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Background Chronic hypersensitivity pneumonitis (CHP) is an interstitial lung disease (ILD) caused by long term exposure to an offending antigen. Antigen avoidance is associated with improved outcomes. We are unable to identify the antigen source in approximately half of patients. When an antigen is successfully identified, patients have difficulty with avoidance. Methods We conducted three structured group discussions with US based ILD specialists utilizing the nominal group technique (NGT). Participants listed barriers to antigen detection and avoidance in CHP. Each participant ranked what they perceived to be the top three barriers in the list in terms of importance. The master list of barriers was consolidated across the three groups into themes that were prioritized based on receiving the highest rankings by participants. Results Twenty-five physicians participated; 56% had experience caring for CHP patients for ≥ 16 years. Sixty barriers to antigen detection were categorized into seven themes of which the top three were: 1. unclear significance of identified exposures; 2. gaps in clinical knowledge and testing capabilities; 3. there are many unknown and undiscovered antigens. Twenty-eight barriers to antigen avoidance were categorized into five themes of which the top three were: 1. patient limitations, financial barriers and lack of resources; 2. individual patient beliefs, emotions and attachments to the antigen source; and 3. gaps in clinical knowledge and testing capabilities. Conclusions This study uncovered challenges at the individual patient, organizational, and societal levels and ranked them in terms of level of importance. These findings provide information to guide development and validation of multidisciplinary support and interventions geared towards antigen identification and avoidance in CHP.

Background: In the past two decades, many advances have been made to our understanding of interstitial lung disease (ILD) and the way we approach its treatment. Despite this, many questions remain unanswered, particularly those related to how the disease and its therapies impact outcomes that are most important to patients. There is currently a lack of guidance on how to best define and incorporate these patient-centered outcomes in ILD research. Objectives: To summarize the current state of patient-centered outcomes research in ILD, identify gaps in knowledge and research, and highlight opportunities and methods for future patient-centered research agendas in ILD. Methods: An international interdisciplinary group of experts was assembled. The group identified top patient-centered outcomes in ILD, reviewed available literature for each outcome, highlighted important discoveries and knowledge gaps, and formulated research recommendations. Results: The committee identified seven themes around patient-centered outcomes as the focus of the statement. After a review of the literature and expert committee discussion, we developed 28 research recommendations. Conclusions: Patient-centered outcomes are key to ascertaining whether and how ILD and interventions used to treat it affect the way patients feel and function in their daily lives. Ample opportunities exist to conduct additional work dedicated to elevating and incorporating patient-centered outcomes in ILD research.

Objectives This study sought to determine the level of understanding and opinion among rheumatologist and pulmonologists regarding risk factors, diagnostic approach and treatment of RA-associated interstitial lung disease (RA-ILD). Methods We conducted an international electronic survey of rheumatologists and pulmonologists utilizing two separate Redcap-based surveys with questions on the epidemiology, workup and management of RA-ILD as well as ILD screening questions using case-based scenarios directed at rheumatologists. The survey also collected demographic data on participants including their practice setting, years in practice and country of practice. Results We received a total of 616 responses (354 rheumatologists and 262 pulmonologists) from six continents. There were significant differences in responses between pulmonologists and rheumatologists in estimated prevalence and mortality, risk factors for the development of ILD in RA and medications that are effective or should be avoided. Rheumatologists were much less likely to consider assessment for ILD in high risk, asymptomatic patients compared with high-risk patients with either symptoms or exam findings suggestive of ILD. Conclusion Our study brought to light the variability in disease assessment and clinical practice among providers caring for patients with RA-ILD and indicate that greater education is needed to optimize clinical decision making in the risk assessment, screening and treatment of RA-ILD. Research questions that address appropriate screening and treatment strategies for RA-ILD will be valuable for rheumatologists given their central role in the overall health and lung health of patients with RA.

Health-Related Quality of Life (HRQOL) is increasingly viewed as an important patient-centered outcome by leading health organizations, clinicians, and patients alike. This is especially true in the interstitial lung disease community where patients often struggle with progressive and debilitating disease with few therapeutic options. In order to test the effectiveness of new pharmacologic therapies and non-pharmacologic interventions globally in ILD, this will require expansion of clinical research studies to a multinational level and HRQOL will be an important endpoint to many. In order to successfully expand trials across multiple nations and compare the results of studies between different communities we must recognize that there are differences in the concepts of HRQOL across the world and have strategies to address these differences. In this review, we will describe the different global influences on HRQOL both generally and in the context of ILD, discuss the processes of linguistic translation and cross-cultural adaptation of HRQOL Patient Reported Outcome Measures (PROMs), and highlight the gaps and opportunities for improving HRQOL measurement in ILD across the world.

RationaleChronic hypersensitivity pneumonitis (CHP) is a distinct form of interstitial lung disease caused by an inhaled environmental antigen. Some patients with CHP develop progressive pulmonary fibrosis and varying degrees of symptom severity, with heterogeneous impact on functioning and overall well-being. There are no universally accepted diagnostic criteria, few FDA-approved (Food and Drug Administration) therapies and no standardised approach to identifying an antigen. The impact that living with CHP has on patients’ quality of life is understudied, preventing the identification of patient-centred research questions and endpoints for future CHP clinical trials.ObjectivesWe explored patients’ experiences, perceptions and expectations of living with CHP.MethodsWe conducted semistructured interviews with patients with CHP. Patients were recruited using a purposive heterogeneous sampling strategy. Interviews were audio recorded and transcribed verbatim. Data were analysed using the grounded theory method.ResultsEighteen patients were interviewed. Six major themes emerged from the interviews: (1) suffering due to lack of knowledge and uncertainty, (2) hypervigilance, (3) psychosocial, (4) physical impacts, (5) interpersonal and (6) self-perception and identity. The need to identify and avoid an antigen played a prominent role across the themes and subthemes.ConclusionsWe identified several key influences on quality of life in patients with CHP that have not been adequately explored. The prevalence of these influences should be quantified, and they should be included in quality of life assessment and should guide the development of targeted interventions to improve quality of life in this patient population.