Prognostic significance of radiological pleuroparenchymal fibroelastosis in Mycobacterium avium complex lung disease: a multicentre retrospective cohort study.

BACKGROUND: Mycobacterium avium complex (MAC) causes chronic respiratory infectious diseases with diverse clinical features and prognoses. Pleuroparenchymal fibroelastosis (PPFE) is a rare disease characterised by pleural fibrosis with subjacent intra-alveolar fibrosis and alveolar septal elastosis, with unique chest high-resolution CT (HRCT) features (radiological PPFE). An association between recurrent respiratory infections and PPFE formation has been hypothesised; however, the clinical significance of PPFE in MAC lung disease remains unclear. METHODS: This retrospective, multicentre study investigated the prevalence of radiological PPFE in patients with MAC lung disease and its association with clinical features and outcomes. Radiological PPFE was diagnosed on the basis of HRCT findings. Prognostic factors were identified using Cox proportional hazards and Fine-Gray models. RESULTS: Of 850 consecutive patients with definite MAC lung disease, 101 (11.9%) exhibited radiological PPFE. Patients with radiological PPFE had unique characteristics, such as lower body mass index, lower survival rate (5-year cumulative survival rate, 63.1% vs 91.7%; p<0.001) and a higher incidence of respiratory-related death (5-year cumulative incidence, 31.1% vs 3.6%; p<0.001), than those without radiological PPFE. In the multivariable analysis, the presence of radiological PPFE was independently associated with all-cause mortality (adjusted HR, 4.78; 95% CI, 2.87 to 7.95; p<0.001) and respiratory-related death (adjusted HR, 3.88; 95% CI, 2.14 to 7.01; p<0.001). INTERPRETATION: This large-scale study demonstrated that in patients with MAC lung disease, radiological PPFE was common, a phenotype associated with unique clinical features and poor prognosis, particularly respiratory-related death. The specific management of this subgroup should be established.

Geriatric Nutritional Risk Index is a predictor of tolerability of antifibrotic therapy and mortality risk in patients with idiopathic pulmonary fibrosis.

BACKGROUND AND OBJECTIVE: Idiopathic pulmonary fibrosis (IPF) is characterized by progressive lung fibrosis of unknown aetiology. Epidemiological studies have suggested that IPF progression may negatively affect nutritional status. Weight loss during antifibrotic therapy is also frequently encountered. The association of nutritional status and outcome has not been fully evaluated in IPF patients. METHODS: This retrospective multicohort study assessed nutritional status of 301 IPF patients receiving antifibrotic therapy (Hamamatsu cohort, n = 151; Seirei cohort, n = 150). Nutritional status was evaluated using the Geriatric Nutritional Risk Index (GNRI). The GNRI was calculated based on body mass index and serum albumin. The relationship between nutritional status and tolerability of antifibrotic therapy as well as mortality was explored. RESULTS: Of 301 patients, 113 (37.5%) had malnutrition-related risk (GNRI < 98). Patients with malnutrition-related risk were older, had increased exacerbations and worse pulmonary function than those without a GNRI status <98. Malnutrition-related risk was associated with a higher incidence of discontinuation of antifibrotic therapy, particulary due to gastrointestinal disturbances. IPF patients with malnutrition-related risk (GNRI < 98) had shorter survival than those without such risk (median survival: 25.9 vs. 41.1 months, p < 0.001). In multivariate analysis, malnutrition-related risk was a prognostic indicator of antifibrotic therapy discontinuation and mortality, independent of age, sex, forced vital capacity, or gender-age-physiology index. CONCLUSION: Nutritional status has significant effects on the treatment and outcome in patients with IPF. Assessment of nutritional status may provide important information for managing patients with IPF.

Impact of antifibrotic therapy on lung cancer development in idiopathic pulmonary fibrosis.

