Mental health in paediatric and adult myositis-related diseases : current state of research, interventions, and future steps from the MIHRA Psychological Impact Scientific Working Group

Psychological and emotional well-being are critical aspects of overall health for individuals with chronic rheumatologic conditions. Mental health-related literature, however, predominantly focuses on systemic lupus erythematosus or rheumatoid arthritis, with limited emphasis on idiopathic inflammatory myopathies (IIMs). High proportions of those with juvenile myositis report psychological distress at levels warranting mental health referral. Adults with dermatomyositis diagnosed with depression or anxiety do not receive adequate mental health care. Mental health symptoms in those with IIMs are associated with worse health-related quality of life, medication adherence, and disease outcomes. Despite demonstrated high rates of mental health burden, access to mental health care remains severely lacking. Data related to mental health burden is limited by small sample size, limited generalisability, variable methods of assessment, and inconsistent diagnosis codes to define mental health conditions. Additional research is needed to validate current screening tools in myositis populations. Other relevant measurable factors include disease severity, non-health- and health-related trauma exposure, loneliness, isolation, loss of control, sleep difficulties, fatigue, pain, self-esteem, body image, sexual health, and health inequities. Studiesare needed investigating the efficacy of therapeutic and pharmacologic interventions among patients with myositis who experience depression and anxiety. Currently, knowledge and resources are limited around mental health burden and potential intervention for those living with IIMs. The Myositis International Health & Research Collaborative Alliance (MIHRA) Psychological Impact Scientific Working Group offers a preliminary road map to characterise and prioritise the work ahead to understand baseline mental health burden and compare avenues for intervention

World Health Organization (WHO) International Classification of Functioning, Disability and Health (ICF) Core Set Development for Interstitial Lung Disease

This is the final version. Available on open access from Frontiers Media via the DOI in this recordData availability statement: The original contributions presented in the study are included in the article/Supplementary Material. Further inquiries can be directed to the corresponding authors.Background: The World Health Organization (WHO) introduced the International Classification of Functioning, Disability, and Health (ICF) as a scientific method of disability data collection comprised of >1,200 categories describing the spectrum of impairment types (functional, symptoms-based and anatomical) under the bio-psycho-social model with consideration of environmental and personal factors (pf). ICF Core Sets and ICF Checklists are streamlined disease-specific resources for clinical use, service provision, and for use in health economics and health policy. ICF can disclose strengths and weaknesses across multiple patient-reported outcome measures (PROMs) and help consolidate best-fitting question-items from multiple PROMs. Interstitial lung diseases (ILDs), are generally progressive, with restrictive physiology sometimes occurring in the context of multi-organ autoimmunity/inflammatory conditions such as connective tissue diseases (CTDs). In spite of significant associated morbidity and potential disability, ILD has yet to be linked to the ICF. Methods: Each instrument and their question-items within the consensus-recommended core sets for clinical trials in ILD were deconstructed to single concept units, and then linked per updated ICF linkage rules. Inter-linker agreement was established. Three additional subsequently validated measures were also included. Results: One-hundred-eleven ICF categories were identified for ten PROMs and three traditional objective measures that were amenable to ICF linkage. The proportion of agreement ranged from 0.79 (95% CI: 0.62, 0.91) to 0.93 (0.76, 0.99) with the overall proportion of inter-linker agreement being very high 0.86 (0.82, 0.89) for the initial instruments, with 94–100% for the three additional PROMs. Thirty-four new ‘Personal Factors’ emerged to capture disease-specific qualities not elsewhere described in ICF, e.g. ‘pf_embarrassed by cough’ or ‘pf_panic/afraid when can’t get a breath’. Conclusion: This first known effort in ICF linkage of ILD has provided important revelations on the current utility of the ICF in lung disease. Results have indicated areas for meaningful assessment of ICF descriptors for lung impairment. The mapping across PROMs provides insight into possibilities of developing more streamline and precise instrumentation. Finally, familiarity with the ICF in ILD may enable clinicians to experience a smoother transition with the imminent harmonization of ICD and ICF, ICD-11

Resilience: Examining the Impacts of the Deepwater Horizon Oil Spill on the Gulf Coast Vietnamese American Community

