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New Yorkers face a multitude of health and safety risks that are exacerbated by a changing climate. These risks include direct impacts from extreme weather events and other climate hazards, as well as indirect impacts occurring through a chain of interactions. Physical safety, physical health, and mental health are all part of the equation-as are the many nonclimate factors that interact with climate change to influence health outcomes. This chapter provides an updated assessment of all these topics at the intersection of climate change, public health and safety, and equity in the state of New York. Key findings are presented below., (© 2024 The Author(s). Annals of the New York Academy of Sciences published by Wiley Periodicals LLC on behalf of The New York Academy of Sciences.)

Objective: To present a series of case studies from our respective countries and disciplines on approaches to implementing the Planetary Health Education Framework in university health professional education programs, and to propose a curriculum implementation and evaluation toolbox for educators to facilitate the adoption of similar initiatives in their programs. We emphasize the importance of applying an Indigenous lens to curriculum needs assessment, development, implementation, and evaluation., Methods: Case studies from Australia and United States were collated using a six-stage design-based educational research framework (Focus, Formulation, Contextualization, Definition, Implementation, Evaluation) for teaching planetary health and methods of curriculum evaluation. These components were then mapped to derive the curriculum implementation toolbox reflecting the six-stage design-based educational research framework., Results: The case studies demonstrated different approaches to successful integration of the Planetary Health Education Framework in medicine, nursing, public health, and allied health disciplines. This integration often involved Indigenous perspectives on environmental stewardship, holistic health, and community well-being into the curriculum. The case studies also highlighted the importance of community engagement, cultural competency, and interdisciplinary collaboration in curriculum development. Findings from case studies were used to propose a curriculum implementation toolbox to assist educators in adapting and integrating planetary health education into their own programs., Discussion: While valuable frameworks for teaching planetary health in health science programs exist, challenges remain in implementing these frameworks in real-world educational environments. The proposed curriculum implementation toolbox offers practical strategies and resources for educators to incorporate these principles into their teaching. Additionally, the case studies reported here contribute to the growing body of literature on planetary health education pertinent to addressing the triple planetary crisis., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Lokmic-Tomkins, Barbour, LeClair, Luebke, McGuinness, Limaye, Pillai, Flynn, Kamp, Leder and Patz.)

Alzheimer's disease and related dementias (ADRD) represent a public health crisis poised to worsen in a changing climate. Substantial dementia burden is modifiable, attributable to risk rooted in social and environmental conditions. Climate change threatens older populations in numerous ways, but implications for cognitive aging are poorly understood. We illuminate key mechanisms by which climate change will shape incidence and lived experiences of ADRD, and propose a framework for strengthening research, clinical, and policy actions around cognitive health in the context of climate change. Direct impacts and indirect risk pathways operating through built, social, interpersonal, and biomedical systems are highlighted. Air pollution compromises brain health directly and via systemic cardiovascular and respiratory ailments. Flooding and extreme temperatures constrain health behaviors like physical activity and sleep. Medical care resulting from climate-related health shocks imposes economic and emotional tolls on people living with dementia and caregivers. Throughout, inequitable distributions of climate-exacerbated risks and adaptive resources compound existing disparities in ADRD incidence, comorbidities, and care burden. Translational research, including work prioritizing underserved communities, is crucial. A mechanistic framework can guide research questions and methods and identify clinical- and policy-level intervention loci for prevention and mitigation of climate-related impacts on ADRD risk and burden., (© The Author(s) 2023. Published by Oxford University Press on behalf of The Gerontological Society of America.)