Patients with idiopathic pulmonary fibrosis (IPF) are at a high risk of lung cancer (LC). Antifibrotic therapy slows disease progression and possibly prolongs survival. However, whether antifibrotic therapy affects LC development in patients with IPF remains unknown. This multicentre retrospective study evaluated 345 patients with IPF. The incidence and prevalence of LC were significantly lower in patients with IPF receiving antifibrotic therapy than those not receiving. Subsequently, LC-related mortality was significantly lower in patients with IPF receiving antifibrotic therapy. These results suggest that antifibrotic therapy was possibly associated with a reduced risk of LC development in patients with IPF, which may be partly associated with its survival benefit.

Idiopathic pleuroparenchymal fibroelastosis: three-dimensional computed tomography assessment of upper-lobe lung volume.

BACKGROUND: Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a rare interstitial lung disease characterised by predominant upper-lobe fibrosis involving the pleura and subpleural lung parenchyma. Despite its poor prognosis, there is no consensus on prognostic determinants of iPPFE to date. Because volume loss in the upper lobe is a distinct feature of iPPFE, we hypothesised that the lung volume of the bilateral upper lobes (upper-lobe volume) accurately indicates disease severity and mortality risk in iPPFE patients. METHODS: This retrospective study assessed two cohorts of 132 patients with iPPFE (69 in Hamamatsu cohort; 63 in Seirei cohort) and 45 controls. Each lobe volume was quantitatively measured using three-dimensional computed tomography at the time of iPPFE diagnosis and standardised using predicted forced vital capacity. RESULTS: The standardised upper-lobe volume in iPPFE patients was less than half that of controls, whereas the lower-lobe volume did not decrease. iPPFE patients with lower standardised upper-lobe volume had significantly shorter survival rates than those with higher volume (median survival: 6.08 versus 2.48 years, p<0.001). In multivariate analysis, the lower standardised upper-lobe volume was significantly associated with increased mortality adjusting for age, sex and forced vital capacity (HR 0.939). A composite scoring model, including age, sex and standardised upper-lobe volume, better predicted risk of death than the gender-age-physiology model. CONCLUSION: Assessment of upper-lobe volume provides useful information for managing iPPFE by evaluating disease severity and mortality risk in clinical practice.

Acute exacerbation of rheumatoid arthritis-associated interstitial lung disease: mortality and its prediction model.

BACKGROUND: Patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD), like those with idiopathic pulmonary fibrosis (IPF), might develop an unexpected acute exacerbation (AE)-a rapidly progressing and deadly respiratory decline. Although AE incidence and risk factors in RA-ILD patients are known, their post-AE clinical course remains unknown owing to the rarity of AE-RA-ILD. This multicentre retrospective study evaluated post-AE mortality and prognostic variables in AE-RA-ILD patients and created a mortality prediction model for AE-RA-ILD. METHODS: This research comprised 58 patients with AE-RA-ILD and 96 with AE-IPF (a control disease). Multivariate Cox regression analysis was performed to identify prognostic variables. A prediction model was created with recursive partitioning (decision tree). RESULTS: The post-AE 90-day mortality rate in the overall AE-RA-ILD group was 48.3%; percent predicted forced vital capacity within 12 months before AE onset (baseline %FVC) and PaO2/FiO2 ratio at AE onset (P/F at AE) were independent predictors of mortality. Post-AE 90-day mortality rates were 40.6% and 43.8%, respectively, in AE-RA-ILD and AE-IPF patients propensity score-matched for age, sex, baseline %FVC and P/F at AE (P = 1.0000). In AE-RA-ILD patients, C-indices of baseline %FVC and P/F at AE to predict post-AE 90-day mortality were 0.604 and 0.623, respectively. A decision tree model based on these prognostic factors classified AE-RA-ILD patients into mild, moderate and severe groups (post-AE 90-day mortality rates: 20.8%, 64.0% and 88.9%, respectively; P = 0.0002); the C-index improved to 0.775. CONCLUSIONS: Post-AE mortality was high in AE-RA-ILD patients similar to AE-IPF patients. The discovered prognostic factors and our mortality prediction model may aid in the management of AE-RA-ILD patients.

