COVID-19 vaccinations and infections among individuals with systemic sclerosis: A Scleroderma Patient-centered Intervention Network (SPIN) Cohort study.

BACKGROUND/PURPOSE: We previously surveyed adults with systemic sclerosis (SSc) regarding COVID-19 vaccination in April-May 2021. The objective of the present study was to update through June-July 2022 and assess self-reported (1) COVID-19 vaccination rates, including boosters; (2) vaccine-related adverse events; (3) peri­vaccination immunosuppressive medication management; (4) vaccine hesitancy; and (5) prevalence and severity of COVID-19 infections. METHODS: In April-May 2021 and June-July 2022, SPIN Cohort participants completed surveys on COVID-19 vaccination and infection. Primary vaccine series was defined according to the standard for each COVID-19 vaccine; additional vaccine administrations were considered booster doses. Fully vaccinated was defined as having completed a primary vaccine series and at least one booster dose. RESULTS: 544 participants completed the 2021 survey only, 101 the 2022 survey only, and 388 both surveys. Among 489 participants with 2022 data, 437 (89 %) had received both primary and booster vaccines. Among all 1,033 participants, 960 (93 %) received at least one dose. At least one adverse reaction was reported by 34 % (330 of 960 participants) following first, 48 % (314 of 657 participants) following second, and 34 % (147 of 437 participants) following booster vaccine doses (primarily sore arm and fatigue); no severe adverse reactions were reported. SSc symptom worsening was reported in 6 % (53 of 960) after the first, 6 % after the second (39 of 657), and 4 % (17 of 437) after the booster dose. Of participants taking methotrexate or mycophenolate (including Cellcept or Myfortic), 34 of 266 (13 %) reported that they temporarily stopped or decreased their medication at the first dose, 32 of 215 (15 %) at the second dose, and 28 of 148 (19 %) for booster vaccination. Of 52 individuals not fully vaccinated with primary and booster doses in 2022, 29 (56 %) reported worry about vaccine related SSc flares. 172 of 489 (35 %) 2022 participants reported a history of at least one COVID-19 infection; 114 (66 %) occurred after receiving at least a primary vaccine series. Among initial COVID-19 infections, 9 (5 %) were asymptomatic, 66 (38 %) involved mild symptoms, 82 (48 %) moderate symptoms, and 15 (9 %) required hospitalization. CONCLUSION: Most people with SSc in the study were fully vaccinated, and most continued their methotrexate or mycophenolate post-primary and booster vaccinations. Over half of vaccine-hesitant participants were concerned regarding risk of SSc flare; however, few vaccinated participants reported this. These data may be useful for counselling people with SSc regarding COVID-19 vaccine safety and outcomes.

Understanding the barriers to integrating maternal and mental health at primary health care in Vietnam.

The prevalence of common perinatal mental disorders in Vietnam ranges from 16.9% to 39.9%, and substantial treatment gaps have been identified at all levels. This paper explores constraints to the integration of maternal and mental health services at the primary healthcare level and the implications for the health system's responsiveness to the needs and expectations of pregnant women with mental health conditions in Vietnam. As part of the RESPONSE project, a three-phase realist evaluation study, we present Phase 1 findings, which employed systematic and scoping literature reviews and qualitative data collection (focus groups and interviews) with key health system actors in Bac Giang province, Vietnam, to understand the barriers to maternal mental healthcare provision, utilization and integration strategies. A four-level framing of the barriers to integrating perinatal mental health services in Vietnam was used in reporting findings, which comprised individual, sociocultural, organizational and structural levels. At the sociocultural and structural levels, these barriers included cultural beliefs about the holistic notion of physical and mental health, stigma towards mental health, biomedical approach to healthcare services, absence of comprehensive mental health policy and a lack of mental health workforce. At the organizational level, there was an absence of clinical guidelines on the integration of mental health in routine antenatal visits, a shortage of staff and poor health facilities. Finally, at the provider level, a lack of knowledge and training on mental health was identified. The integration of mental health into routine antenatal visits at the primary care level has the potential help to reduce stigma towards mental health and improve health system responsiveness by providing services closer to the local level, offering prompt attention, better choice of services and better communication while ensuring privacy and confidentiality of services. This can improve the demand for mental health services and help reduce the delay of care-seeking.

Systemic Sclerosis in Individuals With Exposure to World Trade Center Ground Zero Rescue and Recovery Efforts: A Case Series.

