Multidisciplinary discussion (MDD) requiring close communication between specialists (clinicians, radiologists and pathologists) is the gold standard for the diagnosis of idiopathic interstitial pneumonias (IIPs). However, MDD by specialists is not always feasible because they are often separated by time and location. An online database would facilitate data sharing and MDD. Our aims were to develop a nationwide cloud-based integrated database containing clinical, radiological and pathological data of patients with IIPs along with a web-based MDD system, and to validate the diagnostic utility of web-based MDD in IIPs.Clinical data, high-resolution computed tomography images and lung biopsy slides from patients with IIPs were digitised and uploaded to separate servers to develop a cloud-based integrated database. Web-based MDD was performed using the database and video-conferencing to reach a diagnosis.Clinical, radiological and pathological data of 524 patients in 39 institutions were collected, uploaded and incorporated into the cloud-based integrated database. Subsequently, web-based MDDs with a pulmonologist, radiologist and pathologist using the database and video-conferencing were successfully performed for the 465 cases with adequate data. Overall, the web-based MDD changed the institutional diagnosis in 219 cases (47%). Notably, the MDD diagnosis yielded better prognostic separation among the IIPs than did the institutional diagnosis.This is the first study of developing a nationwide cloud-based integrated database containing clinical, radiological and pathological data for web-based MDD in patients with IIPs. The database and the web-based MDD system that we built made MDD more feasible in practice, potentially increasing accurate diagnosis of IIPs.

Allergic bronchopulmonary aspergillosis (ABPA) is characterized by an early allergic response and late-phase lung injury in response to repeated exposure to Aspergillus antigens, as a consequence of persistent fungal colonization of the airways. Here, we summarize the clinical and pathological features of ABPA, focusing on thick mucus plugging, a key observation in ABPA. Recent findings have indicated that luminal eosinophils undergo cytolytic extracellular trap cell death (ETosis) and release filamentous chromatin fibers (extracellular traps, ETs) by direct interaction with Aspergillus fumigatus. Production of ETs is considered to be an innate immune response against non-phagocytable pathogens using a “trap and kill” mechanism, although eosinophil ETs do not promote A. fumigatus damage or killing. Compared with neutrophils, eosinophil ETs are composed of stable and condensed chromatin fibers and thus might contribute to the higher viscosity of eosinophilic mucus. The major fate of massively accumulated eosinophils in the airways is ETosis, which potentially induces the release of toxic granule proteins and damage-associated molecular patterns, epithelial damage, and further decreases mucus clearance. This new perspective on ABPA as a luminal hypereosinophilic disease with ETosis/ETs could provide a better understanding of airway mucus plugging and contribute to future therapeutic strategies for this challenging disease.

Background: While nontuberculous mycobacterial (NTM) pleuritis rarely complicates pulmonary NTM infection, high mortality has been reported in case reports and small studies. Objectives: The purpose of this study was to clarify the clinical features and treatment outcomes of pulmonary NTM infection cases accompanied by NTM pleuritis. Methods: Medical records of 1,044 patients with pulmonary NTM disease were retrospectively reviewed to select patients complicated by NTM-proven pleuritis. We investigated clinical characteristics, pathogens, pleural effusion examinations, radiographic findings, treatments, and clinical course of the NTM pleuritis patients. Results: Among 1,044 cases with pulmonary NTM, NTM pleuritis occurred in 15 cases (1.4%). The mean age was 69 years with a performance status of mostly 2 or better (80.0%), and 6 cases (40.0%) were complicated by pneumothorax. Subpleural cavities were radiologically detected in 11 cases (73.3%), and extrapulmonary air-fluid level was detected in 14 cases (93.3%). Eleven patients were treated with combinations of 2–4 antimycobacterial drugs, including clarithromycin, and 2 patients were treated with isoniazid, rifampicin, and ethambutol. Chest tube drainage was performed in 11 cases, and surgical approach was added in 6 cases. The pleural effusion of 2 patients treated with only antimycobacterial medications gradually deteriorated. Two patients died from NTM pleuritis, and 1 patient died from pneumonitis during a mean of 1.8 years of follow-up. Conclusions: Comorbid NTM pleuritis was difficult to treat by medical therapy alone and resulted in a poor prognosis. In addition to antimycobacterial agents, chest tube drainage and surgical procedures in the early stages should be considered to treat NTM pleuritis.