Climate change-driven temperature increases worsen air quality in places where coal combustion powers electricity for air conditioning. Climate solutions that substitute clean and renewable energy in place of polluting coal and promote adaptation to warming through reflective cool roofs can reduce cooling energy demand in buildings, lower power sector carbon emissions, and improve air quality and health. We investigate the air quality and health co-benefits of climate solutions in Ahmedabad, India-a city where air pollution levels exceed national health-based standards-through an interdisciplinary modeling approach. Using a 2018 baseline, we quantify changes in fine particulate matter (PM 2.5 ) air pollution and all-cause mortality in 2030 from increasing renewable energy use (mitigation) and expanding Ahmedabad's cool roofs heat resilience program (adaptation). We apply local demographic and health data and compare a 2030 mitigation and adaptation (M&A) scenario to a 2030 business-as-usual (BAU) scenario (without climate change response actions), each relative to 2018 pollution levels. We estimate that the 2030 BAU scenario results in an increase of PM 2.5 air pollution of 4.13 µ g m -3 from 2018 compared to a 0.11 µ g m -3 decline from 2018 under the 2030 M&A scenario. Reduced PM 2.5 air pollution under 2030 M&A results in 1216-1414 fewer premature all-cause deaths annually compared to 2030 BAU. Achievement of National Clean Air Programme, National Ambient Air Quality Standards, or World Health Organization annual PM 2.5 Air Quality Guideline targets in 2030 results in up to 6510, 9047, or 17 369 fewer annual deaths, respectively, relative to 2030 BAU. This comprehensive modeling method is adaptable to estimate local air quality and health co-benefits in other settings by integrating climate, energy, cooling, land cover, air pollution, and health data. Our findings demonstrate that city-level climate change response policies can achieve substantial air quality and health co-benefits. Such work can inform public discourse on the near-term health benefits of mitigation and adaptation., (© 2023 Natural Resources Defense Council.)

Responding effectively to intensifying climate change hazards to protect human health in personal and professional settings is an urgent and pressing challenge. This will require collaboration and subject matter expertise of people across the life course and occupations. In this perspective piece, we build on a previously published compilation of climate and health literacy elements to explore tangible opportunities to strengthen climate and health understanding among individuals spanning educational levels, professional settings, and societal needs. Educational materials addressing climate change and health linkages have historically focused on K-12, college, post-graduate education, and continuing medical education, with less attention devoted to reaching students in trade schools and other professional settings. Here, we outline a flexible blueprint for strengthening climate and health literacy among all people by targeting education in a way that is relevant for each age group or profession. In particular, we discuss the idea of professional adaptability as a way to design practical climate and health training for people currently in the workforce., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Grabow, Stull, Hahn and Limaye.)

Competing Interests: I declare no competing interests.

Most of India's current electricity demand is met by combustion of fossil fuels, particularly coal. But the country has embarked on a major expansion of renewable energy and aims for half of its electricity needs to be met by renewable sources by 2030. As climate change-driven temperature increases continue to threaten India's population and drive increased demand for air conditioning, there is a need to estimate the local benefits of policies that increase renewable energy capacity and reduce cooling demand in buildings. We investigate the impacts of climate change-driven temperature increases, along with population and economic growth, on demand for electricity to cool buildings in the Indian city of Ahmedabad between 2018 and 2030. We estimate the share of energy demand met by coal-fired power plants versus renewable energy in 2030, and the cooling energy demand effects of expanded cool roof adaptation in the city. We find renewable energy capacity could increase from meeting 9% of cooling energy demand in 2018 to 45% in 2030. Our modeling indicates a near doubling in total electricity supply and a nearly threefold growth in cooling demand by 2030. Expansion of cool roofs to 20% of total roof area (associated with a 0.21 TWh reduction in cooling demand between 2018 and 2030) could more than offset the city's climate change-driven 2030 increase in cooling demand (0.17 TWh/year). This study establishes a framework for linking climate, land cover, and energy models to help policymakers better prepare for growing cooling energy demand under a changing climate., Competing Interests: Conflict of interestsThe authors report no conflicts of interest and no competing interests., (© The Author(s) 2022.)

Climate change-driven health impacts are serious, widespread, and costly. Importantly, such damages are largely absent from policy debates around the costs of delay and inaction on this crisis. While climate change is a global problem, its impacts are localized and personal, and there is growing demand for specific information on how climate change affects human health in different places. Existing research indicates that climate-fueled health problems are growing, and that investments in reducing carbon pollution and improving community resilience could help to avoid tens to hundreds of billions of dollars in climate-sensitive health impacts across the USA each year, including those stemming from extreme heat, air pollution, hurricanes, and wildfires. Science that explores the underappreciated local health impacts and health-related costs of climate change can enhance advocacy by demonstrating the need to both address the root causes of climate change and enhance climate resilience in vulnerable communities. The climate crisis has historically been predominantly conceived as a global environmental challenge; examination of climate impacts on public health enables researchers to localize this urgent problem for members of the public and policymakers. In turn, approaches to climate science that focus on health can make dangerous climate impacts and the need for cost-effective solutions more salient and tangible., Competing Interests: Conflict of interestThe authors declare no competing interests., (© The Author(s) 2021.)

