Current guidelines recommend structured training in lung ultrasound (LUS) for interstitial lung disease (ILD). Therefore, this exploratory study evaluated physician perceptions, perceived educational value, and self-reported applicability of a structured LUS workshop involving patients with ILD. A six-hour LUS workshop combining didactic lectures with supervised hands-on training on ILD patients was conducted for a group of actively practicing rheumatologists and pulmonologists. Workshop evaluation was performed using an anonymous questionnaire capturing participants' demographics and pre-course LUS practice, course satisfaction, and perceived barriers to using LUS routinely in ILD care. Ten physicians, predominantly experienced rheumatologists with regular exposure to ILD patients, attended the workshop. Pre-course LUS was infrequent, with only one participant reporting regular use. Following the workshop, all participants reported perceived improvement in LUS knowledge and increased self-confidence in recognizing typical sonographic features of ILD. The workshop was universally rated as clinically relevant, and nine of ten attendees expressed intention to integrate LUS into their clinical practice. Lack of experience and time constraints were identified as main perceived barriers, whereas standardized protocols and focused training were viewed as important facilitators for clinical adoption of LUS in ILD. This exploratory study suggests that a concise, structured LUS workshop involving patients with ILD was perceived as clinically relevant and educationally valuable by participating physicians.

To characterize the clinical and serological correlates of cardiovascular magnetic resonance (CMR)-confirmed myocarditis in idiopathic inflammatory myopathies (IIM), evaluate the diagnostic performance of high-sensitivity cardiac troponin I (hs-TnI), and quantify the independent prognostic impact of myocarditis relative to interstitial lung disease (ILD). Single-center retrospective cohort study (STROBE-compliant) of 142 consecutive adults with IIM (2019-2025). Myocarditis was confirmed by CMR according to the 2018 Lake Louise Criteria, performed both at diagnosis and during follow-up (with or without immunosuppression) upon clinical suspicion and/or elevated hs-TnI. Myositis-specific antibodies (MSA), myositis-associated antibodies (MAA), anti-Ro52 and antiphospholipid antibodies (aPL, Sydney criteria: ≥40 GPL/MPL units confirmed at ≥ 12 weeks) were systematically recorded. Associations were assessed with Fisher's exact test and logistic regression; multivariable models were pre-specified as parsimonious (two predictors) given the limited event count. Overall survival was analyzed with Kaplan-Meier curves, the log-rank test and Cox proportional-hazards regression. Myocarditis was confirmed in 9/142 patients (6.3%), without significant association with IIM class (P = 0.303) or with the antisynthetase antibody subgroup (anti-Jo-1 + PL-12 + PL-7 + EJ; 5.8% vs. 6.7%, P = 1.000). In univariable analyses, myocarditis was associated with Raynaud phenomenon (OR 13.6, 95% CI 2.3-80.0), aPL positivity (OR 10.7, 95% CI 2.6-43.9), anti-Ro52 coexisting with MSA/MAA (OR 8.7, 95% CI 2.2-34.8), anti-PM/Scl (OR 7.1, 95% CI 1.6-30.3), fever (OR 7.1, 95% CI 1.6-30.3) and ILD (OR 4.7, 95% CI 1.1-20.3). hs-TnI was markedly higher in myocarditis (median 366 vs. 3.5 ng/L; P < 0.001) with an area under the receiver-operating characteristic curve (AUROC) of 0.91 (95% CI 0.83-0.98). In the event-constrained multivariable model, Raynaud phenomenon (adjusted OR 13.1, 95% CI 1.5-112.7) and aPL (adjusted OR 7.2, 95% CI 1.4-36.0) remained independently associated. All-cause mortality was higher in myocarditis (44% vs. 9%; OR 7.95, 95% CI 2.0-31.5; log-rank P = 0.030; univariable Cox HR 3.77, 95% CI 1.03-13.78; P = 0.044), and persisted after adjustment for ILD in the logistic model (adjusted OR 5.9, 95% CI 1.3-26.1; P = 0.020), whereas ILD attenuated to a non-significant trend (adjusted OR 2.9, 95% CI 0.9-9.2; P = 0.073). In this single-center retrospective cohort, IIM-associated myocarditis was consistently associated with Raynaud phenomenon, aPL positivity and anti-Ro52 coexistence, and was associated with higher mortality than ILD. These findings support hs-TnI-guided triage to CMR as part of systematic cardiac surveillance in IIM, pending multicenter confirmation.

