Neuromyelitis optica spectrum disorder (NMOSD) is a recurrent demyelinating disease of the central nervous system (CNS)characterized by inflammatory attacks on the CNS. This article reports a 56-year-old male patient admitted with over a year history of shortness of breath, a 2-week history of decreased vision in the right eye, and a 1-week history of hiccups. Physical examination revealed digital clubbing of both hands and bilateral lower lung Velcro rales. The patient had concurrent anti-aquaporin-4 (AQP4) antibody-positive NMOSD, primary Sjögren's syndrome (pSS), and interstitial lung disease (ILD), which were confirmed by characteristic clinical features, serological antibody testing, high-resolution chest CT, cranial and optic nerve MRI, and labial gland biopsy. Treatment included high-dose glucocorticoid pulse therapy combined with efgartigimod for pathogenic autoantibody clearance, inebilizumab targeted therapy, and mycophenolate mofetil (MMF) for long-term immunosuppression. After discharge, sequential maintenance therapy with oral prednisone and MMF tablets was administered. The patient's visual acuity and neurological symptoms improved. At follow-up >2 months after discharge, dyspnea recurred and worsened. After intensifying anti-infective therapy while maintaining the immunomodulatory regimen, the condition was effectively controlled, with follow-up high-resolution CT showing marked resolution of pulmonary interstitial exudates. This case suggests that anti-AQP4 antibodies may co-mediate pathological processes in both the central nervous system and peripheral organs. For patients with anti-AQP4 antibody-positive NMOSD, further screening for ILD or pSS is warranted. Early neurological assessment and antibody screening are crucial, facilitating early diagnosis and individualized treatment under multidisciplinary collaboration.

STING-associated vasculopathy with onset in infancy (SAVI) is an autoinflammatory disease caused by mutations in TMEM173 gene encoding STING (stimulator of interferon genes). It typically presents in infancy and is mainly characterized by interstitial lung disease, skin rash, and systemic inflammation. This report presents the case of adult-onset SAVI:A 28-year-old male patient was admitted with recurrent episode of cough, expectoration, chest tightness with shortness of breath and erythematous rashes on the extremities, chest, and back.His CT revealed bilateral diffuse fine reticular opacities and irregular reticular shadows, with focal areas of honeycombing. Further inquiry into the family history revealed that the patient's mother had been diagnosed with interstitial lung disease (ILD).Thus, a genetic etiology should be highly suspected.Later, whole-exome sequencing identified a heterozygous mutation in the STING1 gene: c.842G>A (p.R281Q), establishing the diagnosis of SAVI. The patient was subsequently referred to a tertiary care hospital for specialized management. During one year of follow-up, the patient underwent lung transplantation in August 2025 and had since received long-term immunosuppressive therapy for rejection prophylaxis. Cough and expectoration improved markedly, although exertional dyspnea persisted; the cutaneous rash had resolved completely.

Objective: To investigate the predictive value of baseline serum KL-6 levels for acute exacerbation (AE) in patients with fibrotic interstitial lung disease (F-ILD). Methods: A single-center retrospective cohort study was conducted, enrolling 265 patients with fibrotic ILD diagnosed by multidisciplinary team (MDT) at Sichuan Provincial People's Hospital from July 2022 to November 2024, including 118 patients with idiopathic pulmonary fibrosis (IPF) and 147 patients with fibrotic connective tissue disease-associated ILD (CTD-ILD). Baseline data were collected, and the primary endpoint was the occurrence of AE during follow-up. Cox regression analysis was used to identify independent risk factors for AE, and ROC curve analysis was performed to evaluate predictive performance. Results: After a median follow-up of 20 months, 77 patients (29.1%) developed AE. Multivariate Cox regression showed that KL-6≥1 363.5 U/ml (HR=3.928, P<0.001) was an independent risk factor for AE. Baseline KL-6 predicted AE in F-ILD with an AUC of 0.795, and the optimal cutoff value was 1 363.5 U/ml. The combined model (KL-6+CA15-3+CYFRA21-1+DLCO%) achieved an AUC of 0.853. Subgroup analysis revealed that KL-6 had the highest predictive value in IPF patients (AUC=0.820), followed by CTD-ILD patients (AUC=0.774). Conclusion: Baseline serum KL-6 is an independent predictor of AE in patients with F-ILD, demonstrating good risk stratification capability, particularly with optimal performance in IPF.

FFA4 inhibits bleomycin-induced pulmonary fibrosis in mice by suppressing IL-33.

Protective effect of bevacizumab against interstitial lung disease in non-squamous non-small-cell lung cancer: a nationwide target trial emulation study.