A new generation of activists is calling for bold responses to the climate crisis. Although young people are motivated to act on climate issues, existing educational frameworks do not adequately prepare them by addressing the scope and complexity of the human health risks associated with climate change. We adapted the US government's climate literacy principles to propose a definition and corresponding set of elements for a concept we term climate and health literacy . We conducted a scoping review to assess how the peer-reviewed literature addresses these elements. Our analysis reveals a focus on training health professionals; more international than US domestic content; and limited information about data and models, fossil fuels, and equity. We propose developing a framework that builds on the elements to support a broader educational agenda that prepares students and future leaders to recognize the complex health ramifications of a changing climate.

National and international assessments have drawn attention to the substantial economic risks of climate change. The costs of climate-sensitive health outcomes responsive to meteorological or seasonal patterns are among the least studied of those risks. In this article we describe how cost valuation analyses that relate climate-sensitive health outcomes to damages in economic terms can illuminate the costs of inaction on the climate crisis and the economic savings of addressing this problem. We identify major challenges to expanding the application of climate-health valuation research and suggest solutions to overcome these obstacles to better characterize the burden of climate-sensitive health outcomes and health disparities. The projected health and economic harms of climate-sensitive risks could be enormous if climate change continues to accelerate and communities are not prepared to reduce or prevent their impact. Expanded valuation of climate-sensitive health outcomes can inform policies that slow climate change and promote stronger investments in health-protective climate change adaptation efforts.

Background: Modeling suggests that climate change mitigation actions can have substantial human health benefits that accrue quickly and locally. Documenting the benefits can help drive more ambitious and health-protective climate change mitigation actions; however, documenting the adverse health effects can help to avoid them. Estimating the health effects of mitigation (HEM) actions can help policy makers prioritize investments based not only on mitigation potential but also on expected health benefits. To date, however, the wide range of incompatible approaches taken to developing and reporting HEM estimates has limited their comparability and usefulness to policymakers., Objective: The objective of this effort was to generate guidance for modeling studies on scoping, estimating, and reporting population health effects from climate change mitigation actions., Methods: An expert panel of HEM researchers was recruited to participate in developing guidance for conducting HEM studies. The primary literature and a synthesis of HEM studies were provided to the panel. Panel members then participated in a modified Delphi exercise to identify areas of consensus regarding HEM estimation. Finally, the panel met to review and discuss consensus findings, resolve remaining differences, and generate guidance regarding conducting HEM studies., Results: The panel generated a checklist of recommendations regarding stakeholder engagement: HEM modeling, including model structure, scope and scale, demographics, time horizons, counterfactuals, health response functions, and metrics; parameterization and reporting; approaches to uncertainty and sensitivity analysis; accounting for policy uptake; and discounting., Discussion: This checklist provides guidance for conducting and reporting HEM estimates to make them more comparable and useful for policymakers. Harmonization of HEM estimates has the potential to lead to advances in and improved synthesis of policy-relevant research that can inform evidence-based decision making and practice. https://doi.org/10.1289/EHP6745.

Herein we report a case of sporadic inclusion-body myositis (sIBM) occurring at an unusually young age in a patient with primary Sjögren syndrome, and use the case to explore possible shared mechanisms for disease susceptibility. Possible factors may include the association of both conditions with the 8.1 ancestral haplotype; the presence of anti-cN1A antibodies, which, although considered specific for sIBM, are also seen in pSS; and the shared association with T-cell large granular lymphocyte leukemia (T-LGLL). Further evaluation of this patient did in fact reveal underlying T-LGLL and mechanisms by which T cells in sIBM may escape immune regulation and contribute to disease phenotype are explored. Despite myofiber infiltration with CD8-positive T cells in sIBM, and, although sIBM is traditionally considered treatment-refractory, we report a significant response to the anti-CD20 monoclonal antibody, rituximab, and discuss possible mechanisms by which this response may be mediated., (© 2020 Wiley Periodicals, Inc.)