Pulmonary fibrosis (PF) is a progressive interstitial lung disease with poor prognosis and limited therapeutic options, largely due to the lack of physiologically relevant and dynamically traceable preclinical models. Here, we establish an induced pluripotent stem cell (iPSC)-derived alveolar organoid platform that faithfully recapitulates PF-like remodeling upon TGFβ1 stimulation. The organoids exhibit lineage commitment to the distal lung epithelium (NKX2.1, SFTPC/SFTPB) and, following TGFβ1 exposure, undergo hallmark fibrotic changes including organoid condensation and size reduction, collagen deposition (Masson's trichrome), myofibroblast activation (α-SMA), up-regulation of profibrotic genes (COL1A1, FN, VIM, ACTA2), and partial EMT-like reprogramming (↑CDH2, TWIST1, and ↓CDH1). To enable label-free, longitudinal readouts, we integrate a deep neural network (YOLOv8-nano) that detects subtle morphological cues directly from bright-field images and classifies treatment status with high fidelity. Across augmented datasets (8892 images), the model achieved strong performance on the original context-preserving images (mAP50-95 up to 0.95; high precision/recall and 98-99% true-positive rates), supporting robust discrimination of control versus TGFβ1-treated organoids. This AI-enhanced organoid system provides a quantitative, label-free platform for monitoring fibrotic remodeling and offers a scalable foundation for preclinical antifibrotic screening and mechanism-of-action studies.

Common variable immunodeficiency (CVID) is associated with diverse clinical manifestations. This systematic review aimed to explore associations between disease factors and clinical outcomes in patients with CVID. Eligible studies included patients with CVID and reported associations between disease factors and clinical outcomes (e.g., autoimmune cytopenia, bronchiectasis, infections, lung damage, malignancy, and mortality). Quality assessment was conducted using the Newcastle-Ottawa Scale. Thirty-nine studies met inclusion criteria. The occurrence of lymphadenopathy or splenomegaly was associated with an increased risk of autoimmune cytopenia and lung damage specific to granulomatous-lymphocytic interstitial lung disease; splenomegaly was associated with increased risk of bronchiectasis. Higher levels of switched memory B (smB) cells and marginal zone B cells were associated with reduced risks of lung damage; higher levels of smB cells were associated with a reduced risk of autoimmune cytopenia. Elevated CD21[low] B cells were associated with increased risks of autoimmune cytopenia, lung damage, and infections. Higher total B cells were associated with lower risk of bronchiectasis and mortality. These findings suggest specific relationships are present between lymphoproliferation markers and B-cell subsets and clinical outcomes in CVID. Limitations including heterogeneity in study designs and low- to moderate-quality evidence underscore the need for additional research to validate these associations.

Pulmonary fibrosis is a chronic, progressive interstitial lung disease marked by excessive buildup of extracellular matrix and abnormal tissue remodeling. This leads to irreversible loss of lung function and a major decline in quality of life. The disease places a heavy burden on patients, their families, and healthcare systems. For years, treatment options were limited, with no proven effective therapies. However, recent progress in understanding the disease mechanisms has spurred the development of new treatments. This review outlines the latest insights into the pathophysiology of pulmonary fibrosis and explores emerging therapies, including medicine, stem cell therapy, gene therapy, and biologics. The goal is to provide a comprehensive resource for clinical practice and to highlight future research directions for improving patient outcomes.