The 2010 Deepwater Horizon oil spill (DWH) was one of the largest hydrocarbon disasters in US history. The estimated 5 million barrels of oil that poured into the Gulf of Mexico had a devastating impact on the natural environment, as well as on the livelihoods of communities residing along the coastal region. This paper explores resilience in individuals that identify as Vietnamese, presenting findings from a study of three Gulf Coast communities impacted by the DWH oil spill. A 60-min, in-person survey was administered to a total of 326 residents from the Gulf Coast communities of Port Sulphur, LA, Galliano, LA, and Bayou La Batre, AL. Logistic regression outcome and key predictors models were used to determine the probability of having higher levels of resilience. The results of this study highlight the role of age, education, and racial/ethnic identification in fostering resilience following disaster exposure. Identifying as Vietnamese was associated with a decrease in the odds of achieving above-threshold levels of resilience. This study may be viewed as the foundation from which to further explore the unique risk and protective profiles of the Vietnamese population, particularly in regard to those residing in critically vulnerable and disaster-prone areas

‘Come Back at Us’: Reflections on Researcher-Community Partnerships during a Post-Oil Spill Gulf Coast Resilience Study

This paper presents findings from eight post-hoc interviews with individuals representing the key community partner organizations that facilitated and hosted data collection for an in-person mixed-methods survey about disaster resilience and preparedness in three communities on the Gulf Coast (U.S.) impacted by the Deepwater Horizon oil spill and numerous disasters from natural hazards. We submit our analysis of these follow-up interviews with community partners as a case study to provide a set of recommendations for future community-engaged research practices, particularly in the field of environmental and disaster resilience. Input from community partners stressed the importance of engaging with local community brokers to enhance trust in research; researcher-partner communication; and researcher interaction with community residents that respects local knowledge and culture. The partners indicated that even communities that have often been the subjects of post-disaster studies are receptive to research participation, especially when the effects of disasters are long-term and ongoing. Recommendations include using research methodologies that are congruent with post-disaster community characteristics such as educational attainment; collaborating with community partners to disseminate research findings; and incorporating theories and practices that center critical reflection and consider power dynamics when working with communities that have experienced disaster and trauma

Resilience: Examining the Impacts of the Deepwater Horizon Oil Spill on the Gulf Coast Vietnamese American Community

The 2010 Deepwater Horizon oil spill (DWH) was one of the largest hydrocarbon disasters in US history. The estimated 5 million barrels of oil that poured into the Gulf of Mexico had a devastating impact on the natural environment, as well as on the livelihoods of communities residing along the coastal region. This paper explores resilience in individuals that identify as Vietnamese, presenting findings from a study of three Gulf Coast communities impacted by the DWH oil spill. A 60 min, in-person survey was administered to a total of 326 residents from the Gulf Coast communities of Port Sulphur, LA, Galliano, LA, and Bayou La Batre, AL. Logistic regression outcome and key predictors models were used to determine the probability of having higher levels of resilience. The results of this study highlight the role of age, education, and racial/ethnic identification in fostering resilience following disaster exposure. Identifying as Vietnamese was associated with a decrease in the odds of achieving above-threshold levels of resilience. This study may be viewed as the foundation from which to further explore the unique risk and protective profiles of the Vietnamese population, particularly in regard to those residing in critically vulnerable and disaster-prone areas

Perceptions of patients, caregivers, and healthcare providers of idiopathic inflammatory myopathies: An international OMERACT Study

Objective. Patient-reported outcome measures (PROM) that incorporate the patient perspective have not been well established in idiopathic inflammatory myopathies (IIM). As part of our goal to develop IIM-specific PROM, the Outcome Measures in Rheumatology (OMERACT) Myositis special interest group sought to determine which aspects of disease and its effects are important to patients and healthcare providers (HCP). Methods. Based on a prior qualitative content analysis of focus groups, an initial list of 24 candidate domains was constructed. We subsequently conducted an international survey to identify the importance of each of the 24 domains to be assessed in clinical research. Patients with IIM, their caregivers, and HCP treating IIM completed the survey. Results. In this survey, a total of 638 respondents completed the survey, consisting of 510 patients, 101 HCP, and 27 caregivers from 48 countries. Overall, patients were more likely to rank “fatigue,” “cognitive impact,” and “difficulty sleeping” higher compared with HCP, who ranked “joint symptoms,” “lung symptoms,” and “dysphagia” higher. Both patients and providers rated muscle symptoms as their top domain. In general, patients from different countries were in agreement on which domains were most important. One notable exception was that patients from Sweden and the Netherlands ranked lung symptoms significantly higher compared to other countries including the United States and Australia (mean weighted rankings of 2.86 and 2.04 vs 0.76 and 0.80, respectively; p < 0.0001). Conclusion. Substantial differences exist in how IIM is perceived by patients compared to HCP, with different domains prioritized. In contrast, patients’ ratings across the world were largely similar