Climate change threatens human health, but there remains a lack of evidence on the economic toll of climate-sensitive public health impacts. We characterize human mortality and morbidity costs associated with 10 climate-sensitive case study events spanning 11 US states in 2012: wildfires in Colorado and Washington, ozone air pollution in Nevada, extreme heat in Wisconsin, infectious disease outbreaks of tick-borne Lyme disease in Michigan and mosquito-borne West Nile virus in Texas, extreme weather in Ohio, impacts of Hurricane Sandy in New Jersey and New York, allergenic oak pollen in North Carolina, and harmful algal blooms on the Florida coast. Applying a consistent economic valuation approach to published studies and state estimates, we estimate total health-related costs from 917 deaths, 20,568 hospitalizations, and 17,857 emergency department visits of $10.0 billion in 2018 dollars, with a sensitivity range of $2.7-24.6 billion. Our estimates indicate that the financial burden of deaths, hospitalizations, emergency department visits, and associated medical care is a key dimension of the overall economic impact of climate-sensitive events. We found that mortality costs (i.e., the value of a statistical life) of $8.4 billion exceeded morbidity costs and lost wages ($1.6 billion combined). By better characterizing health damages in economic terms, this work helps to shed light on the burden climate-sensitive events already place on U.S. public health each year. In doing so, we provide a conceptual framework for broader estimation of climate-sensitive health-related costs. The high health-related costs associated with climate-sensitive events highlight the importance of actions to mitigate climate change and adapt to its unavoidable impacts., Competing Interests: The authors declare no conflicts of interest relevant to this study., (©2019. The Authors.)

Fine particulate matter (PM 2.5 , diameter ≤2.5 μm) is implicated as the most health-damaging air pollutant. Large cohort studies of chronic exposure to PM 2.5 and mortality risk are largely confined to areas with low to moderate ambient PM 2.5 concentrations and posit log-linear exposure-response functions. However, levels of PM 2.5 in developing countries such as India are typically much higher, causing unknown health effects. Integrated exposure-response functions for high PM 2.5 exposures encompassing risk estimates from ambient air, secondhand smoke, and active smoking exposures have been posited. We apply these functions to estimate the future cause-specific mortality risks associated with population-weighted ambient PM 2.5 exposures in India in 2030 using Greenhouse Gas-Air Pollution Interactions and Synergies (GAINS) model projections. The loss in statistical life expectancy (SLE) is calculated based on risk estimates and baseline mortality rates. Losses in SLE are aggregated and weighted using national age-adjusted, cause-specific mortality rates. 2030 PM 2.5 pollution in India reaches an annual mean of 74 μg/m³, nearly eight times the corresponding World Health Organization air quality guideline. The national average loss in SLE is 32.5 months (95% Confidence Interval (CI): 29.7⁻35.2, regional range: 8.5⁻42.0), compared to an average of 53.7 months (95% CI: 46.3⁻61.1) using methods currently applied in GAINS. Results indicate wide regional variation in health impacts, and these methods may still underestimate the total health burden caused by PM 2.5 exposures due to model assumptions on minimum age thresholds of pollution effects and a limited subset of health endpoints analyzed. Application of the revised exposure-response functions suggests that the most polluted areas in India will reap major health benefits only with substantial improvements in air quality.

Climate change will increase extreme heat-related health risks. To quantify the health impacts of mid-century climate change, we assess heat-related excess mortality across the eastern USA. Health risks are estimated using the US Environmental Protection Agency's Environmental Benefits Mapping and Analysis Program (BenMAP). Mid-century temperature estimates, downscaled using the Weather Research and Forecasting model, are compared to 2007 temperatures at 36 km and 12 km resolutions. Models indicate the average apparent and actual summer temperatures rise by 4.5° and 3.3° C, respectively. Warmer average apparent temperatures could cause 11,562 additional annual deaths (95% confidence interval, CI: 2641-20,095) due to cardiovascular stress in the population aged 65 years and above, while higher minimum temperatures could cause 8767 (95% CI: 5030-12,475) additional deaths each year. Modeled future climate data available at both coarse (36 km) and fine (12 km) resolutions predict significant human health impacts from warmer climates. The findings suggest that currently available information on future climates is sufficient to guide regional planning for the protection of public health. Higher resolution climate and demographic data are still needed to inform more targeted interventions.

