Osteochondroplastic tracheobronchopathy: Four case reports. / Traqueobroncopatía osteocondroplástica: reporte de cuatro casos clínicos.

Introduction: Osteochondroplastic tracheobronchopathy is a rare benign chronic disease of unknown etiology. Bronchoscopy remains the gold standard for diagnosing osteochondroplastic tracheobronchopathy. Its typical findings are described as a cobblestone, rock garden, mountainscape, or stalactite cave appearance. The present work aims to show the main clinical features of this rare pathology. Clinical cases: The clinical data of four middle-aged patients, three men and one woman, were analyzed. The main clinical symptoms were chronic cough, dyspnea, and dysphonia. The patient's preliminary diagnosis was made by computed axial tomography of the chest, confirmed by bronchoscopy and histopathological examination. Treatment included medication for symptoms and, in one case, cryosurgery and argon plasma coagulation. Discussion: Diagnosing osteochondroplastic tracheobronchopathy was not easy, given its uncommon nature and non-specific symptoms often found in other pathologies. No case series articles on this pathology have been published in Peru. Therefore, we used the original articles published in other countries to reference our findings. Conclusion: Osteochondroplastic tracheopathy is a benign disease that typically affects adults. Men are more likely to be affected. Its clinical manifestations are non-specific and frequently of pharyngeal origin, and the cause is not yet defined. Chest computed axial tomography combined with bronchoscopy are the main diagnostic procedures. There is no standard treatment with consistent therapeutic effects.

Introducción: La traqueobroncopatía osteocondroplástica es una rara enfermedad crónica benigna de etiología desconocida. La broncoscopía sigue siendo el estándar de oro para el reconocimiento de traqueopatía osteocondroplástica. Sus hallazgos típicos se describen como un empedrado, un jardín de rocas, una apariencia de paisaje montañoso o de una cueva con estalactitas. El objetivo del presente trabajo es mostrar las principales características clínicas de una patología poco conocida. Casos clínicos: Se analizaron los datos clínicos de cuatro pacientes de mediana edad, tres fueron hombres y una mujer. Los principales síntomas clínicos fueron tos crónica, disnea, disfonía. Los pacientes tuvieron un diagnóstico preliminar mediante tomografía axial computarizada de tórax, confirmado por examen video broncoscópico e histopatológico. El tratamiento incluyó medicamentos para los síntomas y en un solo caso criocirugía y coagulación con argón plasma. Discusión: El diagnóstico de traqueobroncopatía osteocondroplástica no fue sencillo por ser una entidad rara, cuyos síntomas son inespecíficos y muy frecuentes en otras patologías. En Perú no se han publicado artículos de serie de casos sobre esta patología. Por lo tanto, tomamos como referencia artículos originales publicados en otros países para compararlos con nuestros hallazgos. Conclusión: La traqueopatía osteocondroplástica es una enfermedad benigna que predispone a los adultos, los hombres tienen más probabilidades de verse afectados. Sus manifestaciones clínicas son inespecíficas; frecuentemente de origen faríngeo y la causa no está aún definida. La tomografía axial computarizada de tórax combinada con video broncoscopía son los principales procedimientos para el diagnóstico. No existe un estándar de tratamiento con efectos terapéuticos consistentes.

[Risk factors for pulmonary atelectasis in adults with tracheobronchial tuberculosis].

