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Bronchiectasis is a chronic respiratory disease characterized by abnormal dilation of the bronchi that causes cough, sputum, and recurrent infections. As it may be associated with various respiratory or systemic diseases, a critical aspect of managing bronchiectasis is to identify the underlying cause. Immunodeficiency is a rare but important cause of bronchiectasis, and its treatability is a significant trait for bronchiectasis management. While primary immunodeficiencies in bronchiectasis are well recognized, secondary immunodeficiencies remain under-reported and under-researched. Secondary immunodeficiencies may result from various diseases and conditions, such as hematologic malignancies, human immunodeficiency virus infection, renal transplantation, or the use of immunosuppressive drugs, and may contribute to the occurrence of bronchiectasis. Recurrent pulmonary and/or extrapulmonary infections in bronchiectasis may indicate the presence of secondary immunodeficiency in patients with these underlying conditions. For treatment, examining the underlying condition, managing bronchiectasis adequately, and prophylactic antibiotics (e.g., macrolide) and/or supplementary immunoglobulin G therapy may provide potential benefits. Considering the projected increase in the prevalence of secondary immunodeficiencies and bronchiectasis, future guidelines and research on the diagnosis and optimized treatment are needed.

Background: Hypersensitivity pneumonitis (HP) is an interstitial lung disease (ILD) that results from an immune-mediated reaction involving various antigens in susceptible individuals. However, the clinical characteristics and outcomes of HP in South Korea are not well understood. Objectives: This study was conducted to identify the clinical characteristics and outcomes of HP in South Korea. Design: This is a retrospective observational study investigating patients with pathologically confirmed HP at our center, along with a comprehensive review of published HP cases in the Republic of Korea. Methods: This retrospective study analyzed 43 patients with pathologically proven HP at a single tertiary hospital in Korea between 1996 and 2020. In addition, case reports of HP published in Korea were collected. The clinical characteristics, etiologies, treatment, and outcomes of patients from our center, as well as case reports, were reviewed. Patients from our hospital were divided into fibrotic and nonfibrotic subtypes according to the ATS/JRS/ALAT guidelines. Results: Among 43 patients with biopsy-proven HP, 12 (27.9%) and 31 (72.1%) patients were classified into the fibrotic and nonfibrotic subtypes, respectively. The fibrotic HP group was older (64.6 ± 8.5 versus 55.2 ± 8.3, p = 0.002) with less frequent complaints of fever (0% versus 45.2%, p = 0.013) compared to the nonfibrotic HP group. The most common inciting antigen was household mold (21, 48.8%), followed by inorganic substances (6, 14.0%). Inciting antigens were not identified in eight (18.6%) patients. Treatment of corticosteroids was initiated in 34 (79.1%) patients. An analysis of 46 patients from Korea by literature review demonstrated that reported cases were relatively younger and drugs were the most common etiology compared to our cohort. Conclusion: The analysis of reported cases, as well as our cohort, showed that exposure history and clinical manifestations are heterogeneous for patients with HP in South Korea.

Tracheobronchopathia osteochondroplastica (TPO) is an idiopathic disease involving the cartilage rings of the large airway, characterized by submucosal calcified nodules. Localized tracheobronchial amyloidosis (TBA) is another rare disease with localized amyloid deposits in the tracheobronchial tree. The two diseases rarely coincide, and only a few case reports and series have been reported.A patient with dyspnea was referred to our clinic for suspicion of TBA. Chest computed tomography (CT) scan showed marked thickening of the tracheobronchial wall with calcified endobronchial submucosal nodules. The nodules were resected with a Diode Laser under rigid bronchoscopy, and results from the biopsy showed both osteochondroid metaplasia on microscopy in Hematoxylin and Eosin staining and apple-green birefringence on polarized microscopy in Congo red staining. This is a rare case in which microscopic findings of both TPO and TBA were observed on one slide. These findings suggest that localized TBA could be a cause of TPO.

Tracheobronchopathia osteochondroplastica (TPO) is an idiopathic disease involving the cartilage rings of the large airway, characterized by submucosal calcified nodules. Localized tracheobronchial amyloidosis (TBA) is another rare disease with localized amyloid deposits in the tracheobronchial tree. The two diseases rarely coincide, and only a few case reports and series have been reported.A patient with dyspnea was referred to our clinic for suspicion of TBA. Chest computed tomography (CT) scan showed marked thickening of the tracheobronchial wall with calcified endobronchial submucosal nodules. The nodules were resected with a Diode Laser under rigid bronchoscopy, and results from the biopsy showed both osteochondroid metaplasia on microscopy in Hematoxylin and Eosin staining and apple-green birefringence on polarized microscopy in Congo red staining. This is a rare case in which microscopic findings of both TPO and TBA were observed on one slide. These findings suggest that localized TBA could be a cause of TPO.

Malignant pulmonary granular cell tumor (GCT) is extremely rare and difficult to distinguish from benign GCT. Most GCTs are neural-type and express S-100. However, a small subset of tumors sub-classified as the non-neural type do not express S-100. We report a case of malignant non-neural-type GCT in the lungs.A 77-year-old woman felt chest discomfort and dyspnea in July 2019. She had never smoked and had no medical history other than hypertension and diabetes mellitus.She was initially evaluated at a local hospital. Flexible bronchoscopy showed total occlusion of the right main bronchus by a mass-like lesion. Biopsy of the mass lesion revealed chronic inflammation. The patient visited for re-evaluation in September 2019. Rigid bronchoscopy showed worsening of the total obstruction of the right main bronchus by a tumor mass, such that the carina was not visible. Additionally, endobronchial nodules were observed on the medial side of left main bronchus. The tumor masses of both main bronchi were removed by bronchoscopic intervention, but the right main bronchus was not opened. Biopsy revealed malignant GCT, favoring the non-neuronal type (S-100-negative).We report an extremely rare case of malignant pulmonary GCT negative for S-100 in immunohistochemistry. In this case, surgical resection was not possible because the tumor was diagnosed at a fairly advanced stage and had spread to involve the contralateral main bronchus. The patient chose to be treated at another hospital and was thereafter lost to follow-up. Keywords: Malignant pulmonary granular cell tumor, S-100-Negative, Non-neural type

Bronchopleural fistula (BPF) is a communication between the pleural space and the bronchial tree. Herein, we report a patient with persistent air leak after necrotizing pneumonia, where tract of BPF was not apparent in computed tomography (CT). Despite control of infection, watchful observation, repetitive procedures, and surgery, air leak was not resolved. This led to consideration of the endobronchial valve (EBV) placement. After identifying the bronchial segment leading to the fistula with indigo carmine, precise occlusion of the lingular division of the left upper lobe (LUL) was performed using an EBV. Subsequently, the air leak was resolved. During 6 months of follow-up, neither recurrence of BPF nor any procedure-related complications were noted. We concluded that precise EBV placement by identifying bronchial segment leading to the BPF, could be an effective treatment for persistent air leak. Keywords: Endobronchial valve, Bronchopleural fistula, Persistent air leak