Anne-Marie Russell

Objectives: Self-efficacy (SE) is central to chronic disease self-management but remains poorly characterized in idiopathic inflammatory myopathies (IIMs). We aimed to identify factors associated with SE, characterize SE profiles and examine associations with patient-reported outcomes (PROs) in IIMs. Methods: Cross-sectional data from 5802 participants across IIMs, other rheumatic diseases (RMDs) and non-rheumatic autoimmune diseases (nRAIDs) were analysed. SE was measured using the Self-Efficacy for Managing Chronic Disease (SEMCD) scale. Hierarchical regression identified factors independently associated with SE, and k-means clustering defined SE health profiles. Associations with PROs were examined in a separate multivariable model. Results: IIM patients had lower SE (median SEMCD 5.3) than those with RMDs (5.8) and nRAIDs (6.5), with confidence lowest for managing fatigue and physical discomfort. In the fully adjusted integrated model (R² = 0.222), mental health comorbidity (B = −0.898) and financial difficulty (B = −0.734) were most strongly associated with lower SE, while regular exercise (B = 0.770) and family support (B = 0.150) were associated with higher SE. The PRO model explained 48.3% of SE variance (R² = 0.483); resilience was the strongest positive association, while fatigue and pain were negatively associated. Four SE health profiles were identified, including a clinically distinctive resilient-despite-burden subgroup (24%) with high symptom burden but preserved SE and mental health. Higher SE was associated with better physical and mental health, greater resilience and life satisfaction, and lower fatigue, pain and loneliness (all P < 0.001). Conclusion: SE in IIMs is associated with modifiable socioeconomic and psychological factors and with better health outcomes. Profile-based stratification may help identify vulnerable subgroups and guide targeted interventions.

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive debilitating lung disease which affects physical and mental well-being. The IPF Patient-Reported Outcome Measure (IPF-PROM) scale is a validated and reliable tool for the self-report of physical and psychological well-being in IPF. Aims and objectives: This study aimed to validate a Greek version of the IPF-PROM scale and further investigate its correlation with clinical features of IPF patients and its interrelation with depressive symptoms and health-related quality of life (HRQoL). Methods: This was a two-centre, observational, cross-sectional study, in which IPF patients completed three scales: IPF-PROM, Patient Health Questionnaire-9 (PHQ-9), an index of depressive symptoms, and Health Survey Questionnaire Short Form-12 (SF-12), an index of HRQoL, at the IPF Outpatient Clinics of two University Hospitals in Greece during 2023–2024. Logistic regression analysis was conducted to assess severe status of IPF-PROM compared with mild/moderate status. Results: The study involved 136 IPF patients (87.9% males) with a mean age of 73.5±7.9 years. Patients were classified overall with moderate disease according to IPF-PROM mean scores (41.7±31.3), particularly in the combined Breathlessness/Fatigue and the Psychological well-being components (40.1 and 43.8, respectively, p>0.050). Patients with severe symptoms, as measured by IPF-PROM, scored higher levels of depressive symptoms on PHQ-9 compared with those with moderate or mild symptoms (17.1, 12.3 and 1.4, respectively, p<0.001) and lower levels of HRQoL in physical (32.6, 34.7 and 47.9, p<0.001) and mental health on SF-12 (23.9, 34.5 and 50.3, p<0.001). Patients with incrementally higher levels of oxygen saturation had lower odds for severe health status according to IPF-PROM (OR=0.83, p=0.018). Conclusions: A significant percentage of IPF patients present with impaired health status and symptoms suggestive of depression. The IPF-PROM scale represents a useful tool that may predict impairment of mental health and HRQoL in IPF, with potential utility for clinical practice and research.

Extract Immune checkpoint inhibitors (ICIs) are immunomodulatory monoclonal antibodies that enhance T-cell-mediated cytotoxicity and have significantly transformed the treatment and outcomes of many cancers [1]. The primary targets of checkpoint inhibition are programmed cell death receptor 1 (PD-1), its ligand programmed cell death ligand 1 (PD-L1) and cytotoxic T-lymphocyte associated antigen 4 (CTLA-4) [2]. Shareable abstract A standardised risk assessment before initiating ICI treatment may improve early detection and management of IR-ILD. Multidisciplinary collaboration is crucial for enhancing patient safety, improving treatment outcomes and advancing research. https://bit.ly/4qAthl6