Objective: To investigate the risk factors for pulmonary atelectasis in adults with tracheobronchial tuberculosis(TBTB). Methods: Clinical data of adult patients (≥18 years old) with TBTB from February 2018 to December 2021 in Public Health Clinical Center of Chengdu were retrospectively analyzed. A total of 258 patients were included, with a male to female ratio of 1∶1.43. The median age was 31(24, 48) years. Clinical data including clinical characteristics, previous misdiagnoses/missed diagnoses before admission, pulmonary atelectasis, the time from symptom onset to atelectasis and bronchoscopy, bronchoscopy and interventional treatment were collected according to the inclusion and exclusion criteria. Patients were divided into two groups according to whether they had pulmonary atelectasis. Differences between the two groups were compared. Binary logistic regression was used to analyze the risk factors for pulmonary atelectasis. Results: The prevalence of pulmonary atelectasis was 14.7%, which was most common in the left upper lobe (26.3%). The median time from symptom onset to atelectasis was 130.50(29.75,358.50)d, and the median time from atelectasis to bronchoscopy was 5(3,7)d. The median age, the proportion of misdiagnosis of TBTB before admission, and the time from symptom onset to bronchoscopy in the atelectasis group were higher than those without atelectasis, and the proportion of receiving bronchoscopy examination and interventional therapy previously, and the proportion of pulmonary cavities were lower than those without atelectasis (all P<0.05). The proportions of cicatrices stricture type and lumen occlusion type in the atelectasis group were higher than those without atelectasis, while the proportions of inflammatory infiltration type and ulceration necrosis type were lower than those without atelectasis (all P<0.05). Older age (OR=1.036, 95%CI: 1.012-1.061), previous misdiagnosis(OR=2.759, 95%CI: 1.100-6.922), longer time from symptom onset to bronchoscopy examination (OR=1.002, 95%CI: 1.000-1.005) and cicatrices stricture type (OR=2.989, 95%CI: 1.279-6.985) were independent risk factors for pulmonary atelectasis in adults with TBTB (all P<0.05). Of the patients with atelectasis who underwent bronchoscopy interventional therapy, 86.7% had lung reexpansion or partial reexpansion. Conclusions: The prevalence of pulmonary atelectasis is 14.7% in adult patients with TBTB. The most common site of atelectasis is left upper lobe. The TBTB type of lumen occlusion is complicated by pulmonary atelectasis in 100% of cases. Being older, misdiagnosed as other diseases, longer time from onset of symptoms to bronchoscopy examination, and being the cicatrices stricture type are factors for developing pulmonary atelectasis. Early diagnosis and treatment are needed to reduce the incidence of pulmonary atelectasis and increase the rate of pulmonary reexpansion.

A case of tracheobronchial amyloidosis with emphasis on differential diagnosis.

Tracheobronchial amyloidosis is a manifestation of amyloidosis of the respiratory tract characterized by focal or diffuse deposition of amyloid in the submucosa of the trachea and proximal bronchi. Tracheobronchial amyloidosis is not associated with systemic amyloidosis or pulmonary parenchymal involvement. It affects predominantly men aged over fifty. Depending on the part of the tracheobronchial tree that is affected, stenosis of the airways causes a variety of unspecific symptoms. Diagnosis is reached by means of typical presentation in CT scan followed by bronchoscopy and histopathological confirmation. Tracheobronchial amyloidosis should be borne in mind in the differential diagnosis of patients with chronic cough and/or dyspnea or recurrent respiratory infections.

Endobronchial hamartoma in a young COVID-19 symptomatic woman. Radical endoscopic treatment with a disposable bronchoscope. Case Report.

Pulmonary hamartomas represent the most frequent family of benign lung tumors that typically involve the lung parenchyma and only rarely grow as endobronchial tumors. The elective treatment of endobronchial hamartoma is the bronchoscopic resection, and in those cases in which tumor extension and localization makes it not possible, surgical treatment must be evaluated. Patients with symptomatic COVID-19, hospitalized, frequently undergo a chest CT scan and in some cases, occasional findings may emerge, requiring diagnostic investigations such as bronchoscopy and interventional pulmonology procedures. Therefore, in such a delicate pathological condition, such as COVID-19, the need to perform bronchoscopy and interventional pulmonology procedures, minimizing the risk of viral transmission and ensuring necessary assistance, represents a great challenge for pulmonologists. In this article authors describe, for the first time in literature, a rare case of endobronchial hamartoma, radically resected using a single use bronchoscope, in a young female patient hospitalized for symptomatic COVID-19.

Left Main Bronchus Stenosis Lesion, Neutrophil Count, and Platelet Count Are Predictors of Post-Tuberculosis Bronchomalacia.

BACKGROUND Post-tuberculosis bronchomalacia (PTBM) is one of the main conditions occurring in patients after tracheobronchial tuberculosis (TBTB), and is also associated with the recurrence of symptoms. The present study aimed to investigate the predictors of PTBM in patients who had been undergoing appropriate TB treatment. MATERIAL AND METHODS Clinical data of 104 patients with symptomatic airway stenosis after TBTB between January 01, 2019 and June 31, 2020 were recorded and analyzed. The association between baseline clinical characteristics, laboratory results, and PTBM was calculated with logistical regression. The time from onset of bronchoscopic intervention was examined by Kaplan-Meier estimates; differences between the 2 groups were tested by the log-rank test. RESULTS Fifty-seven patients (54.81%) had PTBM. In the multivariate logistical analysis, the left main bronchus stenosis lesion (odds ratio [OR]=3.763), neutrophil (NEUT) count (OR=1.527), and platelet (PLT) (OR=1.010) count were predictors of PTBM. During follow-up, patients with BM had a significantly longer duration from onset of bronchoscopic intervention than patients without BM (hazard ratio=2.412, P<0.0001). Further, all patients needing long-term bronchoscopic intervention therapy were subsequently identified as having PTBM. Additionally, blood PLT counts were significantly decreased to normal levels in the non-BM group (P<0.05), but not in the BM group (P>0.05). CONCLUSIONS PTBM is most likely to occur in the left main bronchus. The inflammatory and immune responses associated with NEUT and PLT may represent therapeutic targets of PTBM. Our study is the first to report that decreased blood PLT count has the potential to monitor the treatment response.

