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Frontiers in Immunology 2023Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of disorders characterized by necrotizing inflammation of small- and medium-sized blood...
BACKGROUND
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of disorders characterized by necrotizing inflammation of small- and medium-sized blood vessels and the presence of circulating ANCA. Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic ANCA-associated vasculitis, characterized by peripheral eosinophilia, neuropathy, palpable purpuras or petechiae, renal and cardiac involvement, sinusitis, asthma, and transient pulmonary infiltrates. Middle lobe syndrome (MLS) is defined as recurrent or chronic atelectasis of the right middle lobe of the lung, and it is a potential complication of asthma.
CASE PRESENTATION
Herein, we describe a case of MLS in a 51-year-old woman, never-smoker, affected by EGPA, presenting exclusively with leukocytosis and elevated concentrations of acute-phase proteins, without any respiratory symptom, cough, or hemoptysis. Chest computed tomography (CT) imaging documented complete atelectasis of the middle lobe, together with complete obstruction of lobar bronchial branch origin. Fiberoptic bronchoscopy (FOB) revealed complete stenosis of the middle lobar bronchus origin, thus confirming the diagnosis of MLS, along with distal left main bronchus stenosis. Bronchoalveolar lavage (BAL) did not detect any infection. Bronchial biopsies included plasma cells, neutrophil infiltrates, only isolated eosinophils, and no granulomas, providing the hypothesis of vasculitic acute involvement less likely. First-line agents directed towards optimizing pulmonary function (mucolytics, bronchodilators, and antibiotic course) were therefore employed. However, the patient did not respond to conservative treatment; hence, endoscopic management of airway obstruction was performed, with chest CT documenting resolution of middle lobe atelectasis.
CONCLUSION
To the best of our knowledge, this is the first detailed description of MLS in EGPA completely resolved through FOB. Identification of MLS in EGPA appears essential as prognosis, longitudinal management, and treatment options may differ from other pulmonary involvement in AAV patients.
Topics: Female; Humans; Middle Aged; Middle Lobe Syndrome; Antibodies, Antineutrophil Cytoplasmic; Churg-Strauss Syndrome; Constriction, Pathologic; Granulomatosis with Polyangiitis; Leukocyte Disorders; Pulmonary Atelectasis; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Asthma
PubMed: 37638004
DOI: 10.3389/fimmu.2023.1222431 -
Revista Da Sociedade Brasileira de... 2023
Topics: Humans; Paracoccidioidomycosis; Constriction, Pathologic
PubMed: 37792840
DOI: 10.1590/0037-8682-0343-2023 -
The American Journal of Case Reports Dec 2023BACKGROUND An aortopulmonary window (APW) is an uncommon congenital defect of the septation between the ascending aorta and pulmonary trunk. The combination of APW and...
BACKGROUND An aortopulmonary window (APW) is an uncommon congenital defect of the septation between the ascending aorta and pulmonary trunk. The combination of APW and interrupted aortic arch (IAA) is even rarer, with the hallmark characteristics of high peri-operative mortality and postoperative obstruction of the aortic arch, pulmonary artery, and left main bronchus. These complications often need re-interventions. CASE REPORT We present 2 cases with diagnoses of APW and IAA that were treated with single-stage repair. Case 1: A male 32-week premature newborn (weight 1789 g) had APW type I and IAA type A. He had severe postoperative aortic arch obstruction on postoperative day 1, and we re-intervened promptly. He was still asymptomatic after 6 years. Case 2: A male term neonate had APW type III and IAA type A. He had left vocal cord paralysis and left bronchial compression postoperatively. We applied prolonged noninvasive respiratory supports. The complications resolved without re-intervention on postoperative day 66. Progressive arch stenosis at anastomosis after operation required close follow-up with echocardiography. CONCLUSIONS These 2 reports highlight the feasibility of single-stage surgical repair while addressing 2 challenges: (1) Recurrent arch stenosis: Lower body weight and direct end-to-side anastomosis without patch augmentation could be risk factors for re-intervention. (2) Bronchial compression: Presentation of the second reported case implied that bronchial compression may not warrant immediate re-intervention unless there is complete obstruction, persistent atelectasis, or recurrent infection. Further studies on long-term outcomes of different surgical procedure would help us to clarify the proper way to avoid re-intervention.
Topics: Humans; Infant, Newborn; Male; Aorta; Aorta, Thoracic; Aortopulmonary Septal Defect; Constriction, Pathologic; Treatment Outcome
PubMed: 38124359
DOI: 10.12659/AJCR.942193 -
Canadian Respiratory Journal 2023Endobronchial lipoma (EL) is a rare benign tumor characterized by tracheobronchial smooth-surfaced mass, often resulting in bronchial obstruction without standard...
