A 66-year-old woman was found to have abnormal shadows on a chest radiograph at a previous hospital 4 years ago, which led to a diagnosis of lung adenocarcinoma, cT2aN1M1b stage IVA. First-line treatment included carboplatin and paclitaxel plus thoracic radiotherapy and stereotactic radiation therapy for brain metastases. The patient later underwent second-line pemetrexed treatment, followed by third-line nivolumab, fourth-line docetaxel and bevacizumab, fifth-line tegafur-gimeracil-oteracil, and sixth-line gemcitabine.
View Article and Find Full Text PDFApproximately one-third of fibrosing interstitial lung diseases exhibit progressive pulmonary fibrosis (PPF), a clinicopathological condition distinct yet resembling idiopathic pulmonary fibrosis (IPF). PPF in ANCA-positive ILD (ANCA-ILD) is poorly documented. To clarify incidence, predictors of PPF in ANCA-ILD, and their prognostic impact, 56 patients with ANCA-ILD were followed for ≥ 1 year (April 2004 to April 2021).
View Article and Find Full Text PDFBackground: The ATS/JRS/ALAT Guidelines for the Diagnosis of Hypersensitivity Pneumonitis (GL for HP) were published in 2020. Humidifier lung and summer-type HP are forms of HP, but it is unclear whether they can be diagnosed using GL for HP. This study examined the level of confidence where humidifier lung and summer-type HP can be diagnosed with GL for HP.
View Article and Find Full Text PDFBackground: The indication for and the timing of surgery in patients with pleural infection remains unclear. Determining the need for surgery in patients with pleural infection may help in the early consultation of surgeons.
Methods: Data of 167 consecutive patients with pleural infection were retrospectively reviewed.
Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is often fatal. A straightforward staging system for AE-IPF would improve prognostication, guide patient management, and facilitate research. The aim of study is to develop a multidimensional prognostic AE-IPF staging system that uses commonly measured clinical variables.
View Article and Find Full Text PDFTOLLIP polymorphism has been implicated in the development and prognosis of idiopathic pulmonary fibrosis (IPF), mainly in whites. However, ethnic differences in the characteristics of other interstitial pneumonia (non-IPF) subtypes are unclear. We evaluated the association between the rs3750920 genotype and the clinical characteristics of Japanese patients with fibrosing interstitial lung diseases (ILD).
View Article and Find Full Text PDFBackground: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a fatal event that can occur during the clinical course of idiopathic pulmonary fibrosis (IPF). Although data from clinical trials suggest that the antifibrotic agents pirfenidone and nintedanib might reduce the risk of AE-IPF, the incidence of AE-IPF in patients receiving antifibrotic agents in clinical settings is unclear.
Objectives: To determine the incidence of AE-IPF in patients receiving antifibrotic agents and compare AE-IPF frequency in patients receiving pirfenidone and nintedanib.
Background: The associations of serum monomeric periostin (M-PN) level and serial change in M-PN with acute exacerbation of chronic fibrosing interstitial pneumonia (AE-FIP) are unclear.
Methods: We prospectively measured serum M-PN level from onset of AE to day 14 in 37 patients with AE-FIP and evaluated its association with outcome. To determine localization of periostin expression, immunohistochemical staining of pathological lung tissue from autopsy cases of AE-IPF was evaluated.
Background: There are no established therapeutic options available for idiopathic pleuroparenchymal fibroelastosis (IPPFE) apart from supportive care and lung transplantation. Furthermore, it is known that IPPFE with a usual interstitial pneumonia (UIP) pattern and lower lobe predominance is a disease entity distinct from idiopathic pulmonary fibrosis (IPF). To our knowledge, few studies are available that report on the efficacy of antifibrotic agents for IPPFE with UIP.
View Article and Find Full Text PDFIntroduction: Surgery for acute empyema is associated with postoperative complications and relapse. Establishing a predictor for postoperative complications may improve prognosis.
Objectives: To demonstrate undernutrition as a predictor of complications after surgery for acute empyema.
