Usual Interstitial Pneumonia Pattern Interstitial Lung Disease Developed in a Patient with IgG4-related Chronic Sclerosing Sialadenitis.

A 69-year-old man was diagnosed with immunoglobulin (Ig) G4-related disease (IgG4-RD) at 62 years old. At that time, he had high serum IgG4 levels and bilateral submandibular gland swelling on CT; thus, a gland biopsy was performed. Because a reticular shadow was found on chest CT, a lung surgical biopsy was also performed. The specimens revealed usual interstitial pneumonia (UIP) pattern interstitial pneumonia with some IgG4-positive cells. The patient was subsequently followed up without treatment. His forced vital capacity and radiological findings progressively deteriorated, consistent with UIP pattern interstitial lung disease but different from a lung lesion of IgG4-RD.

Characteristics of airway resistance in obstructive sleep apnea patients with asthma on oscillometry.

BACKGROUND: Asthma and obstructive sleep apnea (OSA) are prevalent chronic respiratory disorders with a high tendency to coexist. Both diseases involve increased airway resistance in the upright position because of narrow upper or lower airways. Moreover, there is a marked increase in airway resistance in the supine position in patients with OSA. We verified the characteristics of OSA in combination with asthma. METHODS: Airway resistance was measured by oscillometry in the upright and supine position in 11 healthy participants (control), 59 patients with OSA alone, and 33 OSA patients with asthma (coexistence) in the hospital between April 2014 and July 2020. We compared the differences in airway resistance between the upright and supine positions among the three groups. In addition, we performed cephalometry to evaluate the upper airway structure in patients with OSA alone and in patients with both OSA and asthma. RESULTS: A marked increase in airway resistance due to postural change was observed in the "OSA alone" group compared to that in the control group. However, this was not observed in the "coexistence" and control groups. Moreover, the "coexistence" group had fewer structural abnormalities in the upper airway than the "OSA alone" group on cephalometry. CONCLUSIONS: The airway resistance of patients with OSA alone markedly increased with posture change because of upper airway abnormalities. However, there was a smaller increase with postural changes in OSA patients with asthma, suggesting the possibility of a smaller degree of upper airway abnormality compared to patients with OSA alone. CLINICAL TRIAL REGISTRATION NUMBER: This study is approved by the research ethics committee of the Shinshu University School of Medicine (permission number: 4272).

Sleep architecture of short sleep time in patients with obstructive sleep apnea: a retrospective single-facility study.

PURPOSE: Sleep architecture consists of rapid eye movement (REM) sleep and non-REM sleep time. Non-REM sleep time is further classified into three stages by depth (stage N1-N3). Some studies have reported that short sleep time predicts all-cause mortality. Short sleep time can have characteristics of sleep architecture which contribute to poor prognosis. Obstructive sleep apnea (OSA) is a disease which causes cessation or decline of ventilation during sleep due to upper airway stenosis and affects sleep architecture. Few studies have reported on the sleep architecture of short sleep time in patients with OSA. Therefore, we aimed to observe this phenomenon. METHODS: From May 2008 to September 2021, patients diagnosed with OSA at our facility were assessed for clinical history and underwent full-night polysomnography (PSG). These patients were classified into two groups: total sleep time (TST) recorded on PSG consisting of a short TST (< 7 h) group and a not short TST (≥ 7 h) group. RESULTS: Of 266 patients with OSA, compared to the not short TST group (n = 131), the short TST group (n = 135) had a lower REM sleep time (%) and a higher stage N1 sleep time (%). There was a significant difference in age between the two groups, so sub-analyses classified the patients by age: non-elderly patients (< 65 years) and elderly patients (≥ 65 years) to adjust for age. Both sub-analyses showed similar results to the analysis for the combined ages regarding sleep architecture. CONCLUSION: Patients with OSA who had short sleep time had disordered sleep architecture with a lower REM sleep time (%) and more stage N1 sleep time.

The positional characteristics of patients with obstructive sleep apnea: a single institute retrospective study in Japan.

Obstructive sleep apnea (OSA) causes sleep-disordered breathing (SDB) due to upper airway obstruction. The severity of OSA changes with position during sleep. Patients with marked significant improvement in apnea-hypopnea index (AHI) level by sleep position change are defined as ''positional patients'' (PP), while those without improvement are defined as ''non-positional patients'' (NPP). We aimed to verify their clinical characteristics. Between May 2008 and May 2020, 237 patients with OSA were registered retrospectively and classified into two groups: PP (n = 158) and NPP (n = 79). The differences in clinical background and full-night polysomnography (PSG) between the two groups were observed. A logistic regression analysis was conducted to identify the risk factors for severe AHI (≥ 30 events/h) in the PP group. Moreover, confounding factor-adjusted sub-analysis by a propensity score matching method was performed, and the PSG results were compared between the two groups. The PP group was older than the NPP group. Furthermore, the PP group had lower body mass index (BMI) and AHI levels compared with the NPP group. The independent risk factors for severe AHI in the PP group were BMI and being in the supine position during sleep. The PP group had a significantly milder nocturnal hypoxemia despite having no significant difference in AHI levels between the two groups. The characteristics of PP were old age, low BMI, and low AHI associated with milder nocturnal hypoxemia. Moreover, they were less likely to worsen with nocturnal hypoxemia compared with NPP having similar severity.