Impact of end-of-life respiratory modalities on quality of dying and death and symptom relief in patients with interstitial lung disease: a multicenter descriptive cross-sectional study.

BACKGROUND: Respiratory modalities applied at the end of life may affect the burden of distressing symptoms and quality of dying and death (QODD) among patients with end-stage interstitial lung disease (ILD); however, there have been few studies into respiratory modalities applied to these patients near death. We hypothesized that high-flow nasal cannula (HFNC) might contribute to improved QODD and symptom relief in patients with end-stage ILD. OBJECTIVES: This multicenter study examined the proportion of end-of-life respiratory modalities in a hospital setting and explored its impact on QODD and symptom relief among patients dying with ILD. METHODS: Consecutive patients with ILD who died in four participating hospitals in Japan from 2015 to 2019 were identified and divided into four groups according to end-of-life respiratory modality: conventional oxygen therapy (COT), HFNC, non-invasive ventilation (NIV), and invasive mechanical ventilation (IMV). In addition, a mail survey was performed to quantify the QODD and symptom relief at their end of life from a bereaved family's perspective. QODD and symptom relief were quantified using the Good Death Inventory (GDI) for patients with a completed bereavement survey. The impact of end-of-life respiratory modalities on QODD and symptom relief was measured by multivariable linear regression using COT as a reference. RESULTS: Among 177 patients analyzed for end-of-life respiratory modalities, 80 had a completed bereavement survey. The most common end-of-life respiratory modality was HFNC (n = 76, 42.9%), followed by COT (n = 62, 35.0%), NIV (n = 27, 15.3%), and IMV (n = 12, 6.8%). Regarding the place of death, 98.7% of patients treated with HFNC died outside the intensive care unit. Multivariable regression analyses revealed patients treated with HFNC had a higher GDI score for QODD [partial regression coefficient (B) = 0.46, 95% CI 0.07-0.86] and domain score related to symptom relief (B = 1.37, 95% CI 0.54-2.20) than those treated with COT. CONCLUSION: HFNC was commonly used in patients with end-stage ILD who died in the hospital and was associated with higher bereaved family ratings of QODD and symptom relief. HFNC might contribute to improved QODD and symptom relief in these patients who die in a hospital setting.

Quality of dying and death in patients with interstitial lung disease compared with lung cancer: an observational study.

BACKGROUND: There is limited knowledge regarding the quality of dying and death (QODD) and end-of-life interventions in patients with interstitial lung disease (ILD). Hence, differences in QODD and end-of-life interventions between patients with ILD and those with lung cancer (LC) remain poorly understood. METHODS: The primary aim of this study was to explore the differences in QODD and end-of-life interventions among patients dying with ILD versus those dying with LC. We performed a mail survey to quantify the QODD of a bereaved family's perspective using the Good Death Inventory (GDI) score. Moreover, we examined the end-of-life interventions by medical chart review. RESULTS: Of 361 consecutive patients analysed for end-of-life interventions, 167 patients whose bereaved families completed questionnaires were analysed for QODD. Patients with ILD had lower GDI scores for QODD than those with LC (p=0.04), particularly in domains related to 'physical and psychological distress relief' and 'prognosis awareness and participation in decision making' (p=0.02, respectively). In end-of-life interventions, patients with ILD were less likely to receive specialised palliative care services (8.5% vs 54.3%; p<0.001) and opioids (58.2% vs 73.4%; p=0.003). Additionally, lower frequencies of participation of patients with ILD in end-of-life discussion were also observed (40.8% vs 62.4%; p=0.007). CONCLUSION: Patients with ILD had lower QODD and poorer access to palliative care and decision making than those with LC. Additional efforts to improve QODD in patients with ILD, particularly in symptom relief and decision-making processes, are urgently warranted.

Experience using mTOR inhibitors for subependymal giant cell astrocytoma in tuberous sclerosis complex at a single facility.