OBJECTIVE: The World Trade Center (WTC) attack in New York resulted in a dust plume containing silica, hydrocarbons, and asbestos. Autoimmune disorders have been reported among those with WTC site exposure. The characteristics of individuals developing systemic sclerosis (SSc) have not been previously described. The purpose of this study was to describe the features of patients with SSc with WTC exposure. METHODS: Data were collected from 11 patients with SSc or SSc spectrum conditions who reported exposure to the WTC site. Seven patients completed an exposure assessment. RESULTS: Of the 11 patients, the majority (n = 8) were female. The median (range) for age at diagnosis was 46 (36-75) years, time between exposure and first non-Raynaud phenomenon SSc symptom was 8 (1-19) years, and time between exposure and diagnosis was 11 (2-18) years. Fifty-five percent had SSc onset > 5 years from WTC exposure. Five patients had limited cutaneous SSc, 3 patients had diffuse cutaneous SSc, 1 patient with SSc features met criteria for mixed connective tissue disease (CTD), and 2 patients had undifferentiated CTD with features of SSc. Four patients had overlapping features with other CTDs. Interstitial lung disease (ILD) was present in 10 patients. Five of 11 patients had a history of tobacco use. Seven of 7 patients who completed the questionnaire reported other hazardous exposures outside of WTC. Of these, only 2 patients reported personal protective equipment use. CONCLUSION: A high frequency of ILD and overlap features were observed among patients with SSc with WTC exposure. Future studies are needed to characterize this association.

What can one legitimately expect from a health system? A conceptual analysis and a proposal for research and action.

In 2007, the WHO proposed the Building Blocks Framework and articulated 'responsiveness' as one of the four goals for health systems. While researchers have studied and measured health systems responsiveness since, several aspects of the concept remain unexamined, including, understanding the notion of 'legitimate expectations'-a notion central to the definition of responsiveness. We begin this analysis by providing a conceptual overview of how 'legitimacy' is understood in key social science disciplines. Drawing on insights from this overview, we examine how 'legitimacy' is understood in the literature on health systems responsiveness and reveal that there is currently little critical engagement with this notion of the 'legitimacy' of expectations. In response, we unpack the concept of 'legitimate' expectations and propose approaches and areas for reflection, research, and action. We conclude that contestation, and ongoing negotiation of entrenched health system processes and norms which establish citizens' 'legitimate' expectations of health systems, is needed-through processes that ensure equitable and wide participation. We also call on researchers, in their capacity as key health policy actors, to trigger and initiate processes and help create equitable spaces for citizens to participate in establishing 'legitimate' expectations of health systems.

Using the WHO Self-Reporting Questionnaire-20 (SRQ-20) to Detect Symptoms of Common Mental Disorders among Pregnant Women in Vietnam: a Validation Study.

Purpose: Detection of antenatal common mental disorders in low-resource settings like Vietnam is important and requires a reliable, valid and practical screening tool. Currently, there is no such tool validated for use among pregnant women in Vietnam. This study aims to assess the validity of the Vietnamese version of the 20-item Self Reporting Questionnaire (SRQ-20) by evaluating its reliability, factorial structure, and performance in detecting common mental disorder (CMD) symptoms, thereby identifying the optimum cut-off score for CMD screening among pregnant women in Vietnam. Participants and Methods: A total of 210 pregnant women from four rural communes participated in a face-to-face interview using the Vietnamese version of the SRQ-20, followed by a clinical diagnostic interview based on ICD-10 diagnostic criteria of CMDs. The reliability of the SRQ-20 was assessed by calculating the scale's Cronbach's alpha to measure internal consistency. Factor analyses were undertaken to examine the factor structure of the instrument. The Receiver Operating Characteristic (ROC) curve analysis was performed to assess the performance of the SRQ-20 against the clinical diagnosis and to identify the optimum cut-off score. Results: Internal consistency was good, with a Cronbach's alpha of 0.87. Factor analyses resulted in a 4-factor solution. The area under the ROC curve (AUC) for detection of CMDs was 0.90. The optimum cut-off score of the SRQ-20 for detection of CMD symptoms among Vietnamese pregnant women was 5/6. Conclusion: The Vietnamese version of the SRQ-20 has the capacity to detect CMDs among pregnant women effectively and is recommended for use as a screening tool for CMDs in antenatal care settings in Vietnam.