Indian cities struggle with some of the highest ambient air pollution levels in the world. While national efforts are building momentum towards concerted action to reduce air pollution, individual cities are taking action on this challenge to protect communities from the many health problems caused by this harmful environmental exposure. In 2017, the city of Ahmedabad launched a regional air pollution monitoring and risk communication project, the Air Information and Response (AIR) Plan. The centerpiece of the plan is an air quality index developed by the Indian Institute of Tropical Meteorology’s System for Air Quality and Weather Forecasting and Research program that summarizes information from 10 new continuous air pollution monitoring stations in the region, each reporting data that can help people avoid harmful exposures and inform policy strategies to achieve cleaner air. This paper focuses on the motivation, development, and implementation of Ahmedabad’s AIR Plan. The project is discussed in terms of its collaborative roots, public health purpose in addressing the grave threat of air pollution (particularly to vulnerable groups), technical aspects in deploying air monitoring technology, and broader goals for the dissemination of an air quality index linked to specific health messages and suggested actions to reduce harmful exposures. The city of Ahmedabad is among the first cities in India where city leaders, state government, and civil society are proactively working together to address the country’s air pollution challenge with a focus on public health. The lessons learned from the development of the AIR Plan serve as a template for other cities aiming to address the heavy burden of air pollution on public health. Effective working relationships are vital since they form the foundation for long-term success and useful knowledge sharing beyond a single city.

Background: Climate change negatively impacts human health through heat stress and exposure to worsened air pollution, amongst other pathways. Indoor use of air conditioning can be an effective strategy to reduce heat exposure. However, increased air conditioning use increases emissions of air pollutants from power plants, in turn worsening air quality and human health impacts. We used an interdisciplinary linked model system to quantify the impacts of heat-driven adaptation through building cooling demand on air-quality-related health outcomes in a representative mid-century climate scenario., Methods and Findings: We used a modeling system that included downscaling historical and future climate data with the Weather Research and Forecasting (WRF) model, simulating building electricity demand using the Regional Building Energy Simulation System (RBESS), simulating power sector production and emissions using MyPower, simulating ambient air quality using the Community Multiscale Air Quality (CMAQ) model, and calculating the incidence of adverse health outcomes using the Environmental Benefits Mapping and Analysis Program (BenMAP). We performed simulations for a representative present-day climate scenario and 2 representative mid-century climate scenarios, with and without exacerbated power sector emissions from adaptation in building energy use. We find that by mid-century, climate change alone can increase fine particulate matter (PM2.5) concentrations by 58.6% (2.50 μg/m3) and ozone (O3) by 14.9% (8.06 parts per billion by volume [ppbv]) for the month of July. A larger change is found when comparing the present day to the combined impact of climate change and increased building energy use, where PM2.5 increases 61.1% (2.60 μg/m3) and O3 increases 15.9% (8.64 ppbv). Therefore, 3.8% of the total increase in PM2.5 and 6.7% of the total increase in O3 is attributable to adaptive behavior (extra air conditioning use). Health impacts assessment finds that for a mid-century climate change scenario (with adaptation), annual PM2.5-related adult mortality increases by 13,547 deaths (14 concentration-response functions with mean incidence range of 1,320 to 26,481, approximately US$126 billion cost) and annual O3-related adult mortality increases by 3,514 deaths (3 functions with mean incidence range of 2,175 to 4,920, approximately US$32.5 billion cost), calculated as a 3-month summer estimate based on July modeling. Air conditioning adaptation accounts for 654 (range of 87 to 1,245) of the PM2.5-related deaths (approximately US$6 billion cost, a 4.8% increase above climate change impacts alone) and 315 (range of 198 to 438) of the O3-related deaths (approximately US$3 billion cost, an 8.7% increase above climate change impacts alone). Limitations of this study include modeling only a single month, based on 1 model-year of future climate simulations. As a result, we do not project the future, but rather describe the potential damages from interactions arising between climate, energy use, and air quality., Conclusions: This study examines the contribution of future air-pollution-related health damages that are caused by the power sector through heat-driven air conditioning adaptation in buildings. Results show that without intervention, approximately 5%-9% of exacerbated air-pollution-related mortality will be due to increases in power sector emissions from heat-driven building electricity demand. This analysis highlights the need for cleaner energy sources, energy efficiency, and energy conservation to meet our growing dependence on building cooling systems and simultaneously mitigate climate change., Competing Interests: I have read the journal’s policy and the authors of this manuscript have the following competing interests: PM has an ownership interest in the MyPower model used to generate power plant emissions estimates for this study. The data from this study are publicly available. JAP served as a Guest Editor on PLOS Medicine's Special Issue on Climate Change and Health.