BACKGROUND: Subependymal giant cell astrocytoma (SEGA) is occasionally seen in tuberous sclerosis complex (TSC). Two main options are currently available for treating SEGA: surgical resection or pharmacotherapy using mammalian target of rapamycin inhibitors (mTORi). We hypothesized that opportunities for surgical resection of SEGA would have reduced with the advent of mTORi. METHODS: We retrospectively reviewed the charts of patients treated between August 1979 and July 2020, divided into a pre-mTORi era group (Pre-group) of patients treated before November 2012, and a post-mTORi era group (Post-group) comprising patients treated from November 2012, when mTORi became available in Japan for SEGA. We compared groups in terms of treatment with surgery or mTORi. We also reviewed SEGA size, rate of acute hydrocephalus, recurrence of SEGA, malignant transformation and adverse effects of mTORi. RESULTS: In total, 120 patients with TSC visited our facility, including 24 patients with SEGA. Surgical resection was significantly more frequent in the Pre-group (6 of 7 patients, 86 %) than in the Post-group (2 of 17 patients, 12 %; p = 0.001). Acute hydrocephalus was seen in 1 patient (4 %), and no patients showed malignant transformation of SEGA. The group treated using mTORi showed significantly smaller SEGA compared with the group treated under a wait-and-see policy (p = 0.012). Adverse effects of pharmacotherapy were identified in seven (64 %; 6 oral ulcers, 1 irregular menstruation) of the 11 patients receiving mTORi. CONCLUSIONS: The Post-group underwent surgery significantly less often than the Pre-group. Since the treatment option to use mTORi in the treatment of SEGA in TSC became available, opportunities for surgical resection have decreased in our facility.

Cause of mortality and sarcopenia in patients with idiopathic pulmonary fibrosis receiving antifibrotic therapy.

BACKGROUND AND OBJECTIVE: Recent research has highlighted the fundamental role of sarcopenia, characterized by loss of skeletal muscle mass and strength, with a risk of poor outcomes. AFT preserves lung function by preventing the annual decline in FVC and is associated with improved outcomes in patients with IPF. However, altered cause of death and prognostic implications of sarcopenia in patients with IPF receiving AFT remain unknown. METHODS: This study comprised two cohorts of patients with IPF receiving AFT, historical cohort of IPF patients without AFT and controls. The cause of mortality was compared with a historical cohort. Sarcopenia was assessed by measuring the ESMCSA and ESMMA via CT. RESULTS: Patients with IPF had smaller ESMCSA and lower ESMMA but similar BMI than controls, suggesting patients with IPF had skeletal muscle loss without any obvious body weight loss. The most common cause of mortality in patients receiving AFT was chronic respiratory failure, accounting for approximately 60%, and decreased proportions of LC were found. Subsequently, low ESMCSA was an independent prognostic factor associated with worse survival rates. Furthermore, combined assessment of ESMCSA , %FVC predicted and BMI values provided clear prognostic distinction. CONCLUSION: Patients with IPF receiving AFT showed skeletal muscle loss without obvious weight loss. These patients mostly died by chronic respiratory failure, and skeletal muscle wasting has prognostic significance, suggesting that preventing sarcopenia as well as preserving lung function are important for managing these patients.

Pneumothorax in Patients with Idiopathic Pleuroparenchymal Fibroelastosis: Incidence, Clinical Features, and Risk Factors.