Successful recovery from critical COVID-19 pneumonia with extracorporeal membrane oxygenation: A case report.

A public health emergency of current international concern is the outbreak of a severe respiratory illness, that is, coronavirus disease (COVID-19). The disease initially started in Wuhan, China, and it rapidly spread to most regions of the world. Herein, we report a case of critical COVID-19 pneumonia treated with extracorporeal membrane oxygenation from symptom onset day 19 (SOD#19) to SOD#30. We describe the patient's clinical course, from mild symptoms at the time of illness onset to symptoms of severe pneumonia as the illness progressed. We provide important information regarding our clinical experience for further understanding of management discrepancies, as treatment with extracorporeal membrane oxygenation or pharmacotherapy (e.g., antivirals, immunomodulators, and glucocorticoids) is often dependent on the severity of symptoms.

Prognosis of patients with acute exacerbation of combined pulmonary fibrosis and emphysema: a retrospective single-centre study.

BACKGROUND: Previous analyses of combined pulmonary fibrosis and emphysema (CPFE) cohorts have provided conflicting data on the survival of patients with CPFE. Therefore, the aim of this study was to investigate the clinical prognosis of acute exacerbations (AE) of CPFE. METHODS: We retrospectively reviewed the medical records of patients who had been treated at the Shinshu University Hospital (Matsumoto, Japan) between 2003 and 2017. We identified 21 patients with AE of CPFE and 41 patients with AE of idiopathic pulmonary fibrosis (IPF) and estimated their prognoses using the Kaplan-Meier method. RESULTS: Treatment content and respiratory management were not significantly different between the two groups before and after exacerbation. At the time of AE, the median serum Krebs von den Lungen-6 level was significantly lower in the CPFE group (Krebs von den Lungen-6: 966 U/µL; white blood cell count: 8810 /µL) than that in the IPF group (Krebs von den Lungen-6: 2130 U/µL, p < 0.001; white blood cells: 10809/µL, p = 0.0096). The baseline Gender-Age-Physiology scores were not significantly different between the two groups (CPFE, 4.5 points; IPF, 4.7 points; p = 0.58). Kaplan-Meier curves revealed that the survival time after AE for patients with CPFE was longer than that for patients with IPF (p < 0.001, log-rank test). CONCLUSIONS: Survival prognoses after AE were significantly better for patients with CPFE than that for those with IPF. Our findings may improve the medical treatment and respiratory management of patients with AE-CPFE.

Twenty-year Follow-up of the First Bilateral Living-donor Lobar Lung Transplantation in Japan.

Patients with end-stage lung disease can undergo living-donor lobar lung transplantation (LDLLT), with survival rates improving every year. We herein report the 20-year follow-up findings of the first patient who underwent LDLLT in Japan. A 24-year-old woman with primary ciliary dyskinesia became ventilator-dependent after severe respiratory failure and right-sided heart failure following repeated respiratory infections. In 1998, she underwent LDLLT and received her sister's right lower lobe and her mother's left lower lobe. Although the patient required 21 hospitalizations and developed unilateral bronchiolitis obliterans syndrome, she is in good physical condition and lives without restriction at 20 years after undergoing LDLLT.

Virtual bronchoscopic navigation as an aid to CT-guided transbronchial biopsy improves the diagnostic yield for small peripheral pulmonary lesions.

BACKGROUND AND OBJECTIVE: Virtual bronchoscopic navigation (VBN) entails the provision of a virtual display of the bronchial routes that lead to small peripheral pulmonary lesions (PPL). It has been predicted that a combination of computed tomography (CT)-guided transbronchial biopsy (CT-TBB) with VBN might improve the diagnostic yield for small PPL. This study sought to investigate that prediction. METHODS: A total of 100 patients with small PPL (<20 mm) were enrolled for CT-TBB and randomly allocated to either a VBN+ or VBN- group (50 subjects per group). Group results were then compared in terms of diagnostic yield, whole procedure time, times at which the first CT scan and biopsy were taken and the number of lung biopsy specimens retrieved. RESULTS: The diagnostic yield for small PPL was significantly higher in the VBN+ group versus VBN- group (84% vs 58%, respectively (P = 0.013)), with no significant difference in (whole) examination time between groups (VBN+: 32:53 (32 min and 53 s) ± 12:01 vs VBN-: 33:06 ± 10:08 (P = NS)). However, the time periods between commencing the examination and either the first CT scan or first biopsy were significantly shorter for the VBN+ group, while the net biopsy time tended to be longer for this group with a significantly higher number of specimens collected (VBN+: 3.54 ± 1.07 specimens vs VBN-: 2.98 ± 1.06 specimens (P = 0.01)). CONCLUSION: Combining VBN with CT-TBB significantly improved the diagnostic yield for small PPL.