Development of polymer-based nanoparticles for zileuton delivery to the lung: PMeOx and PMeOzi surface chemistry reduces interactions with mucins.

In this paper, two amphiphilic graft copolymers were synthesized by grafting polylactic acid (PLA) as hydrophobic chain and poly(2-methyl-2-oxazoline) (PMeOx) or poly(2-methyl-2-oxazine) (PMeOzi) as hydrophilic chain, respectively, to a backbone of α,ß-poly(N-2-hydroxyethyl)-D,L-aspartamide (PHEA). These original graft copolymers were used to prepare nanoparticles delivering Zileuton in inhalation therapy. Among various tested methods, direct nanoprecipitation proved to be the best technique to prepare nanoparticles with the smallest dimensions, the narrowest dimensional distribution and a spherical shape. To overcome the size limitations for administration by inhalation, the nano-into-micro strategy was applied, encapsulating the nanoparticles in water-soluble mannitol-based microparticles by spray-drying. This process has allowed to produce spherical microparticles with the proper size for optimal lung deposition, and, once in contact with fluids mimicking the lung district, able to dissolve and release non-aggregated nanoparticles, potentially able to spread through the mucus, releasing about 70% of the drug payload in 24 h.

Long-term prognostic factors of clinical success after interventional bronchoscopy in patients with scarring central airway stenosis.

BACKGROUND: Scarring central airway stenosis (SCAS) is a potentially life-threatening condition with debilitating symptoms. Interventional bronchoscopy is increasingly used to relieve symptoms in patients with SCAS, but recurrent stenosis is frequently observed. Little data exist on the long-term prognosis of interventional bronchoscopy for SCAS. We aimed to assess the prognostic factors of bronchoscopic interventions in patients with SCAS to optimize treatment. METHODS: This was a retrospective study that enrolled 119 consecutive patients with SCAS from January 2010 to April 2019 at our institution. Long-term clinical success was defined as airway stenosis < 50%, no limitation of physical activity, and a stable condition for > 12 months after the last interventional procedure. We compared patients' demographics, airway stenosis characteristics, and interventional procedures between the successful and unsuccessful groups, and identified significant predictors of long-term outcome with univariate and multivariate logistic regression. RESULTS: A total of 119 patients with 577 therapeutic bronchoscopies were included. Seventy-five (63%) patients were considered to have long-term clinical success. Older age, male gender, smoking, elevated C-reactive protein level, subglottic stenosis, stent or T-tube implantation, previous interventional treatment, and multiple procedures per year were potentially associated with unsuccessful long-term outcomes in the univariate analysis. Current smoker status (odds ratio [OR] 5.70, 95% confidence interval [CI] 1.35-24.17, P = 0.018), subglottic stenosis (OR 4.35, 95% CI 1.31-14.46, P = 0.017), and stent implantation (OR 4.96, 95% CI 1.33-18.48, P = 0.017) were associated with decreased odds of long-term success in the multivariate logistic regression analysis. Of note, there was no significant difference in odds of success between former smokers and nonsmokers. CONCLUSIONS: Current smoker status, subglottic stenosis, and stent implantation are independent factors associated with reduced long-term efficacy of interventional bronchoscopy for SCAS. Smoking cessation should be encouraged to improve the outcome of therapeutic bronchoscopy.

Usefulness of autofluorescence bronchoscopy in early diagnosis of airway complications after lung transplantation.