BACKGROUND: Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare form of idiopathic interstitial pneumonia that is characterized by predominantly upper lobe pleural and subpleural lung parenchymal fibrosis. Pneumothorax is one of the major respiratory complications in PPFE patients; however, its clinical features are poorly understood. OBJECTIVE: We aimed to investigate the complication of pneumothorax in patients with idiopathic PPFE. METHODS: A retrospective multicenter study involving 89 patients who had been diagnosed with idiopathic PPFE was conducted. We investigated the cumulative incidence, clinical features, and risk factors of pneumothorax after the diagnosis of idiopathic PPFE. RESULTS: Pneumothorax developed in 53 patients (59.6%) with 120 events during the observation period (41.8 ± 35.0 months). The cumulative incidence of pneumothorax was 24.8, 44.9, and 53.9% at 1, 2, and 3 years, respectively. Most events of pneumothorax were asymptomatic (n = 85; 70.8%) and small in size (n = 92; 76.7%); 30 patients (56.6%) had recurrent pneumothorax. Chest drainage was required in 23 pneumothorax events (19.2%), and a persistent air leak was observed in 13 (56.5%). Patients with pneumothorax were predominantly male and frequently had pathological diagnoses of PPFE and prior history of pneumothorax and corticosteroid use; they also had significantly poorer survival than those without pneumothorax (log-rank test; p = 0.001). Multivariate analysis revealed that a higher residual volume/total lung capacity ratio was significantly associated with the development of pneumothorax after the diagnosis. CONCLUSION: Pneumothorax is often asymptomatic and recurrent in patients with idiopathic PPFE, leading to poor outcomes in some cases.

Switching antifibrotics in patients with idiopathic pulmonary fibrosis: a multi-center retrospective cohort study.

BACKGROUND: Currently, there are two antifibrotics used to treat idiopathic pulmonary fibrosis (IPF): pirfenidone and nintedanib. Antifibrotics slow disease progression by reducing the annual decline of forced vital capacity (FVC), which possibly improves outcomes in IPF patients. During treatment, patients occasionally switch antifibrotic treatments. However, prognostic implication of changing antifibrotics has not yet been evaluated. METHODS: This multi-center retrospective cohort study examined 262 consecutive IPF patients who received antifibrotic therapy. Antifibrotic agents were switched in 37 patients (14.1%). The prognoses were compared between the patient cohort that switched antifibrotics (Switch-IPF) and those without (Non-Switch-IPF) using propensity-score matched analyses. RESULTS: The median period between the initiation of antifibrotic therapy and the drug switch was 25.8 (12.7-35.3) months. The most common reasons for the switch were disease progression (n = 17) followed by gastrointestinal disorders (n = 12). Of the 37 patients that switched antifibrotics, only eight patients disrupted switched antifibrotics by their adverse reactions. The overall prognosis of the Switch-IPF cohort was significantly better than the Non-Switch-IPF cohort (median periods: 67.2 vs. 27.1 months, p < 0.0001). In propensity-score matched analyses that were adjusted to age, sex, FVC (%), history of acute exacerbation, and usage of long-term oxygen therapy, the Switch-IPF cohort had significantly longer survival times than the Non-Switch-IPF group (median 67.2 vs. 41.3 months, p = 0.0219). The second-line antifibrotic therapy showed similar survival probabilities than those in first-line antifibrotic therapy in multistate model analyses. CONCLUSION: Switching antifibrotics is feasible and may improve prognosis in patients with IPF. A further prospective study will be required to confirm clinical implication of switching the antifibrotics.

Toward Citizen Dialogue-led Environmental Governance: An Exploratory Case Study in Post-Fukushima Japan.

This paper reports on an exploratory case study to help facilitate a culture of dialogue in Japan. There is an emphasis on proposing methods for polyphonic dialogue among citizens, and between citizens and experts, to effectively manage the environment. This paper argues that a culture of dialogue is essential to pluralistic participatory environmental governance. A random sampling-based citizen dialogue-involving experts and citizens-regarding radioactive waste disposal was held in Japanese cities. Three proposed methods-politeness-based facilitation dialogue, evidence-based and position-explicit presentations by experts with differing views and experts reflecting in tandem with citizens engaged in dialogue-might lead to enhanced positive attitudes toward dialogue with others holding different views, as well as better internal self-deliberation. Attitudes for dialogue were measured empirically. The current research suggests that explicit treatment of pluralistic positions and views among citizens and experts would be a key factor for quality social learning and resilience for uncertainty.

Efficacy of corticosteroid and intravenous cyclophosphamide in acute exacerbation of idiopathic pulmonary fibrosis: A propensity score-matched analysis.

