Clinical, Radiological, and Microbiologic Characteristics of Patients with Noncystic Fibrosis Bronchiectasis in a Tertiary Center at Jordan.

BACKGROUND: Only a small number of the investigations that were carried out in the Middle East attempted to characterize patients with NCFB. In order to characterize patients with NCFB, as well as their etiologies, microbiological profiles, and outcomes, we therefore carried out this investigation. METHODS: This retrospective cohort study was carried out at the Jordan University Hospital (JUH), a tertiary facility located in Amman, Jordan. Non-cystic Fibrosis Bronchiectasis (NCFB) was defined as an HRCT scan typical for bronchiectasis along with a negative sweat chloride test to rule out cystic fibrosis. Patients' data were collected by the use of Electronic Medical Records (EMR) at our institution. Frequent exacerbation was defined as more than 2 exacerbations in 1 year of the onset of the diagnosis. RESULTS: A total of 79 patients were included, and 54.4% of them were female. The mean and standard deviation of the patient's age was 48.61 ± 19.62. The etiologies of bronchiectasis were evident in 79.7% of the sample. Asthma, Chronic Obstructive Pulmonary Diseases (COPD), and Kartagener syndrome were the most prevalent etiologies, accounting for related illnesses in 21.8%, 21.5%, and 13.9% of the patients, respectively. The most frequent bacteria cultured in our cohort were Pseudomonas and Candida Species. Moreover, 43 patients of the study cohort were frequent exacerbators, and 5 patients died. CONCLUSION: Our study supports the need to identify several bronchiectasis phenotypes linked to various causes. These findings provide information to clinicians for the early detection and treatment of bronchiectasis in Jordan.

Clustered Cystic Changes in Long-Term Follow-Up Thin-Section Computed Tomographic Findings in Fibrotic Nonspecific Interstitial Pneumonia.

Objectives: The purpose of this study was to retrospectively assess cystic changes in findings on follow-up CT scans of patients with fibrotic nonspecific interstitial pneumonia (NSIP). Methods: The initial and last high-resolution CT scans of 58 patients with pathologically proven fibrotic NSIP were evaluated retrospectively. The median follow-up periods were 48 months (range, 12-183 months). The pattern, extent, and distribution of abnormal CT findings were compared with findings in the same region on previous and subsequent CT scans with a focus on cystic lesions. Results: Cystic lesions in a cluster were shown in 16 patients (28%) with fibrotic NSIP on the last CT scans. Focal clustered cysts were found in 5 cases and diffuse clustered cysts were seen in 11 cases. Focal clustered cysts mimicked honeycombing seen in usual interstitial pneumonia (UIP). Diffuse cysts were uniform in size in 7 of the 11 cases. Traction bronchiectasis in a cluster was seen in 3 of the 7 cases. The clustered cystic changes on CT during the course of NSIP mainly consisted of traction bronchiectasis and bronchiolectasis. Conclusions: Long-standing NSIP did not form honeycombing. The clustered cysts in patients with fibrotic NSIP were mainly remodeling of bronchiectasis.

Pulmonary nocardiosis following nodular bronchiectatic Mycobacterium avium complex pulmonary disease in an immunocompetent patient.

A woman in her 70s with a history of nodular bronchiectatic Mycobacterium avium complex pulmonary disease (MAC-PD) presented with an exacerbated productive cough and worsening findings on chest imaging. Although repeated sputum culture tests were negative for acid-fast bacilli and only revealed normal respiratory flora, a bronchoscopy identified Nocardia sp. Consequently, she was diagnosed with pulmonary nocardiosis and was successfully treated with levofloxacin. It is known that pulmonary nocardiosis can manifest in immunocompetent individuals with bronchiectasis. For cases of refractory nodular bronchiectatic MAC-PD, it is vital to consider bronchoscopy to identify potential co-infections, such as Nocardia.

Differential clinical and CT imaging features of pneumonic-type primary pulmonary lymphoma and pneumonia: a retrospective multicentre observational study.