Background: Fatigue, breathlessness and cough are prevalent symptoms of interstitial lung disease (ILD) adversely impacting quality of life and contributing to psychological distress. The fatigue and breathlessness (FAB) programme facilitates supported self-management for people living with life-limiting conditions. We explore its utility when adapted for people living with ILD. Methods: The 4-week ILD-FAB programme offers each group (n=≤6) weekly 2-hour sessions led by an ILD-specialist physiotherapist and clinical nurse specialist (CNS). Primary focus is on strategies to manage breathlessness, fatigue and well-being. Further, a 1:1 session with the ILD-CNS enables participants to set personalised goals and explore individual health beliefs/behaviours using a cognitive behavioural therapy assessment framework. The self-reporting chronic respiratory questionnaire (CRQ-SR) evaluates breathlessness, fatigue, emotional function and mastery at baseline and after 4 weeks. We facilitated 11 groups between March 2023 and December 2024. Results: 49 participants (26 males; median age 76 years (IQR=14)) were diagnosed with idiopathic pulmonary fibrosis (n=21), progressive pulmonary fibrosis (n=17) or non-progressive ILD (n=11) of various aetiologies. Lung function indicated a range of disease severity (FVC % predicted median: 70% (IQR=34); DLCO % predicted median: 42% (IQR=30)). 37 (76%) participants attended all four sessions, 6 (12%) attended three sessions, 2 (4%) attended two sessions and 4 (8%) attended one session. 37 patients, all of whom attended at least three sessions, completed the CRQ-SR at baseline and week 4. 59% of respondents (n=22) demonstrated clinically significant improvements in dyspnoea scores, 51% (n=19) in emotional functioning scores and 49% (n=18) in fatigue and mastery scores. 35 respondents (95%) demonstrated a clinically significant improvement in at least one domain. All participants (100%) would recommend this programme to others. Conclusion: These data demonstrate feasibility, acceptability and clinical effectiveness of an ILD-specific FAB programme. Further research will explore a range of outcome measures longitudinally in a larger cohort.

Extract In fibrotic interstitial lung disease (F-ILD), characterized by progressive scarring, breathlessness is a defining symptom contributing to hospitalizations, mortality, and poor quality of life (QoL). [1, 2] Patients with F-ILD experience distress from breathlessness, while caregivers report feeling overwhelmed, anxious and depressed due to lack of support [3–5]. Without preparation to manage breathlessness at home, patients often seek emergency care, leading to respiratory hospitalizations and escalating costs [6]. Median survival post hospitalizations are 2.8 months, compared to 27.7 months for non-respiratory hospitalizations [7], highlighting need for dedicated dyspnoea care. Despite guidelines that recommend symptom management and quality of life measures with disease-modifying therapy [8, 9], breathlessness remains an unmet need. Shareable abstract Themes of invisibility and visibility; complexity of assessment; and prioritizing breathlessness in fibrotic interstitial lung disease revealed knowledge gaps, need for activity-based assessment, and experiential learning for clinicians.

Pulmonary fibrosis (PF) causes respiratory insufficiency due to progressive lung scarring and impaired gas exchange. Although no curative treatment exists,

Objective: Patient-reported experience measures (PREMs) are a key component of healthcare accountability frameworks, health policy, integrated care board commissioning and integrated care partnerships generating data which are crucial markers of patient care quality. The Rheumatoid Arthritis Patient-Reported Experience Measure (RA-PREM) incorporates the eight core elements of NHS Patient Experience Framework and is validated in a range of rheumatic conditions. Our objective is to determine the acceptability and feasibility of the RA-PREM for an interstitial lung disease (ILD) population. Design: A mixed-methods patient-centred approach incorporating an interdisciplinary research steering group with patient partners. Patient surveys evaluated the language and meaning of the RA-PREM 8 domains, 24 statements and response categories. A patient focus group examined contentious statements. A consensus group of expert patient-partners agreed statements for the modified RA-PREM. Focus group participants reviewed the modified instrument (ILD-PREM) for acceptability and face/content validity. Setting: A single NHSE-commissioned, regional ILD service/UK. Results: Thirteen patients (10 male) diagnosed with ILD participated in focus group discussions. Critical discussion of the RA-PREM resulted in nuanced modifications of four statements of three domains. Five patients (three male) and three healthcare researchers attained consensus on the face/content validity of statements. Seventy-three patients completed the ILD-PREM following outpatient contact. Conclusion: The ILD-PREM retains 24 statements representing the eight domains of the RA-PREM. It meets face/content validity criteria and is acceptable to an ILD population. Longitudinal validation of the ILD-PREM across ILD services including further testing in global minority groups will establish criterion and construct validity and objective measures of reliability.

Rationale: Equitable care for all individuals living with interstitial lung disease (ILD) must be rooted in rigorous, high-quality research that is globally representative and inclusive. Objectives: The objectives of this American Thoracic Society Statement are to emphasize the importance of racial, ethnic, sex, and gender considerations in ILD research; summarize existing evidence on differences and disparities in ILD care; and suggest practical recommendations to promote equitable inclusion in clinical research. Methods: A multidisciplinary committee of experts in ILD, health equity, sex/gender, and race/ethnicity equity conducted a comprehensive review of the literature related to disparities in ILD. The group identified relevant studies pertaining to clinical trial participation and outcomes by race, ethnicity, sex, and gender. Consensus-based recommendations for equitable inclusion in research were developed through iterative discussion and agreement by all members. Main Results: The review identified significant disparities across ILD domains, including epidemiology, diagnosis, management, treatment access, and clinical outcomes. Minority populations remain underrepresented in ILD research, especially in clinical trials of ILD treatment. Research efforts and programs in ILD must be based on inclusive practices. This can be accomplished by changing how subgroup data are collected, analyzed, and reported in ILD clinical trials, with greater attention to the inclusion of minority populations, at all levels of research. Conclusions: Improving equity in ILD research is paramount to enhancing the generalizability and applicability of findings to the global ILD population. This goal will require coordinated action by all stakeholders, including researchers, institutions, funding agencies, and patient communities.