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Interstitial lung disease (ILD) is a common manifestation of polymyositis (PM), dermatomyositis (DM), and clinically amyopathic dermatomyositis (CADM); however, little is known about the factors influencing the prognosis for PM/DM/CADM-associated ILD. (PM/DM/CADM-ILD). The aim of the present study is to assess prognostic factors for PM/DM/CADM-ILD. The clinical features and survival of 114 consecutive patients diagnosed with PM/DM/CADM-ILD (39 men and 75 women; median age, 56 years) were analyzed retrospectively. The study group included 30 PM-associated ILD, 41 DM-associated ILD, and 43 CADM-associated ILD cases. The clinical presentation of ILD was acute/subacute form in 59 patients (51.8%) and chronic form in 55 patients (48.2%). The major pulmonary symptoms were dyspnea, cough, and fever. High-resolution computed tomography frequently revealed ground-glass opacities, traction bronchiectasis, and consolidation. Most of the patients were treated with corticosteroids or corticosteroids in combination with immunosuppressive agents. The all-cause mortality was 27.2%. Acute/subacute form, % forced vital capacity (FVC), age, % of neutrophils in bronchoalveolar lavage (BAL) fluid, and a diagnosis of CADM (vs. PM) were significantly associated with poor outcome in univariate Cox proportional hazards models. Multivariate Cox proportional hazards analysis validated acute/subacute ILD, %FVC, age, and diagnosis of CADM (vs. PM) as significant predictors of overall mortality. Patients with acute/subacute ILD had a much lower survival rate than those with the chronic form (p<0.001). Patients with CADM-ILD had a lower survival rate than those with PM-ILD (p = 0.034). Acute/subacute form, older age, lower level of FVC and diagnosis of CADM predict poor outcome in PM/DM/CADM-ILD.

Overlap of asthma and COPD has attracted attention recently. We aimed to clarify physiological and morphological differences of the airways between COPD and asthma–COPD overlap (ACO). Respiratory resistance and reactance and three-dimensional computed tomography data were evaluated in 167 patients with COPD. Among them, 43 patients who fulfilled the diagnosis of asthma were defined as having ACO. Among 124 patients with COPD without ACO, 86 with a comparable smoking history and airflow limitation as those with ACO were selected using propensity score matching (matched COPD). The intraluminal area (Ai) and wall thickness (WT) of third- to sixth-generation bronchi were measured and adjusted by body surface area (BSA; Ai/BSA and WT/√BSA, respectively). Patients with ACO had higher respiratory resistance and reactance during tidal breathing, but a smaller gap between the inspiratory and expiratory phases, compared with matched patients with COPD. Patients with ACO had a greater WT/√BSA in third- to fourth-generation bronchi, smaller Ai/BSA in fifth- to sixth-generation bronchi, and less emphysematous changes than did matched patients with COPD. Even when patients with ACO and those with COPD have a comparable smoking history and fixed airflow limitation, they have different physiological and morphological features of the airways.

BackgroundLipids have immunomodulatory functions and the potential to affect cancer immunity.MethodsThe associations of pretreatment serum cholesterol and long-chain fatty acids with the objective response rate (ORR), progression-free survival (PFS), and overall survival (OS) were evaluated in 148 patients with non-small cell lung cancer who received nivolumab.ResultsWhen each lipid was separately evaluated, increased low-density lipoprotein (LDL)-cholesterol (P < 0.001), high-density lipoprotein (HDL)-cholesterol (P = 0.014), total cholesterol (P = 0.007), lauric acid (P = 0.015), myristic acid (P = 0.022), myristoleic acid (P = 0.035), stearic acid (P = 0.028), linoleic acid (P = 0.005), arachidic acid (P = 0.027), eicosadienoic acid (P = 0.017), dihomo-γ-linolenic acid (P = 0.036), and behenic acid levels (P = 0.032) were associated with longer PFS independent of programmed death ligand 1 (PD-L1) expression. Meanwhile, increased LDL-cholesterol (P < 0.001), HDL-cholesterol (P = 0.009), total cholesterol (P = 0.036), linoleic acid (P = 0.014), and lignoceric acid levels (P = 0.028) were associated with longer OS independent of PD-L1 expression. When multiple lipids were evaluated simultaneously, LDL-cholesterol (P = 0.003), HDL-cholesterol (P = 0.036), and lauric acid (P = 0.036) were independently predictive of PFS, and LDL-cholesterol (P = 0.008) and HDL-cholesterol (P = 0.031) were predictive of OS. ORR was not associated with any serum lipid.ConclusionsBased on the association of prolonged survival in patients with increased serum cholesterol and long-chain fatty acid levels, serum lipid levels may be useful for predicting the efficacy of immune checkpoint inhibitor therapy.