BACKGROUND AND OBJECTIVE: Acute exacerbation (AE) is a leading cause of death in patients with idiopathic pulmonary fibrosis (IPF). Although optimal treatment for AE-IPF remains unclear, high-dose corticosteroids (CS) with/without immunosuppressants, including intravenous cyclophosphamide (IVCY), are often used as empirical therapy. However, the survival benefit of adding IVCY to CS therapy is unknown. We investigated the efficacy of this therapy in patients with AE-IPF. METHODS: Overall, 102 consecutive patients with IPF with a first idiopathic AE were included. Post-AE survival rates and treatment safety were retrospectively assessed. Efficacy of CS + IVCY therapy for the first AE was compared with that of CS monotherapy using a propensity score-matched analysis. RESULTS: The post-AE 90-day survival rate of the entire cohort was 64.7%. On the basis of the propensity scores, 26 matched patient pairs were made. Characteristics of matched patients with AE-IPF treated with CS (matched CS group) and those with CS + IVCY (matched CS + IVCY group) were well balanced. No significant between-group differences were observed in post-AE 90-day survival rates (84.6% vs 76.9%; P = 0.70), cumulative survival rates (P = 0.57 by log-rank test) or incidence of adverse events ≥ CTCAE (Common Terminology Criteria for Adverse Events) v5.0 grade 3 (61.5% vs 65.4%; P = 1.00). CONCLUSION: The propensity score-matched analysis demonstrated that compared with CS monotherapy, CS + IVCY therapy did not significantly improve post-AE survival in patients with AE-IPF. Further studies are warranted to assess the efficacy of CS + IVCY therapy for AE-IPF.

Persistent impairment on spirometry in chronic eosinophilic pneumonia: A longitudinal observation study (Shizuoka-CEP study).

BACKGROUND: Chronic eosinophilic pneumonia (CEP) is characterized by the accumulation of eosinophils in the lung with unknown etiology. Although systemic corticosteroid administration leads to dramatic improvement, nearly half the patients with CEP experience relapse and some develop persistent impairment of pulmonary function. However, predictive factors for this persistent impairment have not been determined. OBJECTIVE: To investigate the occurrence of persistent impairment of pulmonary function in CEP and determine its predictive factors. METHODS: This observational study consisted of 133 consecutive patients with CEP who were followed for longer than 1 year. Spirometry was performed at the time of diagnosis and at follow-up. RESULTS: During the observational period (6.1 ± 4.1 years), relapse occurred in 75 patients (56.4%). Remarkably, 42 patients (31.6%) had a persistent pulmonary function defect (27 obstructive, 10 restrictive, and 4 obstructive and restrictive cases) at the last evaluation. Logistic analyses showed that the relapse was associated with neither persistent obstructive nor restrictive defects. Persistent obstructive defect was significantly associated with the comorbidity of asthma and obstructive defect at the initial CEP diagnosis, whereas persistent restrictive defect was significantly related to reticulation at high-resolution computer tomography and restrictive defect at diagnosis. CONCLUSION: Persistent impairment of pulmonary function is common in CEP. Concurrent asthma and obstructive defects at diagnosis were predictors for persistent obstructive impairments, whereas reticulation at high-resolution computer tomography and restrictive defect at diagnosis predicted persistent restrictive impairment. Attention should be paid to these persistent impairments in the management of CEP. TRIAL REGISTRATION: http://www.umin.ac.jp/ctr/index-j.htm Identifier: UMIN000019092 (principal investigator, Takafumi Suda, MD, PhD).

Political and environmental attitude toward participatory energy and environmental governance: A survey in post-Fukushima Japan.

