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Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease with a poor prognosis and an unknown cause that generally progresses to pulmonary fibrosis and leads to irreversible tissue alteration. The "Guidelines for the treatment of idiopathic pulmonary fibrosis 2017," specializing in the treatment of IPF for the first time in Japan and presenting evidence-based standard treatment methods suited to the state of affairs in Japan, was published in 2017, in line with the 2014 version of "Formulation procedure for Minds Clinical Practice Guidelines."

Idiopathic interstitial pneumonias are an independent risk factor of lung cancer, and a chemotherapy-induced acute exacerbation is the most common lethal complication in Japanese patients. The safety and efficacy of carboplatin and weekly paclitaxel for the treatment of non-small cell lung cancer with idiopathic interstitial pneumonias has been previously reported in prospective studies. However, carboplatin + paclitaxel with bevacizumab is currently the standard therapy. We conducted a multicenter, phase II study to confirm the safety and efficacy of carboplatin + weekly paclitaxel + bevacizumab for the treatment of patients with lung cancer complicated by idiopathic interstitial pneumonias.

Numerous studies investigated patients with IPF; however, only a few examined patients with idiopathic interstitial pneumonias (IIPs).

Fiberoptic tracheal intubation (FTI) in bronchoscopy is widely performed with a conventional Portex tracheal tube (PTT). Occasionally, it is difficult for pulmonologists with limited experience to insert a tube beyond the vocal cords and advance it into the trachea. A new endotracheal tube, the Parker Flex-Tip tube (PFT), was recently designed. We compared the usefulness and safety of PFT versus PTT for FTI in bronchoscopy performed by pulmonologists with limited experience.

The present article reports the case of a 51-year-old female patient who was clinically diagnosed with idiopathic pleuroparenchymal fibroelastosis in the initial phase of the disease. The upper and lower lobes showed a pleuroparenchymal fibroelastosis pattern and reticular shadow, respectively. Both upper and lower shadows gradually progressed. Five years following the first detection of the chest shadow, left single-lung transplantation was performed. In the multidisciplinary discussion held after the lung transplantation, most participants recognized this end-stage lung disease as unclassifiable idiopathic interstitial pneumonia though the major pathological findings showing a pleuroparenchymal fibroelastosis pattern.

The presence of histologically evident epithelioid granuloma is required for the diagnosis of sarcoidosis worldwide. The Japan Society of Sarcoidosis and Other Granulomatous Disorders 2015 diagnostic criteria (JSSOG 2015 criteria) includes “clinically proven diagnosis” (involvement of at least 2 of 3 systems confirmed solely by clinical assessment) because of the frequency of sarcoidosis with ocular, cardiac, and respiratory involvement in Japan and the difficulty of obtaining specimens. Here, we describe in detail the clinical presentation of clinically diagnosed sarcoidosis.

Idiopathic pulmonary fibrosis (IPF) is the archetypal interstitial lung disease. It is a chronic progressive condition that is challenging to manage as the clinical course of the disease is often difficult to predict. The prevalence of IPF is rising globally and in Japan, where it is estimated to affect 27 individuals per 100,000 of the population. Greater patient numbers and the poor prognosis associated with IPF diagnosis mean that there is a growing need for disease management approaches that can slow or even reverse disease progression and improve survival. Considerable progress has been made in recent years, with the approval of two antifibrotic therapies for IPF (pirfenidone and nintedanib), the availability of Japanese treatment guidelines, and the creation of global and Japanese disease registries. Despite this, significant unmet needs remain with respect to the diagnosis, treatment, and management of this complex disease. Each of these challenges will be discussed in this review, including making a timely and differential diagnosis of IPF, uptake and adherence to antifibrotic therapy, patient access to pulmonary rehabilitation, lung transplantation and palliative care, and optimal strategies for monitoring and staging disease progression, with a particular focus on the status in Japan. In addition, the review will reflect upon how ongoing research, clinical trials of novel therapies, and technological advancements (including artificial intelligence, biomarkers, and genomic classification) may help address these challenges in the future.

