Correlating Reiff scores with clinical, functional, and prognostic factors: characterizing noncystic fibrosis bronchiectasis severity: validation from a nationwide multicenter study in Taiwan.

BACKGROUND: Our study aimed to confirm a simplified radiological scoring system, derived from a modified Reiff score, to evaluate its relationship with clinical symptoms and predictive outcomes in Taiwanese patients with noncystic fibrosis bronchiectasis (NCFB). METHODS: This extensive multicenter retrospective study, performed in Taiwan, concentrated on patients diagnosed with NCFB verified through high-resolution computed tomography (HRCT) scans. We not only compared the clinical features of various types of bronchiectasis (cylindrical, varicose, and cystic). Furthermore, we established relationships between the severity of clinical factors, including symptom scores, pulmonary function, pseudomonas aeruginosa colonization, exacerbation and admission rates, and HRCT parameters using modified Reiff scores. RESULTS: Data from 2,753 patients were classified based on HRCT patterns (cylindrical, varicose, and cystic) and severity, assessed by modified Reiff scores (mild, moderate, and severe). With increasing HRCT severity, a significant correlation was found with decreased forced expiratory volume in the first second (FEV1) (p < 0.001), heightened clinical symptoms (p < 0.001), elevated pathogen colonization (pseudomonas aeruginosa) (p < 0.001), and an increased annual hospitalization rate (p < 0.001). In the following multivariate analysis, elderly age, pseudomonas aeruginosa pneumonia, and hospitalizations per year emerged as the only independent predictors of mortality. CONCLUSION: Based on our large cohort study, the simplified CT scoring system (Reiff score) can serve as a useful adjunct to clinical factors in predicting disease severity and prognosis among Taiwanese patients with NCFB.

Phenotype and Clinicoradiological Differences in Multifocal and Focal Bronchiectasis.

Introduction: Bronchiectasis is a chronic progressive respiratory disease characterized by permanent dilatation of the bronchi. It is a complex condition with numerous different etiologies, co-morbidities, and a heterogeneous presentation. As we know, there is a lack of studies that describe the differences and compare the characteristics between focal and multifocal bronchiectasis. The aim of this study is to identify differences in clinical characteristics presentation, severity or distribution in focal and multifocal bronchiectasis, and prognostic implications. Methods: 126 patients with computed tomography (CT)-verified bronchiectasis were enrolled. Baseline characteristics that included age, sex, smoking history, and respiratory symptoms were recorded, with special attention paid to hemoptysis appearance, body mass index, and comorbidities. The type of bronchiectasis determined by CT scan and the modified Reiff scores indicating radiological severity were recorded. Patients were divided in two groups (I is focal and II is multifocal). Results: There were no statistically significant differences in age, smoking status, comorbidity, and BMI between the two groups. Multifocality was associated with a significantly higher proportion of females (p = 0.014), the rate of hemoptysis (p = 0.023), and the number of hospitalizations, but not of exacerbations and prevalence of immunodeficiency (p = 0.049). Significantly, a high number of subjects with multifocality had bronchiectasis of moderate severity, and post-infective and asthma-associated phenotypes were the dominant in this group. Unexpectedly, the cystic and varicose radiological phenotype (which need more time to develop) were more dominant in the focal group. The cylindrical phenotype was equally observed in the multifocal and focal groups. Conclusions: Our study suggests that multifocality is not related to age, number of exacerbations, or radiological phenotype, but it seems to be associated with the clinical post-infective phenotype, immunodeficiency, frequent hospitalizations, and severity. Thus, the presence of multifocal bronchiectasis may act as a biomarker of severity and poor outcomes in these patients.

Bronchiectasis After Solid-Organ Transplant: A Retrospective Single Center Study.

