiratory clinic Methods Review of a clinical database identified 91 children Demographics, clinical data, lung function, chest X-ray (CXR), sputum, presumed etiology, admission data, and the NZ deprivation index (NZDep) were collected Univariate and multivariate regression were used to correlate clinical findings with lung function data and CXR scores using the Brasfield Scoring System Results Of the 91 children, 53 (59%) were Pacific Island, 22 (24%) Maori, 14 (15%) European, and 2 (2%) Other The median follow-up period was 67 years (range 50–153 years) and median age at diagnosis was 73 years (range 11 months–16 years) Lung function data (n = 64) showed a mean decline of −16% predicted/year In 30 children lung function declined (mean FEV1 −44% predicted/year, range 1–17%), remained stable in 13 and improved in 21 children (mean FEV1 of +3% predicted/year, range 1–15%) Reduced lung function was associated with male gender, chronic Haemophilus influenzae infection, longevity of disease, and Maori and Pacific Island ethnicity There was a significant correlation with FEV1 and CXR score at beginning (n = 47, r = 045, P = 0001) and end (n = 26, r = 059, P = 0002) of the follow-up period The only variable consistently related to CXR score was chronic Haemophilus influenzae infection occurring in 27 (30%) (r2 = 052, P = <00001) Only four children were chronically infected with Pseudomonas species Six children died Conclusion In our experience despite management in a tertiary multidisciplinary bronchiectasis clinic, progression of lung disease continues in a group of children and young adults Pediatr Pulmonol 2011; 46:131–138 © 2011 Wiley-Liss, Inc

Abstract: Aims: The purpose of this study was to determine whether volatile organic compounds specific to Pseudomonas aeruginosa could be detected in clinical sputum specimens. Methods and Results: Patients were recruited from specialist bronchiectasis and cystic fibrosis clinics. The gold standard for diagnosing Ps. aeruginosa infection was a positive sputum culture. About 72 sputum headspace samples taken from patients at risk of or known to have prior Ps. aeruginosa infection were analysed by solid phase micro-extraction mass spectrometry. 2-nonanone was a marker in Ps. aeruginosa in sputum headspace gas with sensitivity of 72% and specificity of 88%. A combination of volatile compounds, a sputum library of 17 compounds with 2-nonanone, increased sensitivity in the detection of Ps. aeruginosa to 91% with specificity of 88%. Conclusions: In contrast to the 48-hour turnaround for classical microbiological culture, these results were available within 1–2 h. These data demonstrate the potential for rapid and accurate diagnosis of Ps. aeruginosa infection from sputum samples. Significance and impact of the study: 2-Nonanone is a compound requiring further study in the exhaled breath as it may improve diagnostic of Ps. aeruginosa infection when combined with other reported volatile markers.

Abstract: Introduction Chronic obstructive pulmonary disease (COPD) patients with mucus hypersecretion tend to demonstrate increased frequency of infective exacerbations and a steeper slope of decline in lung function. Enhanced mucociliary clearance with high-frequency chest wall oscillation (HFCWO) devices previously used in cystic fibrosis and bronchiectasis patients may offer the opportunity for community-based, self-managed therapy to improve quality of life and lung function.

Abstract: OBJECTIVE. The purpose of this study was to evaluate the association between pulmonary hypertension estimated with CT and outcome among patients with bronchiectasis. MATERIALS AND METHODS. The cases of 91 patients with bronchiectasis were studied. CT signs of pulmonary hypertension examined were main pulmonary artery diameter, right and left main pulmonary artery diameters, and the ratio between the diameters of the main pulmonary artery and the ascending aorta. CT scans were scored for extent of bronchiectasis and presence of bronchial dilatation, bronchial wall thickening, mucous plugging, mosaicism, and emphysema. Univariate, bivariate, and multivariate Cox proportional hazards models were used to test the influence of CT signs on mortality. RESULTS. Average right and left main pulmonary artery diameter was the best predictor of mortality (hazard ratio, 1.24; 95% CI, 1.13–1.35; p < 0.0001) and was associated with outcome independent of CT signs of bronchiectasis. CONCLUSION. Pulmonary hypertension, ref...

Abstract: Objective The aim of this study was to evaluate pulmonary complications in patients with primary antibody deficiency (X-linked agammaglobulinaemia [XLA] and common variable immunodeficiency [CVID]). Methods Thirty patients over six years of age regularly followed in a reference out-patient clinic on primary immunodeficiency were studied. All of them have been treated with intravenous immunoglobulin (IVIG) replacement therapy. Pulmonary complications were evaluated analysing clinical data (medical records review), lung function test (spirometry) and pulmonary imaging (chest computed tomography [CCT]). Results Patients with normal CCT (N=14) and those with abnormal CCT (N=16) have shown no differences regarding the age at onset of symptoms, age of diagnosis, and duration of IVIG treatment. The mean number of pneumonia episodes before IVIG replacement was significantly higher among patients with abnormal CCT (4 vs 7 episodes, p=0.008). CCT abnormalities observed in 16 patients were: bronchiectasis (12/16); peribronchial thickening (3/16); air trapping (5/16); lung volume reduction (4/16); atelectasis (2/16), follicular bronchiolitis and ground-glass abnormality (2/16) and parenchyma nodule (1/16). Lung function tests showed ventilatory disturbance in 18/30: obstructive pattern in 38.8%, restrictive pattern in 44.4%, and mix pattern in 16.7%. There were no significant differences in lung function between those with and without CCT abnormalities. Negative significant correlations were observed between lung function and number of episodes of pneumonia. Chronic persistent cough was associated with a reduction in lung function. Conclusions Pulmonary complications are not rare in patients with antibody deficiencies and they must be monitored.