The latest guidelines on hypersensitivity pneumonitis (HP) categorise the disease as either fibrotic or non-fibrotic because of the greater clinical utility of this stratification. However, the prevalence and incidence of fibrotic and non-fibrotic HP are unknown. This study assessed the exact prevalence and incidence of fibrotic and non-fibrotic HP in Japan in 2021.

Idiopathic pulmonary fibrosis (IPF) is a disease of unknown etiology which accounts for a large proportion of cases of idiopathic interstitial pneumonia. It has a very poor prognosis with a 5-year survival rate of 30% or below, and so far there has been no guideline in Japan offering an established effective therapy based on evidence. In addition to the establishment of basic therapies, there is also an urgent need to establish therapies to deal with complications, as death occurs in many cases due to acute exacerbation or comorbid lung cancer. It was therefore decided to formulate a guideline in order to promote evidence-based clinical practice, to further improve the quality of medical treatment in the clinical setting, and to allow the benefits to be enjoyed by the public.

Several studies have reported that viral infections are related to lung cancer. We previously reported the involvement of Torque teno virus (TTV) in patients with lung cancer and idiopathic pulmonary fibrosis. However, the role of TTV in lung cancer growth, and its influence on changes in TTV DNA titers due to idiopathic pulmonary fibrosis (IPF) in lung cancer patients are poorly understood.

Pirfenidone is an oral drug that exerts not only anti-fibrotic activity but also pleiotropic effects, such as anti-inflammatory and anti-oxidative effects. Because it suppresses reduction in vital capacity and improves progression-free survival, it was approved in October 2008 in Japan for the first time in the world as an anti-fibrotic agent for treatment of idiopathic pulmonary fibrosis (IPF). In October 2014, the agent was approved in the U.S., based on the results of the ASCEND study. Today, it is commercially available in 38 countries worldwide.

Idiopathic pulmonary fibrosis (IPF) is a chronic fibrotic lung disease with a median survival of 2–5 years and limited treatment options. In 2008, pirfenidone became the first drug to be approved for IPF treatment in Japan. The aim of this study was to assess the safety of pirfenidone for IPF treatment in a clinical setting.

A 59-year-old woman, who was given a diagnosis of sarcoidosis by supraclavicular lymph node biopsy 5 years previously, was admitted for further examination following abnormal radiologic findings. Nodular pulmonary and abdominal lesions were observed by computed tomography, and liver biopsy was performed and showed epithelioid cell granulomas. She was asymptomatic and was followed up with no therapy. At 1 year follow-up, the pulmonary and abdominal lesions had nearly complete resolution. Nodular pulmonary and abdominal lesions in patients with sarcoidosis can mimic metastatic disease, lymphoma, and infection, and can reappear during disease activity. Therefore, differential diagnosis and continual follow-up are important.

Background:Interleukin 33 (IL-33) works as a functional mediator in allergic disease by enhancing the activity of eosinophils and inducing expression of T helper 2 (Th2)-associated cytokines. However, the role of IL-33 in pulmonary eosinophilia has not been elucidated. We investigated the levels of IL-33 in eosinophilic pneumonia (EP) together with associated cytokines, and discussed the clinical significance of IL-33 in EP.

The potential of chemically derived graphene as a solution-processable transparent conductive film has been explored. Synthesis of amine-functionalized graphene oxide was intended for its utilization in layer-by-layer (LBL) assembly. LBL assembly of graphene oxide was utilized to fabricate graphene-based thin films in a scalable and highly reproducible way. It was found that the optical transmittance and sheet resistance of the film decrease with an increase in the number of LBL cycles in a reproducible way. The sheet resistance of the LBL-assembled GO film was improved by an order of magnitude at the same optical transparency due to the good uniformity and stacking of graphene flakes. Furthermore, we demonstrated the potential for the window electrodes of silicon thin-film solar cells.

