Systematic review of rheumatic disease phenotypes and outcomes in the Indigenous populations of Canada, the USA, Australia and New Zealand.

We performed a systematic review designed to characterize clinical phenotypes and outcomes in Indigenous populations with rheumatic disease to enhance the understanding of how rheumatic disease presents in Indigenous populations and allow for better projection of the healthcare needs of the communities affected. A systematic search was performed in medical (Medline, EMBASE, CINAHL), Indigenous and conference abstract databases (to June 2015). Search terms for Indigenous populations were combined with terms for inflammatory arthritis conditions, connective tissue disorders, crystal arthritis and osteoarthritis. Studies were included if they reported on disease features, disease activity measures, or patient-reported outcomes in Canadian, American, Australian or New Zealand Indigenous populations. Data were extracted in duplicate, and a narrative summary was prepared. A total of 5269 titles and abstracts were reviewed, of which 504 underwent full-text review and 85 met inclusion criteria. Nearly all the studies described outcomes in the North American populations (n = 77), with only four studies from Australia and four studies from New Zealand. The majority of studies were in rheumatoid arthritis (n = 31) and systemic lupus erythematosus (n = 19). Indigenous patients with rheumatoid arthritis had higher disease activity and reported more significant impact on patient-reported outcomes and quality of life than non-Indigenous patients. Spondyloarthropathy features were described in North American populations, with most patients having advanced manifestations. In systemic lupus erythematosus, nephritis was more frequent in Indigenous populations. Gout and osteoarthritis were more severe in New Zealand Maori populations. The existing literature supports differences in disease phenotype and severity in Indigenous populations of Canada, America, Australia and New Zealand. We encourage investigators in this area of research to undertake contemporary studies that disentangle differences between phenotype and severity that are biologic in etiology or merely reflecting differences in access to care and that provide a longitudinal assessment of outcomes in more diverse populations.

Hypercoagulability and Inflammatory Markers in a Case of Congenital Thrombotic Thrombocytopenic Purpura Complicated by Fetal Demise.

BACKGROUND: Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare disorder caused by an inherited genetic deficiency of ADAMTS13 and affects less than one per million individuals. Patients who are diagnosed with TTP during pregnancy are at increased risk of maternal and fetal complications including fetal demise. We present a case of a 32-year-old G3P0 (gravida 3, para 0) who presented at 20 weeks gestation with a new diagnosis of congenital TTP (cTTP) and fetal demise. METHODS: We describe the pathophysiology of pregnancy complications in a patient with cTTP using platelet procoagulant membrane dynamics analysis and quantitative proteomic studies, compared to four pregnant patients with gestational hypertension, four pregnant patients with preeclampsia, and four healthy pregnant controls. RESULTS: The cTTP patient had increased P-selectin, tissue factor expression, annexin-V binding on platelets and neutrophils, and localized thrombin generation, suggestive of hypercoagulability. Among 15 proteins that were upregulated, S100A8 and S100A9 were distinctly overexpressed. CONCLUSIONS: There is platelet-neutrophil activation and interaction, platelet hypercoagulability, and proinflammation in our case of cTTP with fetal demise.

Effectiveness of simulation-based training for obstetric internal medicine: Impact of cognitive load and emotions on knowledge acquisition and retention.

BACKGROUND: Simulation-based training's impact on learning outcomes may be related to cognitive load or emotions during training. We evaluated the association of validated measures of cognitive load and emotion with learning outcomes in simulation-based obstetric internal medicine cases. METHODS: All internal medicine learners (n = 15) who completed the knowledge test pre-training, post-training (knowledge acquisition), and at 3-6 months (knowledge retention) for all three simulation cases were included. RESULTS: Mean knowledge scores differed over time in all three cases (p < 0.0001 for all). Knowledge retention scores were significantly higher only for cases 1 and 3. Cognitive load associated with frustration was positively associated with knowledge acquisition for case 2 (beta = 5.18, P = 0.007), while excitement was negatively associated with knowledge retention in case 1 (beta = -33.07, p = 0.04). CONCLUSION: Simulation-based education for obstetric internal medicine can be effective in select cases. Attention to cognitive load and emotion may optimize learning outcomes.

Characterization of indigenous community engagement in arthritis studies conducted in Canada, United States of America, Australia and New Zealand.

BACKGROUND: Research adhering to community engagement processes leads to improved outcomes. The level of Indigenous communities' engagement in rheumatology research is unknown. OBJECTIVE: To characterize the frequency and level of community engagement reporting in arthritis studies conducted in Australia (AUS), Canada (CAN), New Zealand (NZ) and the United States of America (USA). METHODS: Studies identified through systematic reviews on topics of arthritis epidemiology, disease phenotypes and outcomes, health service utilization and mortality in Indigenous populations of AUS, CAN, NZ and USA, were evaluated for their descriptions of community engagement. The level of community engagement during inception, data collection and results interpretation/dissemination stages of research was evaluated using a custom-made instrument, which ranked studies along the community engagement spectrum (i.e. inform-consult-involve-collaborate-empower). Meaningful community engagement was defined as involving, collaborating or empowering communities. Descriptive analyses for community engagement were performed and secondary non-parametric inferential analyses were conducted to evaluate the possible associations between year of publication, origin of the research idea, publication type and region of study; and meaningful community engagement. RESULTS: Only 34% (n = 69) of the 205 studies identified reported community engagement at ≥ 1 stage of research. Nearly all studies that engaged communities (99% (n = 68)) did so during data collection, while only 10% (n = 7) did so at the inception of research and 16% (n = 11) described community engagement at the results' interpretation/dissemination stage. Most studies provided community engagement descriptions that were assessed to be at the lower end of the spectrum. At the inception of research stage, 3 studies reported consulting communities, while 42 studies reported community consultation at data collection stage and 4 studies reported informing or consulting communities at the interpretation/dissemination of results stage. Only 4 studies described meaningful community engagement through all stages of the research. Inferential statistics identified that studies with research ideas that originated from the Indigenous communities involved were significantly more associated with achieving meaningful community engagement. CONCLUSIONS: The reporting of Indigenous community engagement in published arthritis studies is limited in frequency and is most frequently described at the lower end of the community engagement spectrum. Processes that support meaningful community engagement are to be promoted.