OBJECTIVES: Bronchiectasis is characterized by abnormal, persistent, and irreversible enlargement of the bronchi. Many etiological factors have been described, but there are limited data on the development of bronchiectasis after organ transplantation. Our study is the first to study evaluate the frequency of bronchiectasis in heart and liver transplants as well as kidney transplants. Our aim is to analyze the frequency of bronchiectasis development after solid-organ transplant and the characteristics of the cases and to evaluate potential relationships. MATERIALS AND METHODS: We retrospectively analyzed data of patients who underwent solid-organ transplant at the Baskent University Faculty of Medicine Hospital through the hospital electronic information system. Demographic, clinical, and laboratory data and thoracic computed tomography scans were evaluated. RESULTS: The study included 468 patients (151 females/317 males). Kidney transplant was performed in 61.5% (n = 207), heart transplant in 20.3% (n = 95), and liver transplant in 18.2% (n = 85) of patients. Development of bronchiectasis was detected in only 13 patients (2.7%). We determined a 13.64-fold risk of developing bronchiectasis in patients with chronic obstructive pulmonary disease and 10.08-fold risk in patients with pneumonia by multivariate regression analyzes, in which all possible risk factors for the development of bronchiectasis after transplant were evaluated. CONCLUSIONS: The pathophysiology of transplantassociated bronchiectasis has not yet been clarified. Underlying diseases, recurrent pulmonary infections, and potential effects from immunosuppressive drugs may contribute to the pathogenesis of bronchiectasis. Further prospective studies are needed to include long-term health outcomes in transplant patients with and without bronchiectasis.

Pectoralis Muscle Area as a Predictor of Mortality in Patients Hospitalized with Bronchiectasis Exacerbation.

INTRODUCTION: Data on factors related to mortality in patients with bronchiectasis exacerbation are insufficient. Computed tomography (CT) can measure the pectoralis muscle area (PMA) and is a useful tool to diagnose sarcopenia. This study aimed to evaluate whether PMA can predict mortality in patients with bronchiectasis exacerbation. METHODS: Patients hospitalized due to bronchiectasis exacerbation at a single center were retrospectively divided into survivors and non-survivors based on 1-year mortality. Thereafter, a comparison of the clinical and radiologic characteristics was conducted between the two groups. RESULTS: A total of 66 (14%) patients died at 1 year. In the multivariate analysis, age, BMI <18.4 kg/m2, sex-specific PMA quartile, ≥3 exacerbations in the previous year, serum albumin <3.5 g/dL, cystic bronchiectasis, tuberculosis-destroyed lung, and diabetes mellitus were independent predictors for the 1-year mortality in patients hospitalized with bronchiectasis exacerbation. A lower PMA was associated with a lower overall survival rate in the survival analysis according to sex-specific quartiles of PMA. PMA had the highest area under the curve during assessment of prognostic performance in predicting the 1-year mortality. The lowest sex-specific PMA quartile group exhibited higher disease severity than the highest quartile group. CONCLUSIONS: CT-derived PMA was an independent predictor of 1-year mortality in patients hospitalized with bronchiectasis exacerbation. Patients with lower PMA exhibited higher disease severity. These findings suggest that PMA might be a useful marker for providing additional information regarding prognosis of patients with bronchiectasis exacerbation.

Latent class analysis of chest CT abnormalities to define subphenotypes in patients with MPO-ANCA-positive microscopic polyangiitis.

BACKGROUND: Patients with microscopic polyangiitis (MPA) and positive myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) may present with various abnormalities in chest computed tomography (CT). This study aimed to identify subphenotypes using latent class analysis (LCA) and to explore the relationship between the subphenotypes and clinical patterns, as well as compare the clinical characteristics of these subphenotypes in patients with MPO-ANCA-positive MPA (MPO-MPA). METHODS: The study identified subphenotypes using LCA based on chest CT findings in 178 patients with MPO-MPA and pulmonary involvement from June 2014 to August 2022. RESULTS: LCA identified 27 participants (15.2%) in class 1, 43 (24.1%) in class 2, 35 (19.7%) in class 3, and 73 (41.0%) in class 4. Class 1 was characterized by prominent inflammatory exudation, class 2 by fibrosis and architectural distortion, class 3 by predominantly bronchiectasis, and class 4 by lesions mixed with inflammation and fibrosis. Class 1 had the highest level of extrapulmonary disease activity, with 77.8% of patients experiencing diffuse alveolar hemorrhage. Class 2 had the lowest level of extrapulmonary disease activity, with 41.9% of patients showing usual interstitial pneumonia. Class 3 patients were more likely to have complications involving the ear, nose, and throat, as well as pulmonary infections before treatment, and they exhibited the best outcomes. The characteristics and outcomes of class 4 were intermediate among the four classes. CONCLUSIONS: These findings suggest that bronchiectasis may represent a unique pattern of pulmonary involvement in MPO-MPA, highlighting the importance of screening for bronchiectasis in MPO-MPA and identifying optimal management strategies.

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.