Endobronchial lipoma (EL) is a rare benign tumor characterized by tracheobronchial smooth-surfaced mass, often resulting in bronchial obstruction without standard guidelines for management. This study seeks to clarify the clinical features and interventions of EL, aiming to improve its diagnosis and outcomes. A retrospective review was conducted on 28516 outpatients treated between January 2015 and December 2019 at the Department of Respiratory and Critical Care Medicine of the Second Affiliated Hospital of Air Force Medical University to collect patients diagnosed with EL. Their clinical, bronchoscopic, chest imaging, and histopathological features along with management were analyzed. Among the patients reviewed, nine were histopathologically diagnosed with EL, comprising seven males and two females. All EL patients exhibited noticeable symptoms, including cough (in eight patients), dyspnea (in six patients), fever (in three patients), expectoration (in two patients), chest pain (in two patients), hemoptysis (in one patient), and fatigue (in one patient). Chest CT abnormalities included endobronchial mass (in four patients), inflammatory exudation (in three patients), atelectasis (in three patients), and infiltration or consolidation (in two patients). In three patients, imaging showed fat density, directly leading to the diagnosis of EL. The EL lesions were distributed with six in the right lung and three in the left lung, all located within the first three subdivisions of the tracheobronchial tree. Treatment approaches varied, with one patient undergoing combined bronchoscopic resection and surgery. The remaining patients received bronchoscopic intervention such as electrosurgical snare resection, argon plasma coagulation (APC), cryotherapy, and holmium laser. Histopathological analysis confirmed the EL diagnosis. Finally, the mass removal restored bronchus patency. Taken together, EL symptoms lack specificity, necessitating reliance on histopathology for EL accurate diagnosis. Bronchoscopic interventions emerge as the preferred option for EL management, surpassing surgical approaches.
Topics: Male; Female; Humans; Constriction, Pathologic; Bronchial Diseases; Bronchi; Lung Neoplasms; Lipoma; Bronchoscopy; Bronchial Neoplasms
PubMed: 38170103
DOI: 10.1155/2023/2799436 -
Journal of Medical Case Reports Aug 2023Granulomatosis with polyangiitis and relapsing polychondritis are rare, multisystemic and potentially life-threatening connective tissue diseases. We present two cases... (Review)
Review
BACKGROUND
Granulomatosis with polyangiitis and relapsing polychondritis are rare, multisystemic and potentially life-threatening connective tissue diseases. We present two cases of severe endobronchial obstruction in the aforementioned conditions and discuss difficulties with detection and treatment. Despite differing underlying pathophysiologies, endobronchial disease is a less frequently reported but serious complication of both conditions.
CASE PRESENTATION
Case 1, a 31-year-old South Asian woman with relapsing polychondritis, required partial tracheal resection and reconstruction in combination with immunosuppressive therapy to achieve respiratory recovery following collapse of her right main bronchus and a stricture in her left main bronchus. Case 2, a 22-year-old Caucasian male with granulomatosis with polyangiitis, underwent surgical resection of an endobronchial growth causing occlusion of his right main bronchus. Although his respiratory status was initially stabilised with increased immunosuppression, he continues to have disease progression in spite of this.
CONCLUSIONS
Our cases highlight the importance of a multidisciplinary approach combining immunosuppression with supportive care and judicious use of surgical interventions in select cases. A further review of the literature shows endobronchial obstruction is potentially under-reported due to overlap in connective tissue disease symptomatology and there is no consensus on best practice.
Topics: Female; Humans; Male; Adult; Young Adult; Polychondritis, Relapsing; Granulomatosis with Polyangiitis; Bronchial Diseases; Airway Obstruction; Bronchi
PubMed: 37528419
DOI: 10.1186/s13256-023-04058-x -
Sheng Wu Yi Xue Gong Cheng Xue Za Zhi =... Dec 2023Central lung cancer is a common disease in clinic which usually occurs above the segmental bronchus. It is commonly accompanied by bronchial stenosis or obstruction,... (Review)
Review
Central lung cancer is a common disease in clinic which usually occurs above the segmental bronchus. It is commonly accompanied by bronchial stenosis or obstruction, which can easily lead to atelectasis. Accurately distinguishing lung cancer from atelectasis is important for tumor staging, delineating the radiotherapy target area, and evaluating treatment efficacy. This article reviews domestic and foreign literatures on how to define the boundary between central lung cancer and atelectasis based on multimodal images, aiming to summarize the experiences and propose the prospects.