Usefulness of the forced oscillation technique in assessing the therapeutic result of tracheobronchial central airway obstruction.

BACKGROUND: Pulmonary function tests (PFTs) comprise the traditional method for detecting central airway obstruction (CAO) and evaluating therapeutic effects, but are effort-dependent. By contrast, the forced oscillation technique (FOT) is performed during tidal breathing in an effort-independent mode and is universally used to assess respiratory function in patients with chronic obstructive pulmonary disease (COPD) and asthma. We used the FOT to measure airway resistance and reactance in patients with CAO before and after interventional bronchoscopy and compared the results to data obtained using PFTs. METHODS: Twelve patients with CAO were recruited from December 2013 to July 2016. The FOT, PFTs, chest computed tomography (CT), COPD Assessment Test (CAT), and the modified Medical Research Council (mMRC) dyspnea scale were employed before and after interventional bronchoscopy. The minimum airway cross-sectional area (MACSA) was calculated using a CT image calculator. RESULTS: Of the 12 patients, 6 had tracheal obstruction and 6 had bronchial obstruction. All FOT measurements, except ΔX5, were significantly improved after interventional bronchoscopy in all cases. The significance of the improvement was greater with the FOT than PFTs. The MACSA, CAT, and mMRC dyspnea scale scores also significantly improved in all cases. Furthermore, only alteration of resistance at 20 Hz (R20) significantly correlated with the alteration of the MACSA after intervention. No significant correlations were found for PFTs. CONCLUSIONS: The FOT is suitable and convenient for assessing therapeutic results in patients with tracheobronchial CAO. The alteration of R20 is useful for estimating the airway dilation of CAO after interventional bronchoscopy.

Bevacizumab-induced tracheoesophageal fistula in a patient suffering from lung cancer with bulky subcarinal lymph node: a case report.

A 66-year-old male with advanced non-small-cell lung cancer (NSCLC) who was previously treated with carboplatin, pemetrexed, and bevacizumab consequently suffered from severe coughing during deglutition. Chest computed tomography (CT) revealed a tracheoesophageal fistula (TEF) between the left main bronchus and esophagus through a subcarinal metastatic lymph node. Given the extreme swelling of the lymph node due to metastatic cancer, it was determined that the walls of the bronchus and esophagus had been injured simultaneously. Delayed and dysfunctional wound healing due to bevacizumab resulted in necrosis of the contact region leading to fistula formation. This case suggests that using bevacizumab for NSCLC in patients with bulky subcarinal lymphadenopathy may increase the risk for TEF.

Associations between the distance covered in the incremental shuttle walk test and lung function and health status in patients with chronic obstructive pulmonary disease.

BACKGROUND: Field walk tests such as the incremental shuttle walk test (ISWT) are simple tests for assessing the degree of disability in individuals with chronic obstructive pulmonary disease (COPD). In the present study, the correlations between exercise performance in the ISWT, lung function, and health status were examined in patients with COPD of varying severities. METHODS: A retrospective examination of 277 COPD patients was performed using the ISWT and lung function tests along with assessment of health status using St. George׳s Respiratory Questionnaire (SGRQ). In addition, we assessed the correlations between the walking distance, lung function parameters, and SGRQ scores. RESULTS: ISWT distances were poorly correlated with lung function parameters and SGRQ scores in mild COPD patients. In contrast, ISWT distances were significantly correlated with pulmonary function parameters, such as vital capacity (%predicted) and forced expiratory volume in one second, and SGRQ scores in moderate and severe COPD patients. CONCLUSIONS: The ISWT is more independent of health status and pulmonary function in patients with mild COPD compared to moderate or severe cases. Therefore, the exercise capacity of patients with mild COPD should be estimated by the ISWT.

Hypertrophic Pulmonary Osteoarthropathy in Anaplastic Lymphoma Kinase (ALK)-positive Lung Cancer.