Thoracic diseases in patients with systemic lupus erythematosus (SLE), especially interstitial pneumonia (SLE-IP), are rare and have been poorly studied. The aims of this multicentre study were to evaluate SLE-IP and elucidate its clinical characteristics and prognosis. Fifty-five patients with SLE-IP who had attended the respiratory departments of participating hospitals were retrospectively evaluated in this multicentre study. Clinical information, high-resolution computed tomography (HRCT), and surgical lung biopsy/autopsy specimens were analysed by respiratory physicians, pulmonary radiologists, and pulmonary pathologists. IP patterns on HRCT and lung specimens were classified based on the international classification statement/guideline for idiopathic interstitial pneumonias. The most frequent form of SLE-IP at diagnosis was chronic IP (63.6%), followed by subacute (20.0%), and acute IP (12.7%). Radiologically, the most common HRCT pattern was “Unclassifiable” (54%). Histologically, “Unclassifiable” was the most frequently found (41.7%) among 12 patients with histologically proven IP. Interestingly, accompanying airway diseases were present in nine of these patients (75%). In multivariate analysis, current smoking (hazard ratio [HR] 6.105, p = 0.027), thrombocytopenia (HR 7.676, p = 0.010), anti-double-strand DNA titre (HR 0.956, p = 0.027), and nonspecific interstitial pneumonia (NSIP) + organizing pneumonia (OP) pattern on HRCT (vs. NSIP, HR 0.089, p = 0.023) were significant prognostic factors. In conclusion, chronic IP was the most frequent form of IP in patients with SLE-IP, and “Unclassifiable” was the commonest pattern radiologically and histologically.

A 41-year-old man was referred to our hospital for the treatment of chronic thromboembolic pulmonary hypertension (CTEPH). On admission, he was in World Health Organization functional class III. Right heart catheterization demonstrated that pulmonary arterial pressure (systolic/diastolic/mean) was 140/42/71 mm Hg, cardiac index was 1.6 L/min/m2 and pulmonary vascular resistance was 1663 dyn ·s·cm -5 . The patient had severe pulmonary hypertension and was considered inoperable because of peripheral organized thrombi and coexisting seminoma; therefore, balloon pulmonary angioplasty (BPA) was performed. After BPA, pulmonary angiography showed improvement of pulmonary arterial flow ( Figure 1A). Pulmonary arterial pressure was 108/42/67 mm Hg and cardiac index was 2.5 L/ min/m 2 when he returned to the cardiac care unit after BPA. His condition temporarily improved; however, 2 hours later, it deteriorated because of reperfusion pulmonary injury and gastrointestinal bleeding. He required mechanical ventilation and percutaneous cardiopulmonary support. To improve hemodynamics, another session of BPA was tried 9 days later (Figures 2A and 3A) and pulmonary arterial pressure seemed to improve to 83/48/58 mm Hg. However, he died from right heart failure on day 26 after BPA despite intensive care.Histological analysis of the autopsy specimens showed recanalized thrombi in the bilateral elastic pulmonary arteries ( Figure 4). Diffuse pulmonary arterial medial and intimal thickening were also observed in muscular pulmonary arteries. These findings are consistent with CTEPH and the diagnosis was confirmed. The arterial media was dissected near the lamina elastica interna by BPA ( Figures 1B, 2B, and 3B). The organized thrombi were forced to one side and the dissection formed pseudovascular spaces that configured new lumina, which were larger than the original channels. Newly formed intima was observed on the inner surface of these pseudovascular spaces ( Figures 1B-c, 2B-b, and 3B-b, black arrowheads).According to the guidelines for the diagnosis and treatment of pulmonary hypertension, pulmonary endarterectomy is a standard therapy for patients with CTEPH with proximal thrombi.1 However, not all patients can undergo this curative surgery because of the presence of thrombi in distal pulmonary arteries, difficulty of the operation, or comorbidities. We have reported that BPA could improve hemodynamics in inoperable patients with CTEPH.2 However, it is unknown how pulmonary arteries are changed by BPA and the mechanism by which hemodynamic improvement is achieved, although thrombi are not removed from the affected arteries. We have recently reported a case in which we were able to examine a pulmonary artery in a single lobe after BPA.3 A pathological examination of the dilated lesion showed that the vascular lumen was dilated by a small incision and compression of the thrombi without dissection. In contrast, the histology of the present case demonstrated that the lumina were dilated by the dissection at a plane in the media (...