INTRODUCTION: Pneumonic-type primary pulmonary lymphoma (PPL) is often misdiagnosed as pneumonia in clinical practice. However, this disease requires different treatments, which calls for a correct diagnosis. MATERIALS AND METHODS: A total of 227 patients with pneumonic-type PPL (n=72) and pneumonia (n=155) from 7 institutions were retrospectively enrolled between January 2017 and January 2022. Clinical features (age, sex, cough, sputum, fever, haemoptysis, chest pain, smoking, weight loss and laboratory results (haemoglobin, white blood cell count, C reactive protein level and erythrocyte sedimentation rate)) and CT imaging characteristics (air bronchogram, bronchiectasis, halo sign, pleural traction, pleural effusion, lymphadenopathy, lesion maximum diameter and CT attenuation value) were analysed. Receiver operating characteristic curve analysis was performed for model construction based on independent predictors in identifying pneumonic-type PPL. In addition, we used a calibration curve and decision curve analysis to estimate the diagnostic efficiency of the model. RESULTS: The patients with pneumonia showed a higher prevalence of sputum, fever, leucocytosis and elevation of C reactive protein level than those with pneumonic-type PPL (p=0.002, p<0.001, p=0.011 and p<0.001, respectively). Bronchiectasis, halo sign and higher CT attenuation value were more frequently present in pneumonic-type PPL than in pneumonia (all p<0.001). Pleural effusion was more commonly observed in patients with pneumonia than those with pneumonic-type PPL (p<0.001). Also, sputum, fever, elevation of C reactive protein level, halo sign, bronchiectasis, pleural effusion and CT attenuation value were the independent predictors of the presence of pneumonic-type PPL with an area under the curve value of 0.908 (95% CI, 0.863 to 0.942). CONCLUSION: Pneumonic-type PPL and pneumonia have different clinical and imaging features. These differential features could be beneficial in guiding early diagnosis and subsequent initiation of therapy.

Lady Windermere syndrome with haemoptysis: suspected pulmonary aspergilloma and MAC pulmonary disease.

We present a case of a woman in her 70s, with a history of Mycobacterium avium complex (MAC) pulmonary disease, pectus excavatum, s-shaped thoracolumbar scoliosis, bronchiectasis of the right middle lobe, lingula of left upper lobe, and malnutrition with low body mass index of 14 kg/m2, who presented to the hospital due to worsening shortness of breath and small volume haemoptysis over 2 weeks. The patient was diagnosed with pulmonary MAC infection for the first time in 1999 without known history of pulmonary disease. Later on, it was complicated by massive haemoptysis requiring bronchial artery embolisation; however, she was unable to complete an oral antibiotic regimen due to gastrointestinal adverse reactions. Chest CT identified a newly found mass in the left upper lobe bulla, consistent with a radiological finding of aspergilloma. We present a rare symptoms constellation, described as 'Lady Windermere syndrome' and chronic untreated MAC infection progressing from reticulonodular changes to fibrocavitary lung disease and suspected aspergilloma formation.

Association of blood total immunoglobulin E and eosinophils with radiological features of bronchiectasis.

BACKGROUND: Our study aimed to investigate whether serum total IgE and blood eosinophils were associated with radiological features of bronchiectasis in a Chinese cohort. METHODS: We retrospectively enrolled bronchiectasis patients who visited Peking University Third Hospital from Jan 1st, 2012 to Oct 7th, 2021. The clinical, laboratory and chest CT characteristics were analyzed in association with serum total IgE level and blood eosinophil count. RESULTS: A total of 125 bronchiectasis patients were enrolled, with 50.4% (63/125) female, and a mean age of 62.4 ± 14.1 years. The median serum total IgE level and blood eosinophil count were 47.7 (19.8, 123.0) KU/L and 140 (90, 230) cells/µl, respectively. In patients with a higher than normal (normal range, 0-60 KU/L) total IgE (43.2%, n = 54), more lobes were involved [4 (3, 5) vs. 3 (2, 4), p = 0.008], and mucus plugs were more common (25.9% vs. 9.9%, p =0.017) on HRCT, as compared to those with a normal level of total IgE. The higher IgE group was more likely to have bilateral involvement (p = 0.059), and had numerically higher Smith and Bhalla scores, but the differences were not statistically significant. In patients with an eosinophil count ≥ 150 cells/µl (49.6%, n = 62), the number of lobes involved was greater [4 (3, 5) vs. 3 (2, 4), p = 0.015], and the Smith and Bhalla scores were higher [9 (5, 12) vs. 6 (3, 9), p = 0.009, 7 (5, 11) vs. 5 (3, 9), p = 0.036]. The Smith score was correlated positively with the eosinophil count (r = 0.207, p = 0.020). Fractional exhaled nitric oxide (FeNO) was correlated with total IgE (r = 0.404, p = 0.001) and eosinophil count (r = 0.310, p = 0.014). CONCLUSIONS: Our study demonstrated that serum total IgE and the blood eosinophil count were associated with the radiological extent and severity of bronchiectasis, necessitating further investigation on the role of T2 inflammation in structural abnormalities of this heterogeneous disease.