CONQUER Scleroderma: association of gastrointestinal tract symptoms in early disease with resource utilization.

OBJECTIVES: SSc is associated with increased health-care resource utilization and economic burden. The Collaborative National Quality and Efficacy Registry (CONQUER) is a US-based collaborative that collects longitudinal follow-up data on SSc patients with <5 years of disease duration enrolled at scleroderma centres in the USA. The objective of this study was to investigate the relationship between gastrointestinal tract symptoms and self-reported resource utilization in CONQUER participants. METHODS: CONQUER participants who had completed a baseline and 12-month Gastrointestinal Tract Questionnaire (GIT 2.0) and a Resource Utilization Questionnaire (RUQ) were included in this analysis. Patients were categorized by total GIT 2.0 severity: none-to-mild (0-0.49); moderate (0.50-1.00), and severe-to-very severe (1.01-3.00). Clinical features and medication exposures were examined in each of these categories. The 12-month RUQ responses were summarized by GIT 2.0 score categories at 12 months. RESULTS: Among the 211 CONQUER participants who met the inclusion criteria, most (64%) had mild GIT symptoms, 26% had moderate symptoms, and 10% severe GIT symptoms at 12 months. The categorization of GIT total severity score by RUQ showed that more upper endoscopy procedures and inpatient hospitalization occurred in the CONQUER participants with severe GIT symptoms. These patients with severe GIT symptoms also reported the use of more adaptive equipment. CONCLUSION: This report from the CONQUER cohort suggests that severe GIT symptoms result in more resource utilization. It is especially important to understand resource utilization in early disease cohorts when disease activity, rather than damage, primarily contributes to health-related costs of SSc.

A critical interpretive synthesis of migrants' experiences of the Australian health system.

While the health of and healthcare use by migrants has received significant scholarly and policy attention in Australia, current debates highlight that a critical examination of the theoretical underpinnings of these inquiries and responses is needed. We conducted a systematic review and critical interpretive synthesis (CIS) to critically examine how the policy and scholarly literature conceptualises migrants' interactions with and experiences of the Australian health system. Guided by PRISMA, we searched for literature without imposing any limits. We also searched key State and Federal Government websites for relevant policy documents. Our initially broad inclusion criteria became refined as the CIS progressed. We prioritised the likely relevance and theoretical contribution of the papers to our inquiry over methodological quality. The CIS of 104 papers revealed that the Australian scholarly literature and policy documents consistently homogenise and reduce migrants according to an assumed, (1) cultural identity, (2) linguistic affiliation, and/or (3) broad geographic origin. Based on these three critiques and drawing on the theoretical literature, we propose a synthesising argument on how the Australian literature could better conceptualise migrants' experiences of the Australian health system. We contend that both research and policy should explicitly recognise and engage with the multifaceted and shifting ways that migrants define themselves, generally, and during their encounters with destination country health systems. Engagement with this notion is necessary for also understanding how aspects of migrants' identities are dynamically co-constructed during their interactions with the health system. These understandings have implications for improving the design and implementation of policies and programs directed at improving the responsiveness of Australia's health system to the needs and expectations of migrant communities specifically, and destination countries broadly.

2022 American College of Rheumatology Guideline for Vaccinations in Patients With Rheumatic and Musculoskeletal Diseases.

OBJECTIVE: To provide evidence-based recommendations on the use of vaccinations in children and adults with rheumatic and musculoskeletal diseases (RMDs). METHODS: This guideline follows American College of Rheumatology (ACR) policy guiding management of conflicts of interest and disclosures and the ACR guideline development process, which includes the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. It also adheres to the Appraisal of Guidelines for Research and Evaluation (AGREE) criteria. A core leadership team consisting of adult and pediatric rheumatologists and a guideline methodologist drafted clinical population, intervention, comparator, outcomes (PICO) questions. A review team performed a systematic literature review for the PICO questions, graded the quality of evidence, and produced an evidence report. An expert Voting Panel reviewed the evidence and formulated recommendations. The panel included adult and pediatric rheumatology providers, infectious diseases specialists, and patient representatives. Consensus required ≥70% agreement on both the direction and strength of each recommendation. RESULTS: This guideline includes expanded indications for some vaccines in patients with RMDs, as well as guidance on whether to hold immunosuppressive medications or delay vaccination to maximize vaccine immunogenicity and efficacy. Safe approaches to the use of live attenuated vaccines in patients taking immunosuppressive medications are also addressed. Most recommendations are conditional and had low quality of supporting evidence. CONCLUSION: Application of these recommendations should consider patients' individual risk for vaccine-preventable illness and for disease flares, particularly if immunosuppressive medications are held for vaccination. Shared decision-making with patients is encouraged in clinical settings.