The US Environmental Protection Agency (EPA) and other federal agencies face a number of challenges in interpreting and reconciling short-duration (seconds to minutes) readings from mobile and handheld air sensors with the longer duration averages (hours to days) associated with the National Ambient Air Quality Standards (NAAQS) for the criteria pollutants-particulate matter (PM), ozone, carbon monoxide, lead, nitrogen oxides, and sulfur oxides. Similar issues are equally relevant to the hazardous air pollutants (HAPs) where chemical-specific health effect reference values are the best indicators of exposure limits; values which are often based on a lifetime of continuous exposure. A multi-agency, staff-level Air Sensors Health Group (ASHG) was convened in 2013. ASHG represents a multi-institutional collaboration of Federal agencies devoted to discovery and discussion of sensor technologies, interpretation of sensor data, defining the state of sensor-related science across each institution, and provides consultation on how sensors might effectively be used to meet a wide range of research and decision support needs. ASHG focuses on several fronts: improving the understanding of what hand-held sensor technologies may be able to deliver; communicating what hand-held sensor readings can provide to a number of audiences; the challenges of how to integrate data generated by multiple entities using new and unproven technologies; and defining best practices in communicating health-related messages to various audiences. This review summarizes the challenges, successes, and promising tools of those initial ASHG efforts and Federal agency progress on crafting similar products for use with other NAAQS pollutants and the HAPs. NOTE: The opinions expressed are those of the authors and do not necessary represent the opinions of their Federal Agencies or the US Government. Mention of product names does not constitute endorsement., Competing Interests: Conflicts of Interest: The authors declare no conflict of interest.

Background: The accelerating accumulation of greenhouse gases in the Earth's atmosphere is changing global environmental conditions in unprecedented and potentially irreversible ways. Climate change poses a host of challenges to the health of populations through complex direct and indirect mechanisms. The direct effects include an increased frequency of heat waves, rising sea levels that threaten low-lying communities, anticipated extremes in the global hydrologic cycle (droughts, floods, and intense storms), and adverse effects on agricultural production and fisheries due to environmental stressors and changes in land use. Indirectly, climate change is anticipated to threaten health by worsening urban air pollution and increasing rates of infectious (particularly waterborne and vector-borne) disease transmission., Objective: To provide a state-of-the-science review on the health consequences of a changing climate., Findings: Environmental public health researchers have concluded that, on balance, adverse health outcomes will dominate under these changed climatic conditions. The number of pathways through which climate change can affect the health of populations makes this environmental health threat one of the largest and most formidable of the new century. Geographic location plays an influential role the potential for adverse health effects caused by climate change, and certain regions and populations are more vulnerable than others to expected health effects. Two kinds of strategies are available for responding to climate change: mitigation policies (which aim to reduce greenhouse gas emissions) and adaptation measures (relating to preparedness for anticipated impacts)., Conclusions: To better understand and address the complex nature of health risks posed by climate change, interdisciplinary collaboration is critical. Efforts to move beyond our current reliance on fossil fuels to cleaner, more sustainable energy sources may offer some of the greatest health opportunities in more than a century and cobenefits beyond the health sector. Because the nations least responsible for climate change are most vulnerable to its effects, the challenge to reduce greenhouse gas emissions is not merely technical, but also moral., (Copyright © 2014 Icahn School of Medicine at Mount Sinai. Published by Elsevier Inc. All rights reserved.)

Introduction: We sought to study the intensity and pattern of major histocompatibility complex (MHC) I and II expression in muscle from patients with biopsy-proven idiopathic inflammatory myositis (IIM) including the subgroups, polymyositis (PM), dermatomyositis (DM), and inclusion body myositis (IBM)., Methods: A total of 120 muscle biopsies (61 PM, 14 DM, and 45 IBM) were immunostained for MHC I and II. Staining was graded as follows. 0: no staining, 1+: ≤10% fibers, 2+: 10% to 25%, 3+: 25% to 50%, 4+: 50% to 99%, and 5+ 100%., Results: All IIM biopsies showed MHC I positivity; 93% showed MHC II positivity. The proportion of patients with MHC II score ≥3+ was higher in IBM than DM or PM. In DM, MHC I expression showed a perifascicular pattern. All IBM biopsies were immunopositive for MHC I and II; 30/45 were scored 5+., Discussion: Immunostaining for MHC I and II is a useful adjunctive test in diagnosis and subclassification of IIM.