The Great East Japan Earthquake and subsequent Fukushima nuclear accident triggered citizen dialogue on energy and environmental management in Japan. However, an international survey has shown that the willingness of Japanese citizens to participate in political and social activities has declined since the Fukushima accident. Employing an internet-based survey in four urban or nuclear power plant-hosting prefectures in Japan, this study examines the willingness to participate in random sampling deliberation on post-disaster energy and environmental policy. It focuses on the effects of political, environmental, and social attitudes towards willingness to participate in citizen dialogue. The survey shows around 40% of respondents may participate in energy and environmental deliberation. Statistical analysis reveals that environmental consciousness raises the propensity to participate in deliberation, while political obedience and social hesitation decreases the will to participate. The effect of environmental attitudes is larger than that of political and social attitudes. The survey also finds that governmental response to deliberation, i.e., information generation and disclosure based on requests from citizen dialogue, encourages participation in deliberation in a conservative prefecture, when citizen dialogue is held at the national level. Random sampling deliberation opens a new mode of environmental governance regardless of local political and social characteristics.

[A case of esophageal and intestinal tuberculosis that occurred during treatment of rheumatoid arthritis with etanercept].

An 88-year-old woman with rheumatoid arthritis who had started etanercept treatment in July 2011 was referred to our hospital in February 2012 for right-sided pleural effusion. Chest computed tomography showed right pleural effusion, partial swelling of a calcified mediastinal lymph node, and mid-esophageal thickening of the mucosal wall. Gastroendoscopy showed mid-esophageal ulceration. Histological examination of biopsy specimens from this ulceration revealed noncaseating granulomas with Langhans giant cells. Ziehl-Neelsen staining of this section was positive for acid-fast bacilli. Polymerase chain reaction analysis of gastric juice was positive for Mycobacterium tuberculosis; we therefore diagnosed the patient with esophageal tuberculosis. However, since abdominal computed tomography showed swelling of mesenteric lymph nodes, we also suspected intestinal tuberculosis. Colonoscopy showed multiple ileal erosions; histological analyses of biopsied specimens revealed granulomas with Langhans giant cells, similar to the esophageal findings. We finally diagnosed the patient with both esophageal and intestinal tuberculosis. After anti-tuberculosis treatment, the right pleural effusion disappeared and the abdominal lesions improved. Although mycobacterial involvement of both the esophagus and intestine is rare in immunocompromised and immunocompetent hosts, differential diagnosis of these diseases is likely to become more important.

Prognostic significance of body mass index and weight loss in patients with idiopathic pleuroparenchymal fibroelastosis.

BACKGROUND AND AIM: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare form of idiopathic interstitial pneumonias; its physical characteristics include a slender build with platythorax and progressive weight loss. However, the clinical significance of body mass index (BMI) and weight loss remains unclear in patients with IPPFE. Therefore, we aimed to clarify the association between baseline BMI, weight loss after diagnosis, and the prognosis of patients with IPPFE. METHODS: This retrospective study included 71 patients diagnosed with IPPFE at our institution between 2005-2021. BMI at diagnosis was classified into three: underweight (<18.5 kg/m2), normal weight (≥18.5 to <25.0 kg/m2), or overweight (≥25.0 kg/m2). An annual rate of weight change after the diagnosis was evaluated, and ≥5% per year decrease was defined as a significant weight loss. We investigated clinical features and prognosis based on baseline BMI and weight loss. RESULTS: Of the 71 patients, 48 (67.6%) and 23 (32.4%) were classified as underweight and normal weight, respectively, and none were overweight. Significant weight loss occurred in 24 (33.8%) patients, and they tended to have more cases of dyspnea and had significantly older age, lower BMI, higher rates of co-existence of lower-lobe interstitial lung disease, lower pulmonary function test results and higher incidence of pneumothorax after the diagnosis than those without weight loss. Patients with BMI <18.5 kg/m2 and those with weight loss had a significantly worse prognosis than those with BMI ≥18.5 kg/m2 or those without weight loss, respectively (p=0.005, p<0.001). Multivariate analysis revealed that low BMI and weight loss were independent poor prognostic factors. CONCLUSIONS: Low BMI and weight loss are associated with poor prognosis in patients with IPPFE.

An Adult Case of Idiopathic Pulmonary Hemosiderosis Associated with Pulmonary Fibrosis and Emphysematous Change.

