Yusuke Inoue

Background: Patients with fibrotic interstitial lung diseases (ILDs) experience impaired health-related quality of life (HRQoL). The King's Brief Interstitial Lung Disease (KBILD) questionnaire was designed to evaluate disease-specific HRQoL; however, its practical application in real-world clinical settings has not been thoroughly investigated. Methods: This prospective longitudinal study included 289 patients with fibrotic ILD initiated antifibrotic therapy. Their HRQoL was evaluated using the KBILD questionnaire at baseline, 6 months and 1 year. A total of 289, 164 and 159 patients completed the KBILD assessment at the aforementioned time points, respectively. The associations between the KBILD scores, clinical parameters and mortality risk were evaluated. Results: The KBILD Total score demonstrated a moderate correlation with dyspnoea scale but a weak or no correlation with pulmonary function test parameters and ILD aetiology. Interestingly, more than 20% of the patients showed significant improvement (≥5 points) at 6 months and 1 year. A lower baseline KBILD Total score was associated with disease progression and reduced survival, independent of the ILD–gender–age–physiology index. In addition, longitudinal assessment showed that the cross-sectional KBILD Total score at 6 months and 1 year and longitudinal decreases in the KBILD Total score at 1 year were associated with increased mortality risk. Conclusion: Antifibrotic therapy has a positive impact on HRQoL in a subset of patients with fibrotic ILDs. Repeated KBILD assessment may aid in monitoring mortality risk, independent of lung function tests.

In individuals without underlying causes known to cause pleuroparenchymal fibroelastosis, apical scarring at chest CT was more prevalent with age, associated with a lean body habitus, and more likely to progress over time when baseline lesions were more extensive.

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 number of patients with cancer qualifying for treatment with immune checkpoint inhibitors (ICIs) continues to increase, and a clearer understanding of the mechanisms underlying their activity-driven side effects or immune-related adverse events (irAEs) has become crucial. Patients receiving ICIs can develop irAEs in any organ, and numerous studies have suggested that irAE development may be associated with improved ICI efficacy. However, the robustness and magnitude of such associations are unclear, and little is known about the relationship between irAE development and ICI efficacy at the individual organ level. A precise understanding of these links could improve patient care and provide further insight into the immunological mechanisms underlying both irAE development and ICI efficacy. We herein review the prognostic implications of irAEs occurring in patients with cancer treated with ICIs and discuss outstanding issues that should be addressed in future studies.