Before the introduction of antifibrotic therapy, patients with familial pulmonary fibrosis (FPF) were reported to have a higher mortality risk than those with sporadic disease. Genetic predisposition plays an important role in the development of interstitial lung disease (ILD), including idiopathic pulmonary fibrosis (IPF). The 2023 European Respiratory Society consensus statement defined FPF and recommended antifibrotic drugs for treatment; however, their efficacy in FPF remains unclear. We conducted a retrospective multicohort study of 280 patients with IPF receiving antifibrotic therapy (exploratory cohort, n = 141; validation cohort, n = 139). FPF was defined as fibrotic ILD in at least two first- or second-degree relatives. We examined tolerability, causes of drug discontinuation, incidence of acute exacerbation (AE), and mortality. Among all patients, 45 (16.1%) had FPF–IPF. These patients were younger and included a lower proportion of men compared with sporadic IPF. No significant differences were observed between groups in drug tolerability, discontinuation causes, AE incidence, or mortality. Multivariate analyses adjusting for gender–age–physiology index confirmed that FPF was not associated with increased mortality. In conclusion, under antifibrotic therapy, FPF–IPF and sporadic IPF demonstrated comparable tolerability, risk of AE, and mortality.

Background Chemotherapy-induced nausea and vomiting is a common adverse event of cancer treatments. Despite prophylactic antiemetic treatment, nausea remains a particular problem. We aimed to identify risk factors and clarify the usefulness of olanzapine for the control of carboplatin-induced nausea. Methods This was a pooled analysis of data from a single-arm, open-label, phase II trial and a prospective, randomized, double-blind, placebo-controlled phase III trial. We combined data from two trials with similar inclusion and exclusion criteria and treatment schedules. Chemotherapy-naïve patients aged ≥ 20 years with solid cancers who were scheduled to receive a first course of carboplatin-containing chemotherapy were enrolled. Patients in the olanzapine and placebo groups received olanzapine 5 mg or placebo, respectively, in combination with the neurokinin-1 (NK 1 ) receptor antagonist aprepitant, a 5-hydroxytryptamine-3 (5-HT 3 ) receptor antagonist, and dexamethasone. Olanzapine was administered on days 1–4 after the evening meal. The primary endpoint was the proportion of patients without nausea in the overall phase (0–120 h). Intergroup differences and their 95% confidence intervals (CIs) were estimated in each phase and population. Univariable and multivariable logistic regression analyses were performed to determine the risk factors associated with nausea, appetite loss, and complete response (no vomiting and no rescue medication use) rate. Odds ratios (ORs), 95% CIs, and p-values for each background factor were calculated. Results A total of 388 patients were evaluated (79.4% male, median age 72 years), including 208 patients in the olanzapine group. Overall, 87.5% in the olanzapine group and 75.0% in the placebo group were free of nausea (intergroup difference 12.5%, 95% CI, 4.7–20.3, p  = 0.002), and the difference in proportion of patients reporting no appetite loss was 20.6% (95% CI, 11.5–29.6, p  < 0.001). The overall complete response rates were 88.0% in the olanzapine group and 80.6% in the placebo group ( p  = 0.049). Multivariable analysis only identified no olanzapine use as significantly associated with a decreased risk of nausea (adjusted OR, 0.45, 95% CI, 0.26–0.76). Conclusions This analysis revealed that adding olanzapine to triple antiemetic therapy was associated with improved control of carboplatin-induced nausea. Trial registration The trial was registered with the University Hospital Medical Information Network Clinical Trial Registry (UMIN) 000026739, registered on 1 April 2017 and 000037749, registered on 1 September 2019.

No prospective studies have investigated the relationship between nutritional status, tolerability to antifibrotic therapy, and mortality in patients with fibrotic interstitial lung diseases (ILDs). This prospective longitudinal study enrolled 290 consecutive patients with fibrotic ILDs who initiated antifibrotic therapy, including 164 with idiopathic pulmonary fibrosis (IPF) and 126 with non-IPF. Nutritional status was assessed using the Geriatric Nutritional Risk Index (GNRI). Overall, 106 patients (36.6%) were classified as having malnutrition-related risk (GNRI < 98) at baseline. The prevalence of malnutrition-related risk was comparable between patients with IPF and non-IPF, although it tended to be higher in the non-IPF group than in the IPF group. Patients with malnutrition-related risk showed higher cumulative incidence of antifibrotic therapy discontinuation. Importantly, in both IPF and non-IPF groups, the mortality risk was significantly higher in patients with malnutrition-related risk than in those without. Longitudinally, a lower GNRI at 1 year was associated with shorter survival. In multivariable analyses, baseline malnutrition-related risk was independently associated with increased risk of therapy discontinuation and mortality, even after adjusting for the ILD–gender–age–physiology index. These findings indicate that assessment of nutritional status helps predict antifibrotic therapy tolerability and mortality risk in patients with fibrotic ILD.