Safety and efficacy of bronchial thermoplasty in Australia 5 years post-procedure.

BACKGROUND AND OBJECTIVE: Outside clinical trials, there is limited long-term data following bronchial thermoplasty (BT). In a cohort of real-world severe asthmatics in an era of biological therapy, we sought to evaluate the safety and efficacy of BT 5 years post-treatment. METHODS: Every patient treated with BT at two Australian tertiary centres were recalled at 5 years, and evaluated by interview and record review, Asthma Control Questionnaire (ACQ), spirometry and high-resolution CT Chest. CT scans were interpreted using the modified Reiff and BRICS CT scoring systems for bronchiectasis. RESULTS: Fifty-one patients were evaluated. At baseline, this cohort had a mean age of 59.0 ± 11.8 years, mean ACQ of 3.0 ± 1.0, mean FEV1 of 55.5 ± 18.8% predicted, and 53% were receiving maintenance oral steroids in addition to triple inhaler therapy. At 5 years, there was a sustained improvement in ACQ scores to 1.8 ± 1.0 (p < 0.001). Steroid requiring exacerbation frequency was reduced from 3.8 ± 3.6 to 1.0 ± 1.6 exacerbations per annum (p < 0.001). 44% of patients had been weaned off oral steroids. No change in spirometry was observed. CT scanning identified minor degrees of localized radiological bronchiectasis in 23/47 patients with the modified Reiff score increasing from 0.6 ± 2.6 at baseline to 1.3 ± 2.5 (p < 0.001). However, no patients exhibited clinical features of bronchiectasis, such as recurrent bacterial infection. CONCLUSION: Sustained clinical benefit from BT at 5 years was demonstrated in this cohort of very severe asthmatics. Mild, localized radiological bronchiectasis was identified in a portion of patients without clinical features of bronchiectasis.

Chronic Suppurative Lung Disease in Children: A Case Based Approach.

Bronchiectasis is a pathologic state of conducting airways manifested radiographically by evidence of bronchial dilation and clinically by chronic productive cough. Considered an "orphan disease" for long, it remains a major contributor to morbidity and mortality in both developed and underdeveloped countries. With the advances in the medical field accompanied by widespread access to vaccines and antibiotics, improved health services and better access to nutrition, the incidences of bronchiectasis have markedly decreased, particularly in developed countries. This review summarizes the current knowledge pertaining to the clinical definition, etiology, clinical approach and management related to pediatric bronchiectasis.

Etiology and clinical features of children with bronchiectasis in China: A 10-year multicenter retrospective study.

INTRODUCTION: The current study aims to investigate the etiology spectrum and the clinical characteristics of bronchiectasis in Chinese children. METHODS: The study is designed as a multicenter retrospective study. 193 cases were enrolled in 13 centers in China between 2008 and 2017. The inclusive cases must meet the clinical as well as the HRCT criteria. Only if both two radiologists confirmed the diagnosis, the case could be enrolled. The cases that could not provide clinical and imageology data were excluded. The data were entered into the specialized system and then analyzed. RESULTS: One hundred sixty-nine cases (87%) were found to have the underlying etiology. Post-infective (46%), primary immunodeficiency (14%), and PCD (13%) were the common causes. All cases came from 28 provinces in Mainland China. The median age of symptom onset was 5.8 (2.0, 8.9) years. The median age of diagnosis was 8.4 (4.5, 11.6) years. The main symptoms were cough, sputum expectoration, and fever during the exacerbation. Nineteen percent of patients suffered from limited exercise tolerance. Clubbing was found in 17% of cases. Nearly 30% of patients presented growth limitations. On the HRCT findings, 126 cases had diffused bronchiectasis, and bilateral involvement was found in 94 cases. The lower lobes and right middle lobes were most commonly involved. Approximately 30% of cultures of sputum and bronchoalveolar lavage were positive. CONCLUSION: A majority of cases could be found the underlying etiology. Post-infective, primary immunodeficiency, and PCD were the most common causes. Some clinical figures might indicate a specific etiology.