Abstract: Sarcoidosis is a systemic granulomatous disease of undetermined etiology characterized by a variable clinical presentation and disease course. Although clinical granulomatous inflammation may occur within any organ system, more than 90% of sarcoidosis patients have lung disease. Sarcoidosis is considered an interstitial lung disease that is frequently characterized by restrictive physiologic dysfunction on pulmonary function tests. However, sarcoidosis also involves the airways (large and small), causing obstructive airways disease. It is one of a few interstitial lung diseases that affects the entire length of the respiratory tract - from the nose to the terminal bronchioles - and causes a broad spectrum of airways dysfunction. This article examines airway dysfunction in sarcoidosis. The anatomical structure of the airways is the organizational framework for our discussion. We discuss sarcoidosis involving the nose, sinuses, nasal passages, larynx, trachea, bronchi and small airways. Common complications of airways disease, such as, atelectasis, fibrosis, bullous leions, bronchiectasis, cavitary lesions and mycetomas, are also reviewed.

Abstract: Background: Diagnosis of lower respiratory disease requires collection of airway samples to confirm the etiology of disease. Bronchoscopic evaluation is commonly performed in dogs but less information is available in cats. Hypothesis: The presence and number of bronchoscopic abnormalities visualized during bronchoscopic evaluation of cats with lower respiratory disease will correlate with the type of disease and total and differential cell counts in bronchoalveolar lavage (BAL) fluid. Animals: Forty-eight cats prospectively evaluated by a single bronchoscopist. Methods: Bronchoscopy was performed during clinical evaluation of cats presenting with cough, respiratory distress, or both. Cats were evaluated for airway hyperemia, stenosis, or collapse, mucus accumulation, bronchiectasis, and epithelial irregularities. Cats were placed into groups of bronchitis/“asthma,” pneumonia, or neoplasia based on BAL findings, histopathology, and response to appropriate medical therapy. Summation of bronchial abnormalities and total and differential cell counts were compared among groups. Results: Endobronchial abnormalities were common in cats with feline bronchitis/asthma, pneumonia, and neoplasia and no differentiating features were found. Excessive mucus accumulation was common (83%), followed by stenosis of bronchial openings and nodular epithelial irregularities (56%), airway hyperemia (54%), airway collapse (48%), and bronchiectasis (27%). Total bronchoscopic score and total cell count did not differ among groups, although differential cell counts were significantly different. A weak correlation (R2= 0.16, P= .006) between age and total bronchoscopic score was noted. Conclusions and Clinical Relevance: Bronchoscopic abnormalities are common in cats with lower respiratory tract disease, and visualization of the airways provides additional nonspecific clinical information in cats.

Abstract: To the Editor: Pulmonary nocardiosis is a rare disorder that mainly affects immunocompromised patients. Several risk factors have been identified, such as corticosteroid therapy, chronic obstructive pulmonary disease (COPD), cystic fibrosis and bronchiectasis. Diagnosis of nocardiosis is difficult as bacteriological culture can be problematic. However, if observations are atypical, clinicians should consider Nocardia in immunocompetent patients, particularly when the patient is also suffering from COPD. Herein, we ask whether COPD alone is a risk factor for pulmonary nocardiosis. We report the case of a 71-yr-old female smoker suffering from COPD who had not received inhaled or oral (bolus or long-term) corticosteroid therapy. She had suffered from bronchiectasis after pulmonary tuberculosis 60 yrs previously. She presented to the hospital with an exacerbated chronic cough that had lasted several months and chronic fever, with no weight loss. Prior to admission, amoxicillin followed by spiramycin therapy was not effective. Laboratory evaluation showed hyperleukocytosis (14,130 cells per mm3, with 10,753 polynuclear neutrophils per mm3) and C-reactive protein 75 mg·L−1 (normal range <5 mg·L−1), with no renal failure or hepatic biological abnormalities. Her gamma globulin level was normal (11.7 g·L−1). Aspergillosis serology and the tuberculin intradermal reaction were negative; sputum examination was sterile, without Mycobacterium or Aspergillus . Chest radiography showed linear opacity of the right upper lobe with bronchiectasis and excavated opacity in the right upper lobe. A computed tomography scan of the chest revealed bronchiectasis with a thickened lining and cystic dilatation of the right upper lobe, as well as diffuse centrolobular micronodules in all lobes of the right lung (fig. 1). …

Abstract: Human infection with Lophomonas blattarum is extremely rare. The clinical manifestations of 45 cases of bronchopulmonary L. blattarum infection occurring in China, including one case diagnosed and treated at the authors' hospital, are described. The most common manifestations included fever, cough with expectoration, chest stuffiness or shortness of breath, bronchiectasis and pulmonary abscess. A course of metronidazole treatment lasting 14-38 days was effective in controlling the disease. The diagnosis of L. blattarum infection should be based on one or more of bronchoscopic brush smear, bronchoscopic biopsy smear and bronchoalveolar lavage.

Abstract: Background Radiologically evident bronchiectasis is seen in 30% to 50% of patients with advanced chronic obstructive pulmonary disease (COPD). As COPD is now becoming more common around the world, bronchiectasis is as well.