Topics: Humans; Lung Neoplasms; Pulmonary Atelectasis; Bronchi; Constriction, Pathologic; Multimodal Imaging
PubMed: 38151951
DOI: 10.7507/1001-5515.202304016 -
JGH Open : An Open Access Journal of... Jan 2024The prevalence of eosinophilic esophagitis (EoE) is rising in the West. However, data from the Indian subcontinent is limited. In this prospective cross-sectional study,...
BACKGROUND AND AIM
The prevalence of eosinophilic esophagitis (EoE) is rising in the West. However, data from the Indian subcontinent is limited. In this prospective cross-sectional study, we estimated the prevalence of EoE among children undergoing elective upper gastrointestinal endoscopy (UGIE).
METHODS
We enrolled 200 consecutive children (123 boys, median age 10.25 years [interquartile range 8.25-14.5]) between March 2020 and November 2022 at our center. Clinical characteristics, endoscopic findings, and laboratory parameters were noted. A total of 12 mucosal biopsies (3 each from the middle and lower third of the esophagus, stomach, and duodenum) were obtained. EoE was diagnosed if the peak eosinophil count was ≥15/high-power field (HPF) in absence of gastric and duodenal eosinophilia.
RESULTS
The commonest indications for UGIE were gastroesophageal reflux disease-like symptoms (29%), inflammatory bowel disease (22.5%), celiac disease (15%), and abdominal pain (13%). EoE was detected in seven children, suggesting an overall prevalence of 3.5%. Of the 20 children evaluated for dysphagia, 4 (20%) had EoE. Also, two of three (67%) children presented with food bolus impaction along with dysphagia had EoE. Of the seven children with EoE, three (43%) had bronchial asthma, two (28.5%) had peripheral eosinophilia, and one (14%) had elevated serum IgE. Trachealization and linear furrows were found in 57% and 71% cases, respectively. Four children received high-dose proton pump inhibitor (PPI) for 12 weeks, two received PPI+ stricture dilatation, and one received systemic steroids. All achieved clinical, endoscopic, and histopathological remission.
CONCLUSION
Hospital-based prevalence of EoE among children undergoing elective UGIE was 3.5%. EoE patients had favorable outcomes with PPI.
PubMed: 38268956
DOI: 10.1002/jgh3.13024 -
Radiation Oncology (London, England) Aug 2023The management of ultracentral thoracic tumors with ablative dose of radiotherapy remains challenging given proximity to critical central structures. We report patient...
Ten fraction hypofractionated stereotactic body radiotherapy for the management of ultracentral lung tumors: a retrospective analysis of dosimetry, outcomes, and toxicity.
BACKGROUND
The management of ultracentral thoracic tumors with ablative dose of radiotherapy remains challenging given proximity to critical central structures. We report patient outcomes, toxicity, and dosimetry for ultracentrally located tumors with hypofractionated stereotactic body radiotherapy (hfSBRT).
METHODS
Seventy-eight individuals (50 initial radiotherapy, 28 re-irradiation) undergoing 10 fraction hfSBRT for ultracentrally located thoracic tumors treated between 2009 and 2020 at a single institution were retrospectively reviewed. Overall survival (OS), progression free survival (PFS), and local control (LC) were calculated. Incidence and grade of treatment related toxicity were evaluated. Dosimetric analysis of treatment plans and critical adjacent OARs was performed.
RESULTS
At a median follow up time of 13 months, 1- and 3-year OS, PFS, and LC were 89%/63%, 37%/18%, and 84%/65%, respectively. Median dose was 65 Gy (BED = 107.25 Gy). Median primary bronchial tree maximum dose (Dmax) was 60 Gy (V50 = 0.96 cc). Median esophageal Dmax was 38 Gy (V40 = 0 cc). Median great vessel Dmax was 68 Gy (V50 = 3.53 cc). The most common ≥ grade 2 adverse event was pneumonitis, in 15 individuals (20%). Grade 3 or higher toxicity was observed in 9 individuals (12%): three cases of grade 3 pneumonitis (two re-irradiation, one initial radiotherapy), one grade 3 esophageal stricture following re-irradiation, two grade 3 endobronchial obstructions both following initial radiotherapy, and three grade 5 hemoptysis events (two re-irradiation, one initial radiotherapy). One hemoptysis event was categorized as "possibly" related to treatment, while the remaining two events were categorized as "unlikely" related to treatment in patients with clear evidence of disease progression.
CONCLUSIONS
hfSBRT to ultracentral lung tumors delivered over 10 fractions is a safe and effective treatment option, with acceptable rates of toxicity and good rates of tumor control.
TRIAL REGISTRATION
IRB registration number 12573.
Topics: Humans; Carcinoma, Non-Small-Cell Lung; Retrospective Studies; Radiosurgery; Hemoptysis; Lung Neoplasms
PubMed: 37533092
DOI: 10.1186/s13014-023-02298-1