2022 American College of Rheumatology Guideline for Vaccinations in Patients With Rheumatic and Musculoskeletal Diseases.

OBJECTIVE: To provide evidence-based recommendations on the use of vaccinations in children and adults with rheumatic and musculoskeletal diseases (RMDs). METHODS: This guideline follows American College of Rheumatology (ACR) policy guiding management of conflicts of interest and disclosures and the ACR guideline development process, which includes the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. It also adheres to the Appraisal of Guidelines for Research and Evaluation (AGREE) criteria. A core leadership team consisting of adult and pediatric rheumatologists and a guideline methodologist drafted clinical population, intervention, comparator, outcomes (PICO) questions. A review team performed a systematic literature review for the PICO questions, graded the quality of evidence, and produced an evidence report. An expert Voting Panel reviewed the evidence and formulated recommendations. The panel included adult and pediatric rheumatology providers, infectious diseases specialists, and patient representatives. Consensus required ≥70% agreement on both the direction and strength of each recommendation. RESULTS: This guideline includes expanded indications for some vaccines in patients with RMDs, as well as guidance on whether to hold immunosuppressive medications or delay vaccination to maximize vaccine immunogenicity and efficacy. Safe approaches to the use of live attenuated vaccines in patients taking immunosuppressive medications are also addressed. Most recommendations are conditional and had low quality of supporting evidence. CONCLUSION: Application of these recommendations should consider patients' individual risk for vaccine-preventable illness and for disease flares, particularly if immunosuppressive medications are held for vaccination. Shared decision-making with patients is encouraged in clinical settings.

Chronic activation of pDCs in autoimmunity is linked to dysregulated ER stress and metabolic responses.

Plasmacytoid dendritic cells (pDCs) chronically produce type I interferon (IFN-I) in autoimmune diseases, including systemic sclerosis (SSc) and systemic lupus erythematosus (SLE). We report that the IRE1α-XBP1 branch of the unfolded protein response (UPR) inhibits IFN-α production by TLR7- or TLR9-activated pDCs. In SSc patients, UPR gene expression was reduced in pDCs, which inversely correlated with IFN-I-stimulated gene expression. CXCL4, a chemokine highly secreted in SSc patients, downregulated IRE1α-XBP1-controlled genes and promoted IFN-α production by pDCs. Mechanistically, IRE1α-XBP1 activation rewired glycolysis to serine biosynthesis by inducing phosphoglycerate dehydrogenase (PHGDH) expression. This process reduced pyruvate access to the tricarboxylic acid (TCA) cycle and blunted mitochondrial ATP generation, which are essential for pDC IFN-I responses. Notably, PHGDH expression was reduced in pDCs from patients with SSc and SLE, and pharmacological blockade of TCA cycle reactions inhibited IFN-I responses in pDCs from these patients. Hence, modulating the IRE1α-XBP1-PHGDH axis may represent a hitherto unexplored strategy for alleviating chronic pDC activation in autoimmune disorders.

Peoples' expectations of healthcare: A conceptual review and proposed analytical framework.

Expectations shape how one experiences the healthcare one receives. In this paper we argue that the current conceptualisations of expectations within the healthcare literature have much to gain from the many recent and adjacent conceptual developments in other disciplines. The concept of expectations has been extensively studied across disciplines - we review the key texts on the subject in the business, management, social psychology, and sociology literatures to provide a conceptual overview and propose an integrative analytical framework for better understanding individuals' expectations in healthcare. We argue that peoples' expectations of a care encounter are usefully understood as being shaped by their social locations at particular points of time, which is at the intersection of multiple social structures and relations. Peoples' future expectations of care may also be influenced by the experiences of past and current care encounters, framed again by intersecting social structures and relations at that point in time. We demonstrate how an intersectional, translocational and relational analytical approach can allow researchers and practitioners to consider how peoples' social locations shape their expectations of care, not only within a given social environment, but at certain points in time and over time. We emphasise that, given the mobilities and mixing societies are experiencing globally, such an approach is particularly useful for understanding healthcare-related expectations and experiences of all.