A 49-year-old man was admitted to a hospital with chest pain and polyarthralgia. Chest radiography showed abnormal findings, and chest computed tomography showed a mass in the right lung. A transbronchial lung biopsy led to a diagnosis of anaplastic lymphoma kinase (ALK)-positive adenocarcinoma. Bone scintigraphy revealed bilateral symmetrical accumulations of (99m)Technetium complexes in the long bones, suggesting co-existing hypertrophic pulmonary osteoarthropathy (HPO). The patient underwent four courses of chemotherapy with cisplatin plus pemetrexed, which led to decreased (99m)Technetium accumulations in the long bones. To the best of our knowledge, this is the first reported case of HPO associated with ALK-positive lung cancer.

Late-onset rejection of a unilateral donor lung with vascular C4d deposition in bilateral living-donor lobar lung transplantation: an autopsy case report.

A 37-year-old woman had undergone bilateral living-donor lobar lung transplantation 11 years previously for idiopathic pulmonary arterial hypertension. Her father donated the right lobe and her brother donated the left lobe. She subsequently developed progressively worsening respiratory dysfunction due to pneumonia. CT showed left dominant pulmonary artery dilatation, bronchial wall thickening and airway stenosis, followed by sudden death. An autopsy showed marked pathologic left dominant rejection of the pulmonary artery, small airway and large airway. Notably, only the left lung showed C4d vascular deposition, thus suggesting that antibody-mediated lung rejection may have occurred.

Primary malignant sarcomatoid mesothelioma in the pericardium.

Primary malignant pericardial mesothelioma is an exceptionally rare tumor, and making an antemortem diagnosis of this disease is notoriously difficult. We herein report the case of a 61-year-old woman with pericardial mesothelioma who presented with shortness of breath and peripheral edema of the lower limbs. Chest computed tomography (CT) showed an anterior mass and thickened pericardium with multiple pericardial nodules. A biopsy of the mediastinal mass was performed using right thoracotomy, and the histological findings indicated a sarcomatoid tumor. The patient was treated with chemotherapy; however, she but died three months after diagnosis. An autopsy confirmed a final diagnosis of sarcomatoid type primary malignant pericardial mesothelioma following extensive immunohistopathological examinations.

Synchronous gastrointestinal stromal tumor and primary lung adenocarcinoma.

Although rare, gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract. The asynchronous occurrence of other malignancies in patients with GIST during the clinical course is relatively common. However, the synchronous coexistence of GIST and lung cancer has only rarely been reported. We experienced a case of coincidental primary lung adenocarcinoma and intestinal GIST. The present case is not only of interest due to the rare coincidence of GIST and lung cancer, but also because there was an epidermal growth factor receptor gene mutation in the lung cancer and a c-kit mutation in the GIST.

Acute neurogenic pulmonary edema following electroconvulsive therapy: a case report.

OBJECTIVE: We report the case of a 47-year-old man with depression who developed acute dyspnea, hypoxemia, and mild hemoptysis after electroconvulsive therapy (ECT). METHOD: Intravenous carbazochrome sodium sulfate hydrate as a hemostatic drug (100 mg/day) was prescribed for 2 days. On the day of ECT, oxygen inhalation (4 L/min) was continued, and SpO2 was maintained at 94-96%. RESULTS: Chest radiography showed improvement in alveolar infiltration. Chest CT 6 days after ECT also confirmed the disappearance of ground glass opacities in the lung fields. CONCLUSION(S): NPE is life threatening and should be recognized as an uncommon adverse event associated with ECT.

Myocardial performance index in subjects susceptible to high-altitude pulmonary edema.

OBJECTIVE: A recent study concerning high-altitude pulmonary edema (HAPE), a non-cardiogenic pulmonary edema, suggested that it is initially a hydrostatic-type pulmonary edema. We suspect that some extent of cardiac insufficiency may likely relate to the mechanism of the development of this disease. METHODS: By Doppler echocardiography, the Tei index (a new quantitative index proposed for the evaluation of global myocardial performance) and the systolic pulmonary artery pressure (sPAP) were measured before and after 30 minutes of hypoxic breathing. PATIENTS: Eleven HAPE-susceptible subjects (HAPE-s) and nine HAPE-resistant subjects (HAPE-r). RESULTS: The results of Tei index indicated an enhanced left myocardial performance but an impaired right performance in HAPE-s during hypoxic breathing. The sPAP of HAPE-s was significantly increased after hypoxic breathing, which was not correlated with the heart functions such as right ventricular (RV) Tei index, cardiac index (CI), percent ejection fraction (EF%) and percent fractional shortening (FS%) under hypoxic condition. Comparatively, the HAPE-r subjects did not show such significant changes of Tei index after hypoxic breathing. The results suggested that a paradoxical myocardial performance, in a format of an augmented left ventricular (LV) in contrast to an attenuated RV, was observed in the HAPE-s exposed to acute hypoxia. CONCLUSION: The responses of the left and right myocardial performances to hypoxia may be involved in the pathogenesis of HAPE.