A 48-year-old woman was admitted to our hospital with acute respiratory failure. Chest computed tomography showed ground-glass opacity and patchy emphysematous lesions in both lungs. Corticosteroid therapy was effective; however, the disease worsened with the tapering of corticosteroids. Bronchoalveolar lavage revealed hemosiderin-laden macrophages, and video-assisted thoracic surgery showed diffuse interstitial fibrosis with diffuse alveolar hemorrhage (DAH). There was no evidence of vasculitis nor autoimmune diseases. This patient was diagnosed with idiopathic pulmonary hemosiderosis (IPH) that progressed to end-stage pulmonary fibrosis despite treatment. Autopsy demonstrated DAH with pulmonary fibrosis and emphysematous change, suggesting IPH-related pulmonary lesions.

Pneumococcal vertebral osteomyelitis at three teaching hospitals in Japan, 2003-2011: analysis of 14 cases and a review of the literature.

BACKGROUND: Pneumococcal vertebral osteomyelitis (PVO) is a rare disease whose clinical characteristics have not been clarified. This study aimed to investigate the clinical features and outcomes of patients with PVO. METHODS: We retrospectively evaluated all adult patients diagnosed with PVO at three teaching hospitals in Japan from January 2003 to December 2011. All cases were identified through a review of the medical records of patients with invasive pneumococcal disease (IPD). RESULTS: Among 208 patients with IPD, we identified 14 with PVO (6.4%; 95% CI, 3.5-10%). All 14 patients (nine male, five female; median age 69 years) had acquired PVO outside the hospital and had no recent history of an invasive procedure or back injury. Five patients (36%) had diabetes mellitus, and four (29%) had heavy alcohol intake. Fever (n = 13; 93%) or back pain/neck pain (n = 12; 86%) were present in most patients. The lumbar spine was affected in nine patients (64%) but the cervical spine was the site of infection in four patients (29%). All patients except one had a positive blood culture for Streptococcus pneumoniae, and there were no distant infected sites in most patients (n = 10; 71%). Intravenous beta-lactam therapy was initiated within 1 week after the onset of symptoms in 11 patients (79%). No patients died within 30 days, but one patient died from aspiration pneumonia on day 37 after admission. CONCLUSIONS: PVO was relatively common among adult patients with IPD, and mortality was low in this study. S. pneumoniae may be the causative pathogen of vertebral osteomyelitis, especially among community-onset cases without a history of invasive procedures or back injury.

A retrospective cohort study of panipenem/betamipron for adult pneumococcal bacteremia at three teaching hospitals in Japan.

Panipenem/betamipron (PAPM/BP) may be highly effective for life-threatening Streptococcus pneumoniae infection. However, the efficacy of PAPM/BP for S. pneumoniae infections has not been compared with that of other antimicrobial agents. We retrospectively compared PAPM/BP with other carbapenems for treatment of life-threatening infections in newly hospitalized adults with pneumococcal bacteremia. Clinical information for cases of pneumococcal bacteremia was collected from three teaching hospitals in Japan from January 2003 to December 2010. In total, 17 patients who received PAPM/BP therapy and 34 treated with other carbapenems (27 with meropenem, 4 with imipenem/cilastatin, and 3 with biapenem) were identified. The mean age (71 vs. 70 years old), sex distribution (women, 29 vs. 21 %), Charlson comorbidity index (CCI) (1.5 vs. 1.6), and rates of septic shock (29 vs. 38 %), and meningitis (5.9 vs. 8.8 %) did not differ significantly between the two groups. The inpatient mortality rates were lower in the PAPM/BP group (12 vs. 44 %, p = 0.03). Multiple logistic regression analysis adjusted for age, sex, CCI, and severe sepsis/septic shock showed that use of other carbapenems was associated with higher in-hospital mortality, with an odds ratio of 6.922 (95 % CI, 1.171-40.92) compared to PAPM/BP therapy. Initial PAPM/BP therapy might have a therapeutic advantage over other carbapenems in treatment of severe Streptococcus pneumoniae infections.