Interstitial lung disease (ILD) encompasses a wide variety of disorders with variable progression patterns. Monitoring typically involves assessing dyspnoea scores, spirometry parameters, 6-minute walk test (6MWT) parameters, and high-resolution CT (HRCT) scans. The agreement between the relative changes in these parameters over time is not known. To evaluate the concordance between changes in clinical, functional, physiological, and radiological parameters over 6 months in a diverse ILD cohort. Prospective observational study conducted from 01 July 2022 to 31 January 2024 at a tertiary care teaching hospital. Ninety-nine ILD patients underwent baseline and 6-month assessments of dyspnoea (mMRC), forced vital capacity (FVC), six-minute walk distance (6MWD), distance-saturation product (DSP), and HRCT. Patients were categorised as having lung-restricted or systemic disease-associated ILD. Changes were classified using established minimal clinically important differences. Concordance was analysed using weighted kappa; correlations were evaluated with Spearman's test. Of 99 patients, 67.7% had ILD secondary to a systemic condition. Correlations between FVC, DSP, and 6MWD were moderate to strong both at baseline and at 6 months. Concordance among changes in clinical scores, FVC, 6MWD, and HRCT at the 6-month follow-up was low, with weighted-kappa values ranging from 0.023 to 0.158 for the overall study group. The strongest agreement was observed between clinical scores and FVC. The concordance was similarly poor in both subgroups. Despite moderate baseline correlations, changes in different ILD monitoring parameters showed poor agreement, underscoring the multidimensional nature of disease progression. Comprehensive, multimodal follow-up remains essential, and multicentre validation is warranted.

Validation and minimum important difference of the chronic respiratory disease questionnaire in patients with interstitial lung disease.

Aspergillosis complicating idiopathic lung fibrosis: a multicentric series.

Outcomes of stereotactic ablative radiotherapy or surgery for early-stage lung cancer in patients with interstitial lung disease.

Relapsed small cell lung cancer (SCLC) treatment has limited evidence-based options. Solvent-based paclitaxel (PTX) and albumin-bound nanoparticle paclitaxel (nab-PTX) are commonly prescribed though never comprehensively quantified. A systematic review and single-arm meta-analysis was conducted (PROSPERO Registration: CRD42024592475). PubMed, Web of Science, Cochrane Library, and EMBASE were searched for interventional or observational studies reporting PTX or nab-PTX monotherapy in relapsed SCLC. Primary outcomes were the pooled 3-month progression-free survival rate (PFS), 6-month overall survival rate (OS), objective response rate (ORR), and disease-control rate (DCR). Random-effects models generated pooled estimates; prespecified subgroup analyses compared Asian versus Euro-American cohorts, and PTX versus nab-PTX treatment. Fifteen studies (11 retrospective and 4 prospective) comprising 631 patients met the eligibility criteria of the study. Pooled efficacy estimates were: 3-month PFS 39% [95% confidence interval (CI)=28-50%, I2=84%], 6-month OS 46% (95%CI=32-61%, I2=91%), ORR 16% (95%CI=12-21%, I2=51%) and DCR 51% (95%CI=41-61%, I2=83%). Grade 3 or higher toxicities were infrequent - neutropenia 18% (95%CI=11-25%, I2=89%) and peripheral neuropathy 2% (95%CI=0-3%, I2=0%). Among 45 patients with pre-existing interstitial lung disease (ILD), treatment-related ILD exacerbations were 17% (95%CI=6-28%, I2=0%). Asian cohorts demonstrated greater neutropenia (26% vs. 5%) than Euro-American cohorts. Efficacy and safety were similar between PTX and nab-PTX monotherapy. Despite substantial heterogeneity, paclitaxel-based regimens demonstrated modest but clinically meaningful activity with generally favorable safety profile in relapsed SCLC, highlighting them as a pragmatic salvage option. In patients with ILD, their use warrants caution due to the risk of ILD exacerbation.

When MicroRNAs meet hypoxic pulmonary hypertension.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a disorder characterized by neuroendocrine cell hyperplasia, tumorlets and tumors, manifesting as lung nodules, chronic cough, and airflow obstruction. Often misdiagnosed as asthma, DIPNECH lies at the cross-section of oncology, obstructive airway disease and interstitial lung disease. With the increasing use of CT scans leading to higher rates of lung nodule detection, it is important for clinicians to be familiar with DIPNECH. The evidence base in DIPNECH is sparse and limited to retrospective case series. In the last 5 years, clinical experiences in large academic centers have been published describing variability in clinical presentation and pulmonary function, use of DOTATATE scans, efficacy of somatostatin analogs, and principles of surveillance imaging. More awareness of DIPNECH is needed among pulmonary clinicians. Apart from the usual presentation of cough with lung nodules and spirometric obstruction in women, patients also present with dyspnea and/or restrictive patterns on PFTs. Variability in diagnosis and management is widespread. Multidisciplinary assessment is helpful in guiding management. Multicenter and multispecialty collaboration is needed to establish best practices and improve clinical management.