Biopsy by video-assisted thoracoscopic surgery (VATS) for interstitial pneumonia allows collection of samples sufficient for accurate histologic diagnosis. Although VATS is relatively safe, several reports have suggested that surgical lung biopsy may be a risk factor for acute exacerbation of idiopathic pulmonary fibrosis (IPF). We retrospectively reviewed data on the 113 cases that underwent biopsy by VATS to diagnose diffuse parenchymal lung disease in our department between 1994 and 2006, and analyzed its complications, in particular, risk of acute exacerbation of IPF. As the final diagnosis, idiopathic interstitial pneumonia was most frequent, involving 52 cases, of which IPF was most frequently found followed by nonspecific interstitial pneumonia and cryptogenic organizing pneumonia, in that order. Among our cases, there were 2 deaths after VATS (mortality rate, 1.8), and both were IPF cases with acute exacerbation. When examining clinical markers in the 2 fatal IPF cases with acute exacerbation, we found that the percentage of predicted forced vital capacity was 55 or lower, percentage of predicted carbon monoxide diffusing capacity was 40 or lower, serum interstitial pneumonia markers KL-6 and SP-D were elevated, intraoperative inhalation of 100 O2was 80 minutes or longer, and postoperative thoracic drainage was required for 10 days or longer. Although acute exacerbations of IPF seem to occur at any time during the course of disease, it is important to be aware of the possibility of acute exacerbation of IPF after VATS.

ABSTRACTZoonotic transmission of hepatitis E virus (HEV) from captured wild deer or boars to humans has been suggested. Antibody to HEV was detected in 9% of 35 wild boars and 2% of 117 wild deer tested, and a presumably indigenous HEV of genotype 3 was isolated from a boar in Japan.

AbstractRecent studies suggest that nitric oxide (NO) may play an important role in the pathophysiology of pulmonary hypertension. Nitroglycerin is metabolized to NO, which is a potent vascular smooth muscle relaxant. The aim of the present study was to compare the effects of inhaled and infused nitroglycerin on pulmonary hemodynamics and gas exchange in anesthetized, artificially ventilated dogs. Nitroglycerin was administrated either by inhalation or by infusion. Systemic blood pressure (SBP), pulmonary arterial pressure (PAP), and pulmonary capillary wedge pressure (PCWP) were measured, and cardiac output was estimated by an electromagnetic flowmeter. Blood gas measurements were performed during hypoxic gas exposure (FiO2; 0.1) with a continuous inhalation or infusion of nitroglycerin (1, 2.5 µg/kg/min). Inhaled (n = 4) and infused (n = 4) nitroglycerin (1 µg/kg/min) did not produce any detectable effects on the hemodynamics. Inhaled nitroglycerin (2.5 µg/kg/min) reduced SBP, PAP and calculated pulmonary vascular resistance (PVR) in all dogs. Cardiac output did not change. In addition, inhaled nitroglycerin increased PaO2. In contrast, the continuous infusion of nitroglycerin (2.5 µg/kg/min) did not change in PAP, whereas infused nitroglycerin decreased the mean SBP. Infused nitroglycerin did not alter cardiac output and calculated PVR. A decreased PaO2was noted in 2 dogs on nitroglycerin infusion. These findings indicate that inhaled nitroglycerin effects the pulmonary circulation relatively more than infused nitroglycerin, which tends to have more of a general effect on the systemic circulation. The effects of inhaled nitroglycerin may thus be comparable to the effects of NO inhalation.

Phase IV clinical trials in Western countries have reported that combined therapy with pirfenidone and nintedanib for idiopathic pulmonary fibrosis (IPF) has a manageable safety profile. However, data on the long-term safety and tolerability of this combination treatment in the real-world setting in Japan are limited.

Chemically synthesized graphene is promising for device applications because the chemical approach enables ease of mass production and chemical modification of its properties. However, a major drawback of graphene based devices is that it is difficult to integrate the small flakes of graphene into device architectures. In order to overcome this limitation, we describe a simple procedure for patterning graphene oxide (GO) flakes onto predefined locations of silicon substrates. We exploited the negatively charged surface of GO flakes, and successfully patterned GO flakes onto photolithographically defined positively charged regions on silicon substrates. We demonstrate the simultaneous fabrication of multiple GO flakes device structures by controlling the surface chemistry of substrates. Our procedure for the precise positioning of GO flakes will be an important step in the fabrication of graphene devices.