A Draft Modified Core Domain Set for Patient-Reported Outcomes (PRO) in Patients with Idiopathic Inflammatory Myopathies (IIM): An Omeract Report

Background/Purpose: The OMERACT Myositis special interest group (SIG) represents clinicians, patients, and researchers from four continents. Focus groups were conducted including 61 patients on three countries resulting in a list of 26 domains (1). In collaboration with International Myositis Assessment Clinical Study Group (IMACS), our goal was to identify a set of core patient-reported outcomes (PRO) in regards to life impact important to assess in clinical trials and clinical practice in myositis. Methods: Patients with adult polymyositis, dermatomyositis, antisynthetase syndrome, or immune-mediated necrotizing myopathy (IMNM) in South Korea, Sweden and USA (N=638) responded to the first online modified Delphi in 2016. The second modified Delphi included patients (N=563), health care providers (HCP) (N=101), care givers (N=27) and regulatory agencies (n=xx) from multiple countries in 2017. A third modified Delphi was administered in 2018 including 410 patients, 109 HCP, 22 caregivers. Results: From this work, four domains were deemed mandatory to measure in all clinical trials for IIM: fatigue, pain, levels of physical activity, and muscle symptoms (Figure 1). Additional optional domains include skin symptoms, lung symptoms, and joint symptoms. Several other domains were deemed important to study with further research efforts including sleeping difficulty, cognitive distress, ability to work, and emotional distress. Conclusion: A draft set of core PRO has been developed through validated methods based on OMERACT guidelines. Fatigue, pain, levels of physical activity and muscle symptoms were included in the inner circle and should always be used in clinical trials in IIM. We next seek to develop corresponding instruments with each of these domains with future efforts

OMERACT 2018 Modified Patient-reported outcome domain core set in the life impact area for adult idiopathic inflammatory myopathies

Objective. To present and vote on a myositis modified patient-reported outcome core domain set in the life impact area at the Outcome Measures in Rheumatology (OMERACT) 2018. Methods. Based on results from international focus groups and Delphi surveys, a draft core set was developed. Results. Domains muscle symptoms, fatigue, level of physical activity, and pain reached ≥ 70% consensus and were mandatory to assess in all trials. Domains lung, joint, and skin symptoms were mandatory in specific circumstances. This core set was endorsed by > 85% at OMERACT 2018. Conclusion. We propose a life impact core set for patients with idiopathic inflammatory myopathies and will proceed with instrument selections

Assessing the content validity of patient-reported outcome measures in adult myositis: A report from the OMERACT myositis working group

Objective To investigate the content validity of several patient-reported outcome measures (PROMs) in patients with idiopathic inflammatory myopathies (IIM). Methods Seven individual PROM instruments were selected by the Outcome Measures in Rheumatology (OMERACT) Myositis Working Group relating to the following domains: pain, fatigue, physical function and physical activity. Twenty patients from the Johns Hopkins Myositis Center were selected for one-on-one face-to-face or phone interviews for cognitive interviewing of individual PROMs to assess comprehension and content validity. Additionally, patients were asked if they thought muscle symptoms, an area originally identified in qualitative studies, were encapsulated by the other four domains. Results The majority of patients (>70%) felt that each of the instruments was clear, easy to read and understand, and could be used for assessment of its domain. Two-thirds (66%) of patients felt that ‘muscle symptoms’ were captured by the other domains. Conclusions We provided evidence to support adequate content validity for several PROMs. Further research is needed to determine whether ‘muscle symptoms’ warrant a separate domain