Despite the promising results achieved so far in long-term survival after lung transplantation (LuTx), airway complications (ACs) still arise in the post-operative period. Early diagnosis and prompt treatment of ACs play a critical role in preventing their onset. Specifically, large bronchi ischemia has been recognized as a triggering factor for ACs. Autofluorescence bronchoscopy, which was first introduced for early cancer diagnosis, displays ischemic mucosae as red spots, while normal vascularized mucosae appear in green. The aim of this study is to investigate whether a significant correlation exists between ACs and the red/green (RG) ratio detected on scheduled autofluorescence bronchoscopy up to 1 year after LuTx. This prospective, observational, single-center cohort study initially considered patients who underwent LuTx between July 2014 and February 2016. All patients underwent concomitant white-light and autofluorescence bronchoscopy at baseline (immediately after LuTx), on POD7, POD14, POD21, POD28, POD45, 3 months, 6 months, and 1 year after LuTx. An autofluorescence image of the first bronchial carina distal to the anastomosis was captured and analyzed using histograms for red and green pixels; the R/G ratio was then recorded. Potential ACs were classified according according to the presence of a white-light following the MDS (macroscopic aspect, diameter and suture) criteria. The authors assessed the association between the R/G ratio and the ACs occurrence using a generalized estimating equations model. Thirty-one patients met the inclusion criteria and were therefore selected. Out of a total of 53 bronchial anastomoses, 8 developed complications (late bronchial stenosis), with an average onset time of 201 days after LuTx. ACs showed a similar baseline covariate value when compared to anastomoses that involved no complication. Generalized estimating equations regression indicated a clear association over time between the R/G ratio and the rise of complications (p = 0.023). The authors observed a significant correlation between post-anastomotic stenosis and the delayed decrease of the R/G ratio. Preliminary outcomes suggest that autofluorescence bronchoscopy may be an effective and manageable diagnostic tool, proving complementary to other instruments for early diagnosis of ACs after LuTx. Further research is needed to confirm and detail preliminary findings.

Tracheobronchial amyloidosis in primary Sjögren syndrome: A case report.

RATIONALE: Tracheobronchial amyloidosis (TBA) associated with Sjögren syndrome is very rare. Here, we describe a case with this phenomenon, in order to better understand the condition. PATIENT CONCERNS: A 52-year-old woman presented after 6 months of coughing, sputum, and dyspnea. Chest computed tomography revealed thickened bronchial walls, which were irregular on the left side the trachea. She had a history of dry eye and dry mouth of at least 3 years' duration. DIAGNOSES: Sjögren syndrome was diagnosed based on her symptoms, ophthalmological and parotid examination, and immunological and autoantibody tests. The diagnosis of TBA was confirmed by Congo red staining of a tracheal biopsy. INTERVENTIONS: The patient was given glucocorticoids without any other immunosuppressants. OUTCOMES: The symptoms improved after 6 months. LESSONS: TBA associated with Sjögren syndrome is a rare condition. TBA is characterized by amyloid deposition to the trachea in the absence of systemic amyloidosis. Diagnosis requires tissue biopsy with demonstration of amyloid deposition.

Airway-centered Interstitial Fibrosis Involving Smooth Muscle Hyperplasia with Severe Pulmonary Hypertension.

In the 2013 updated classification of the American Thoracic Society/European Respiratory Society, airway-centered interstitial fibrosis (ACIF) is included as a bronchiolocentric pattern of interstitial pneumonia (IP) among idiopathic IPs. We encountered a case of severe pulmonary hypertension (PH) with chronic IP. The patient initially presented with shortness of breath and often lost consciousness due to PH, and seven years after his first visit, he ultimately died. An autopsy revealed ACIF and usual IP. In particular, the ACIF comprised non-atypical smooth muscle hyperplasia, and pulmonary hypertensive vascular degeneration was detected. This case may represent a new pathological feature of ACIF.

Bronchiolitis and Bronchiolar Disorders.

Bronchioles are noncartilaginous small airways with internal diameter of 2 mm or less, located from approximately the eighth generation of purely air conducting airways (membranous bronchioles) down to the terminal bronchioles (the smallest airways without alveoli) and respiratory bronchioles (which communicate directly with alveolar ducts and are in the range of 0.5 mm or less in diameter). Bronchiolar injury, inflammation, and fibrosis may occur in myriad disorders including connective tissue diseases, inflammatory bowel diseases, lung transplant allograft rejection, graft versus host disease in allogeneic stem cell recipients, neuroendocrine cell hyperplasia, infections, drug toxicity (e.g., penicillamine, busulfan), inhalation injury (e.g., cigarette smoke, nylon flock, mineral dusts, hard metals, Sauropus androgynous); idiopathic, common variable immunodeficiency disorder, and a host of other disorders or insults. The spectrum of bronchiolar disorders is wide, ranging from asymptomatic to fatal obliterative bronchiolitis. In this review, we discuss the salient clinical, radiographic, and histological features of these diverse bronchiolar disorders, and discuss a management approach.

Drastic healing process after pembrolizumab monotherapy in a case of advanced squamous cell carcinoma with severe bronchial stenosis observed over a two-year period using continuous bronchoscopy: A case report.