Purpose: To report a case of systemic lupus erythematosus vaso-occlusive retinopathy with severe visual loss treated with intravenous pulsed cyclophosphamide., Methods: Retrospective interventional case report., Results: A 20-year-old Cambodian woman with newly diagnosed systemic lupus erythematosus presented with acute visual loss. Fluorescein fundus angiography demonstrated occlusive retinal vasculitis. Treatment with pulsed intravenous cyclophosphamide, intravenous methylprednisolone, and anticoagulation resulted in recovery of vision from count fingers to 6/6 in both eyes., Conclusion: Early aggressive immunosuppression and anticoagulation for systemic lupus erythematosus retinal vasculitis can be beneficial in preventing disease progression and restoring vision. Further studies are needed to compare dosage regimens.

The aim of this study was to determine the HLA and autoantibody associations of patients with histologically confirmed idiopathic inflammatory myositis (IIM). Serum and DNA were archived from South Australian patients with biopsy-proven dermatomyositis (DM), polymyositis (PM) and inclusion body myositis (IBM). HLA typing for Class I and II alleles was performed by serology and DNA-based technology, respectively, for 133 myositis patients and 166 Caucasian population-based controls. Myositis-specific and myositis-associated autoantibodies were detected by line immunoblot. All alleles of the 8.1AH were associated with myositis susceptibility. The B8-DR3 haplotype fragment conferred the strongest susceptibility (OR 2.9, 95% CI 1.8-4.6), and the B-DR region of other ancestral haplotypes was associated with myositis subgroups. Autoantibodies were present in 42/130 (32%) IIM patients and were more frequent in DM (11/17, 65%) than PM (23/70, 33%) or IBM (8/43, 19%), P = 0.002. Autoantibodies were associated with DRB1 03 (P = 0.0005) but also with DRB1 04 (P = 0.004). The frequency of autoantibodies in the three myositis subgroups mirrored the frequency of DR4. Polyarthralgia (±synovitis) was more common in DM/PM (30/76, 39%) than IBM (3/32, 9%), P = 0.004, and there was a strong ordinal association between the prevalence of autoantibodies and polyarthralgia ± synovitis (proportional OR = 5.5, 95% CI 2.3-13.7, P = 0.0004). The central MHC region confers the strongest susceptibility for IIM and also modulates disease phenotype. Our findings reveal a novel association of autoantibodies with DR4 and with arthralgia/synovitis in IIM and raise the possibility of a genetically (DR4) determined citrullination of myositis autoantigens expressed in muscle and synovium.

Aims: The long-terms complications of immunosuppressive and anti-inflammatory treatment in idiopathic inflammatory myositis (IIM) are unknown. We sought to determine the complications of these treatments in a large cohort of patients with biopsy-proven IIM., Methods: A South Australian database for patients with biopsy-proven IIM was established. Clinical details of patients including treatment received were recorded., Results: Forty-three patients with dermatomyositis (DM), 184 with polymyositis (PM) and 117 with inclusion body myositis (IBM) were registered on the database. The prevalence of hypertension and diabetes in this population was 62% and 29%, respectively, considerably higher than the background prevalence of 9.4% and 4%, making detection of treatment-related adverse effects difficult. Hypertension and ischemic heart disease were more likely to be present prior to the diagnosis of IIM rather than following it. Hypertension and diabetes occurred more frequently following the diagnosis of myositis, in patients with DM compared with PM or IBM., Conclusions: We report a novel association of IIM with hypertension, diabetes and ischemic heart disease, indicating that a comprehensive assessment of vascular risk factors is essential in IIM.

The idiopathic inflammatory myopathies are a group of systemic autoimmune syndromes characterized by striated muscle inflammation. Here, we discuss the clinical features of this group of conditions and review the recent developments in the understanding of the pathogenesis and immunogenetics of the idiopathic inflammatory myopathies. The role of myositis-specific autoantibodies and their clinical significance and an overview of management are also provided.

We present a case of antisynthetase syndrome manifesting with interstitial lung disease, mechanic's hands, nailfold abnormalities, and subclinical myositis, in the presence of antibodies to the aminoacyl tRNA synthetase PL-12 and also to Ro52. Antibodies to Ro52 have been recently associated with idiopathic inflammatory myositis, but there have only been occasional reports of this antibody occurring in association with aminoacyl tRNA synthetases, including PL-12. Our case adds to the descriptions of the concurrence of antibodies to PL-12 and Ro52. The mechanism for the coupling of antibody response remains elusive but is likely to play a fundamental role in disease pathogenesis.