Background: Ultrasonic humidifier lung is a rare form of hypersensitivity pneumonitis (HP), and its clinical and radiological features are unclear. This study examined the clinical and radiological characteristics of humidifier lung.
Methods: Data from 18 patients with humidifier lung (mean age, 67.
Background: Pirfenidone treatment can slow decline in forced vital capacity (FVC) in idiopathic pulmonary fibrosis (IPF). However, its effects for usual interstitial pneumonia (UIP) with pleuroparenchymal fibroelastosis-like lesions (UIP+PPFELL) and UIP with nonspecific interstitial pneumonia (UIP+NSIP) are unclear.
Objectives: The aim of study is to assess pirfenidone effectiveness for UIP+PPFELL and UIP+NSIP.
Sarcoidosis Vasc Diffuse Lung Dis
August 2020
Background: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is often fatal. Although pharmacological treatments have been studied, outcomes remain poor. This study evaluated the effectiveness of pharmacological treatments for AE-IPF.
View Article and Find Full Text PDFBackground: Recent clinical trials demonstrated the benefits of several guided-bronchoscopy technologies for the diagnosis of peripheral pulmonary lesions (PPLs). However, introduction of these technologies is expensive. Therefore, in clinical practice, these are unavailable in many hospitals.
View Article and Find Full Text PDFBackground And Objective: High-mobility group box 1 (HMGB1) protein is important in acute lung injury. However, the role of HMGB-1 in acute exacerbation of fibrosing interstitial pneumonia (AE-FIP) has not been adequately studied.
Methods: We prospectively measured serum HMGB1 level from disease onset to day 7 in 36 patients with AE-FIP6 patients had missing data because of early death (within 7 days).
Background: Atypical idiopathic pulmonary fibrosis (IPF) including multiple cysts or markedly atelectatic induration in upper lung predominance occasionally can confirm the diagnosis of IPF through a multidisciplinary discussion (MDD) between clinician, radiologist and, pathologist in clinical practice. The aim of this study was to clarify the differences in clinico-radiological characteristics and the efficacy of anti-fibrotic agents between atypical IPF and typical IPF.
Methods: We retrospectively evaluated the differences in clinico-radiological characteristics between patients with atypical IPF (n=44) and those with typical IPF (n=87) and examined efficacy of anti-fibrotic agents in atypical IPF.
Background And Objectives: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is an often-fatal condition associated with endothelial damage and abnormalities of the coagulation system. Recombinant human soluble thrombomodulin (rhTM) has anti-inflammatory effects and regulates the coagulation pathway. This study evaluated the effectiveness of rhTM for the treatment of AE-IPF.
View Article and Find Full Text PDFObjective An acute exacerbation (AE) of idiopathic pulmonary disease (IPF) represents a life threatening condition. The activities of daily living (ADL) and quality of life of patients who survive an AE of IPF (AE-IPF) are often diminished. However, the association between AE-IPF and the ADL has yet to be evaluated.
View Article and Find Full Text PDFBackground: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a rapid and ultimately fatal condition, and no effective treatment has been established. Pirfenidone has antifibrotic effects in IPF; however, its efficacy for AE-IPF is unclear.
Objectives: To evaluate the efficacy of pirfenidone for AE-IPF.
The strong association between myositis and malignancy has been well recognised. Cancer-associated myositis (CAM) is thought to be a cross-reaction to regenerating muscle tissue similar to tumour antigen. We report a case of CAM due to oesophageal adenocarcinoma arising in Barrett's oesophagus without elevation of myogenic enzymes, diagnosed by MRI and repeated endoscopy.
View Article and Find Full Text PDFAntiphospholipid syndrome (APS) is clinically characterized by arterial or venous thrombosis; however, non-thromboembolic lung manifestations, such as diffuse alveolar hemorrhage (DAH), have also been previously reported. DAH is relatively common in APS patients with systemic lupus erythematosus, although it is rare in primary APS. We encountered a 78-year-old man who presented with hemoptysis and dyspnea.
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