Mortality causes and outcomes in Indigenous populations of Canada, the United States, and Australia with rheumatic disease: A systematic review.

BACKGROUND: Indigenous populations of Canada, America, Australia, and New Zealand have increased rates and severity of rheumatic disease. Our objective was to summarize mortality outcomes and explore disease and social factors related to mortality. METHODS: A systematic search was performed in medical (Medline, EMBASE, and CINAHL), Indigenous and conference abstract databases (to June 2015) combining search terms for Indigenous populations and rheumatic diseases. Studies were included if they reported measures of mortality (crude frequency, mortality rate, survival, and potential years of life lost (PYLL)) in Indigenous populations from the four countries. RESULTS: Of 5269 titles and abstracts identified, 504 underwent full-text review and 12 were included. No studies from New Zealand were found. In five Canadian studies of systemic lupus erythematosus (SLE) patients, First Nations ethnicity was associated with lower survival after adjusting for disease and social factors, and an increased frequency of death from lupus and its complications compared to Caucasians was found. All-cause mortality was higher in Native Americans (n = 2 studies) relative to Whites with SLE after adjusting for disease and social factors, but not in those with lupus nephritis alone. Australian Aborigines with SLE frequently developed infection and lupus complications leading to death (n = 3 studies). Mortality rates were increased in Pima Indians in the United States with rheumatoid arthritis (RA) compared to those without RA. One study in Native Americans with scleroderma found nearly all deaths were related to progressive disease. CONCLUSIONS: Canadian and American Indigenous populations with SLE have increased mortality rates compared to Caucasian populations. Mortality in Canadian and Australian Indigenous populations with SLE, and in Native American populations with RA and scleroderma, is frequently attributed to disease progression or complications. The proportional attribution of rheumatic disease severity and social factors to mortality and complications leading to death between Indigenous and non-Indigenous populations has not been fully evaluated.

Systematic review of rheumatic disease epidemiology in the indigenous populations of Canada, the United States, Australia, and New Zealand.

OBJECTIVE: Past publications have highlighted an excess rheumatic disease incidence and prevalence in indigenous populations of Canada (First Nations, Inuit, and Métis), and the United States of America (Alaska Native and American Indian). We have updated these reviews and expanded the scope to include New Zealand (Maori) and Australia (Aborigine) indigenous populations. METHODS: We performed a broad search using medical literature databases, indigenous specific online indexes, and government websites to identify publications reporting the incidence and/or prevalence of arthritis conditions (rheumatoid arthritis, spondyloarthropathies, gout, osteoarthritis, systemic autoimmune rheumatic diseases, and juvenile idiopathic arthritis) in the indigenous populations of Canada, America, New Zealand, and Australia. A narrative synthesis by type of arthritis was prepared given the heterogeneity of study designs used in the primary studies. RESULTS: Of 5269 titles and abstracts, 88 met inclusion criteria. Osteoarthritis was found to affect up to 17% of American Indian/Alaska Native women, 22% of Canadian First Nations, 32% of Australian Aborigine, and 6% of New Zealand Maori populations. The prevalence of rheumatoid arthritis, systemic lupus erythematosus, and juvenile idiopathic arthritis was consistently significantly higher in indigenous populations. Several studies describing the prevalence of spondyloarthropathy in North American northern populations were identified, but with no comparison populations the relative frequency could not be commented on. Gout was more prevalent in Maori compared to general population New Zealanders. CONCLUSIONS: This comprehensive summary describes rheumatic disease burden in indigenous populations in four countries with similar disparities in social determinants of health, to inform clinical service requirements to meet population need.

Healthcare utilization for arthritis by indigenous populations of Australia, Canada, New Zealand, and the United States: A systematic review☆.

PURPOSE: Indigenous populations of Australia, Canada, New Zealand, and the United States of America (USA) experience a higher prevalence of arthritis conditions. Differences in clinical outcomes and mortality may reflect healthcare service use inequities. The objective of this study was to summarize healthcare service use patterns described in the existing literature in order to identify gaps and inform strategies to limit the pronounced negative impact of arthritis on Indigenous populations. METHODS: Medline, EMBASE, CINAHL, and Indigenous-specific electronic databases (to June 2015) were used to identify cohort, case-control and cross-sectional studies describing healthcare service use by Indigenous populations with specified inflammatory arthritis, osteoarthritis, or rheumatic disease conditions. We extracted information on the study setting and methodology, primary outcome and assessed study quality, and risk of bias. RESULTS: In total, 19 studies were identified describing three types of healthcare service use: physician visits, hospitalizations, and surgeries. In Canada and New Zealand, Indigenous populations had 36-51% fewer visits to specialists than the non-Indigenous population. Indigenous populations in Canada, New Zealand, and the USA had 37-300% more hospitalizations due to arthritis complications than the non-Indigenous population. Indigenous populations in Australia, Canada, and New Zealand had 27-85% fewer arthroplasties for osteoarthritis than the non-Indigenous population. CONCLUSIONS: Indigenous populations had higher hospitalization rates but lower use of specialized services for arthritis conditions. Strategies to improve access to specialized arthritis services might reduce health outcome inequities.