Inhaled Treprostinil Use and Pre-Transplant Outcomes in Patients with Pulmonary Hypertension Associated with Interstitial Lung Disease

Interstitial Lung Disease with and without Mean Pulmonary Artery Pressure &gt;20 mmHg: Interim Results from the PHINDER Study

Significance of geriatric nutritional risk index in predicting lung-transplant waiting list mortality of patients with interstitial lung disease regardless of percentage forced vital capacity.

Influence of Pulmonary Hypertension on Lung Transplant Survival and Graft Laterality in Interstitial Lung Disease

Nintedanib combined with dual immunosuppression for interstitial lung disease in systemic sclerosis and rheumatoid arthritis: A systematic review and pooled multicentre real-world cohort study.

Fibrotic interstitial lung diseases (ILDs) cause loss of forced vital capacity (FVC) and increased risk of death over time. Most clinical trials aim to slow FVC decline and reduce mortality. However, the association of lower FVC with higher mortality will bias simple estimates of differences in FVC progression between groups. Therefore, both the time-dependent decline in FVC and increase in mortality should be jointly modeled. We developed a Bayesian, joint mixed-effects disease progression model (DPM), using minimally informative prior distributions, for FVC trajectory and the hazard for ILD-related mortality over time. This model minimizes bias due to mortality in estimating differences in the rate of FVC decline and is suitable for use when characterizing populations or in estimating a treatment effect in a clinical trial. The DPM was applied to individual patient data from prospective cohort studies of fibrotic ILD. The DPM yields a higher estimated rate of FVC decline (6.0% vs 4.7%/year) and a more precise fit than a linear mixed model of FVC alone, and replicates the nonlinear pattern in the observed data. By modeling the full FVC trajectory rather than only the change from baseline at a given time point, the DPM increases the information from each patient and reduces both the time to information and the effect of variability in baseline FVC measurements on the estimation of treatment effects. The joint DPM provides an integrated approach to minimizing bias in the estimation of treatment effects in clinical trials in fibrotic ILDs.

Treatment of pulmonary fibrosis and combined pulmonary fibrosis and emphysema syndrome with herbs for promoting blood circulation and removing blood stasis: A review of pharmacological studies.

Hypoxic pulmonary hypertension (HPH) is a representative vascular remodeling disease with a poor prognosis. Previous findings from our study have implicated the NICD4 (Notch4 intracellular domain) in pulmonary artery smooth muscle cells (PASMCs) in the pathogenesis of HPH. However, the underlying regulatory mechanisms remain unclear. In this study, we aimed to elucidate the potential regulatory mechanism of NICD4 in HPH. Using coimmunoprecipitation combined with mass spectrometry, we identified USP8 (ubiquitin-specific peptidase 8) as a novel binding protein of NICD4 in PASMCs. The functional role of USP8 was investigated in vivo using smooth muscle cell-specific Usp8 knockout (Usp8Acta2-/-) mice and in vitro using primarily cultured PASMCs, alongside pharmacological inhibition with DUB-IN-2 (deubiquitinase-inhibitor-2). USP8 was significantly upregulated in lung tissues from patients with HPH due to interstitial lung disease or chronic obstructive pulmonary disease, HPH rodent models, as well as in hypoxic PASMCs. Usp8 deficiency in Acta2-positive mice (Usp8Acta2-/-) or pharmacological inhibition of USP8 by DUB-IN-2 markedly attenuated HPH development. In vitro, USP8 knockdown suppressed hypoxia-induced PASMC proliferation, migration, and apoptosis resistance by modulating the NICD4-MAPK pathway. Mechanistically, USP8 was bound directly to NICD4 to maintain its stability by removing the K48-linked ubiquitin chain on NICD4 at lysine 1760, thus preventing proteasomal degradation. Furthermore, USP8 can be transcriptionally upregulated by CSL/NICD4 under hypoxia, forming a NICD4/USP8-positive feedback loop. Our study unveils a critical NICD4/USP8-positive feedback loop that drives HPH pathogenesis, highlighting the importance of ubiquitination in pulmonary vascular remodeling. Targeted disruption of this loop represents a promising therapeutic strategy for HPH.