Programmed cell death-1 immune checkpoint inhibitor (ICI) antibody has proven to be effective in advanced non-small cell lung cancer (NSCLC) patients positive for programmed cell death-1 ligand-1. However, there are currently no reports which evaluate drug efficacy by continuous bronchoscopic observation. A 75-year-old man with complete right atelectasis was diagnosed with squamous cell carcinoma (SCC) of the right lower lobe (tumor proportion score: TPS 90%, cT4N3M0, stage 3C). For first-line chemotherapy, carboplatin and nab-paclitaxel were effective for the primary lesion and the right lung atelectasis improved. However, due to repeated febrile neutropenia with pneumonia, treatment was modified to pembrolizumab monotherapy. Bronchoscopic rebiopsy prior to second-line treatment revealed high TPS, with a severe stenosis in the right main bronchus. After three courses of pembrolizumab, the right main bronchus opened completely, and no signs of malignancy were observed. Bronchoscopic narrow-band and autofluorescence imaging also confirmed a complete endobronchial response. Subsequent bronchoscopic observation two years after the initial diagnosis showed a complete and continued response to treatment. ICIs can result in a drastic bronchoscopic response. In this case, the healing process was notable with minimal scarring, and resulted in continued locally bronchoscopic and complete pathological response to treatment compared to previous cytotoxic chemotherapy.

Feasibility study of ultrasound video bronchoscopy for sampling endobronchial lesions.

INTRODUCTION: Endobronchial biopsy using forceps is generally performed with a standard bronchoscope, while a needle is used with an endobronchial ultrasound (EBUS) bronchoscope. An EBUS video bronchoscope, such as the EB-530US instrument (Fujifilm, Tokyo, Japan), provides good visibility and may, therefore, enable both forceps biopsies and needle aspirations to be performed. OBJECTIVES: The aim of this study was to investigate the feasibility of performing forceps biopsies and needle aspirations using the EBUS video bronchoscope for diagnosing endobronchial lesions. METHODS: Seventy patients with suspected endobronchial lesions based on a computed tomography scan were recruited and underwent forceps biopsy using the EB-530US EBUS bronchoscope. If the result of a rapid on-site cytological evaluation was negative, an additional needle aspiration of the same lesion or other target lesions was performed. The primary outcome was the completion rate of bronchoscopy, using only the EBUS bronchoscope without removal. RESULTS: In the 70 patients, forceps biopsies and/or needle aspirations using the EBUS video bronchoscope were performed and completed without removing the EBUS bronchoscope in 67 patients (95.7%). The remaining three patients required the removal of the EBUS bronchoscope from the trachea (blood clot obstruction of the working channel in one patient and change to a thin bronchoscope to sample an EBUS bronchoscope-inaccessible lesion in two others). The EBUS bronchoscope provided diagnostic material in 66 patients (94.3%). One case each of bleeding and pneumonia were observed (1.4%). CONCLUSION: Both forceps biopsy and needle aspiration are feasible using a Fujifilm EB-530US EBUS video bronchoscope.

Overview and Challenges of Bronchiolar Disorders.

Bronchiolar abnormalities are common and can occur in conditions that affect either the large airways or the more distal parenchyma. In this review, we focus on the diagnosis and management of primary bronchiolar disorders, or conditions in which bronchiolitis is the predominant pathologic process, including constrictive bronchiolitis, follicular bronchiolitis, acute bronchiolitis, respiratory bronchiolitis, and diffuse panbronchiolitis. Due to the nature of abnormalities in the small airway, clinical and physiological changes in bronchiolitis can be subtle, making diagnosis challenging. Primary bronchiolar disorders frequently present with progressive dyspnea and cough that can be out of proportion to imaging and physiologic studies. Pulmonary function tests may be normal, impaired in an obstructive, restrictive, or mixed pattern, or have an isolated decrease in diffusion capacity. High-resolution computed tomography scan is an important diagnostic tool that may demonstrate one or more of the following three patterns: 1) solid centrilobular nodules, often with linear branching opacities (i.e., "tree-in-bud" pattern); 2) ill-defined ground glass centrilobular nodules; and 3) mosaic attenuation on inspiratory images that is accentuated on expiratory images, consistent with geographic air trapping. Bronchiolitis is often missed on standard transbronchial lung biopsies, as the areas of small airway involvement can be patchy. Fortunately, many patients can be diagnosed with a combination of clinical suspicion, inspiratory and expiratory high-resolution computed tomography scans, and pulmonary function testing. Joint consultation of clinicians with both radiologists and pathologists (in cases where histopathology is pursued) is critical to appropriately assess the clinical-radiographic-pathologic context in each individual patient.