This study aimed to assess the clinical utility of serum interferon-lambda 3 (IFN-λ3) as a sequential biomarker for treatment response and disease control in patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis (DM)-associated interstitial lung disease (ILD). Serum IFN-λ3 levels were measured in 24 patients with anti-MDA5 antibody-positive DM-ILD at diagnosis and 1 month after initiating immunosuppressive therapy. Patients were categorized into two groups based on clinical outcomes: a good control group ( n  = 16; survived without relapse for ≥ 1 year) and a poor control group ( n  = 8; died from ILD progression or relapse within 1 year). Changes in serum IFN-λ3 levels and differences between groups were analyzed. In the good control group, serum IFN-λ3 levels significantly decreased from 94.6 to 12.7 pg/mL ( p  < 0.001), whereas no significant change was observed in the poor control group (129.0 to 118.8 pg/mL). Furthermore, serum IFN-λ3 levels at 1 month were significantly lower in the good control group than in the poor control group ( p  = 0.004). Serum IFN-λ3 levels may reflect short-term treatment response and could serve as a useful sequential biomarker for assessing disease control in patients with anti-MDA5 antibody-positive DM-ILD.

The aim of this study was to evaluate the cumulative incidence and the predictive factors for collagen vascular disease (CVD) in patients initially diagnosed with idiopathic pulmonary fibrosis (IPF), and to examine the features of patients who then developed CVD. This was a retrospective review of 111 consecutive patients with IPF diagnosed at our institution. None of the patients fulfilled any of the CVD criteria from the American College of Rheumatology (ACR) within 6 months or more after the diagnosis of IPF. Ten patients (9.0%) developed CVD during the follow-up period: four had rheumatoid arthritis (RA); four had microscopic polyangiitis (MPA); one had systemic sclerosis (SSc); and one had SSc and Sjogren's syndrome (SjS). The mean time until CVD diagnosis was 3.9 years. The cumulative incidences of CVD at 1, 5, and 10 years were 0.91%, 9.85%, and 15.5%, respectively. Patients who developed CVD were significantly younger, more likely to be women and had a better prognosis than those with IPF. Cox proportional hazards regression analysis showed that female sex and the presence of lymphoid aggregates with germinal centers were significantly associated with the occurrence of CVD in patients initially diagnosed with IPF. CVD is an important underlying condition in IPF, and shows better prognosis. The possibility of the development of CVD should remain a consideration in the follow-up of IPF.

Objectives To investigate the risk factors and prognosis associated with acute exacerbation (AE) in patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD). Design A retrospective case–control study. Setting A single academic hospital. Participants 51 consecutive patients diagnosed with RA-ILD between 1995 and 2012. All patients fulfilled the diagnostic criteria of the American College of Rheumatology for RA. ILD was diagnosed on the basis of clinical presentation, pulmonary function tests, high-resolution CT (HRCT) findings and lung biopsy findings. Main outcome measures Overall survival and cumulative AE incidence were analysed using Kaplan-Meier method. Cox hazards analysis was used to determine significant variables associated with AE occurrence and survival status. Results A total of 11 patients (22%) developed AE, with an overall 1-year incidence of 2.8%. Univariate analysis revealed that older age at ILD diagnosis (HR 1.11; 95% CI 1.02 to 1.21; p=0.01), usual interstitial pneumonia (UIP) pattern on HRCT (HR 1.95; 95% CI 1.07 to 3.63; p=0.03) and methotrexate usage (HR 3.04; 95% CI 1.62 to 6.02; p=0.001) were associated with AE. Of 11 patients who developed AE during observation period, 7 (64%) died of initial AE. In survival, AE was a prognostic factor for poor outcome (HR 2.47; 95% CI 1.39 to 4.56; p=0.003). Conclusions In patients with RA-ILD, older age at ILD diagnosis, UIP pattern on HRCT and methotrexate usage are associated with the development of AE. Furthermore, AE has a serious impact on their survival.

Assessment of the clinical course of sarcoidosis requires long-term observation. However, the appropriate period of follow-up for sarcoidosis remains unclear, especially in patients without indication of corticosteroid therapy at the time of diagnosis. This study aimed to clarify the cumulative incidence and identify risk factors for disease progression in corticosteroid-naïve sarcoidosis patients. The clinical courses of 150 Japanese patients with sarcoidosis, who were followed for more than 2 years and had no indication for corticosteroid therapy at diagnosis, were retrospectively reviewed. Disease progression was defined as worsening of pulmonary sarcoidosis, development of new organ involvement, or extrapulmonary organ damage. The cumulative incidence of progression was estimated by generating a cumulative incidence curve with the Fine and Gray method. The median follow-up duration was 7.7 years (interquartile range, 4.7-13.6 years). Thirty-two (21%) patients experienced disease progression. New organ involvement appeared in 16 patients (11%). The 6-month, and 1-, 5-, 10-, and 15-year cumulative incidence of progression was 2%, 5%, 15%, 28%, and 31%, respectively. The number of organs involved at diagnosis was an independent predictor for progression with a multifactorial adjusted hazard ratio of 1.71 (95% confidence interval, 1.11-2.62). The optimal cut-off of the number of organs involved at diagnosis to identify future progression was three. In corticosteroid-naïve sarcoidosis patients, the risks of disease progression are comparable from 0-5 years and 5-10 years after diagnosis. The number of organs involved at diagnosis is a useful predictor for progression of sarcoidosis.