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OBJECTIVE: Adult-onset Still's disease (AOSD) and secondary hemophagocytic lymphohistiocytosis (sHLH) are both hyperferritinemic cytokine storm syndromes that can be difficult to distinguish from each other in hospitalized patients. The objective of this study was to compare the inflammatory markers ferritin, D-dimer, C-reactive protein (CRP), and soluble CD25 (sCD25) in patients with AOSD and sHLH. These four markers were chosen as they are widely available and represent different aspects of inflammatory diseases: macrophage activation (ferritin); endothelialopathy (D-dimer); interleukin-1/interleukin-6/tumour necrosis factor elevation (CRP) and T cell activation (sCD25). METHODS: This was a single-center retrospective study. Patients diagnosed by the Hematology service at Vancouver General Hospital for AOSD or sHLH from 2009 to 2023 were included. RESULTS: There were 16 AOSD and 44 sHLH patients identified. Ferritin was lower in AOSD than HLH (median 11 360 µg/L vs. 29 020 µg/L, p = .01) while D-dimer was not significantly different (median 5310 mg/L FEU vs. 7000 mg/L FEU, p = .3). CRP was higher (median 168 mg/L vs. 71 mg/L, p <.01) and sCD25 was lower (median 2220 vs. 7280 U/mL, p = .004) in AOSD compared to HLH. The combined ROC curve using CRP >130 mg/L and sCD25< 3900 U/mL to distinguish AOSD from HLH had an area under the curve (AUC) of 0.94 (95% confidence interval 0.93-0.97) with sensitivity 91% and specificity 93%. CONCLUSIONS: These findings suggest that simple, widely available laboratory tests such as CRP and sCD25 can help clinicians distinguish AOSD from HLH in acutely ill adults with extreme hyperferritinemia. Larger studies examining a wider range of clinically available inflammatory biomarkers in a more diverse set of cytokine storm syndromes are warranted.
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OBJECTIVES: To evaluate the effectiveness and safety of upadacitinib in treatment-refractory inflammatory myositis. METHODS: Patients with refractory inflammatory myositis treated with upadacitinib from a single urban centre in Vancouver, British Columbia, Canada, were included from September 2020 to June 2023. The medical records of these patients were retrospectively reviewed. RESULTS: 10 total patients were identified for review, including 5 classic dermatomyositis (DM), 3 amyopathic DM (ADM) and 2 antisynthetase syndrome. The patients failed an average of four immunosuppressants before initiation of upadacitinib. Three had prior Janus kinase inhibitor therapy with tofacitinib. In the classic DM and ADM aggregate group, upadacitinib offered clinically and statistically significant cutaneous improvement. Lack of active muscle disease at baseline precluded analysis of the effect of upadacitinib on muscle weakness. Nine patients remained on upadacitinib at the end of the study period. One patient discontinued upadacitinib due to severe facial acne. CONCLUSION: Upadacitinib appears to be effective in targeting cutaneous manifestations of refractory inflammatory DM. Further research is still needed to validate its efficacy on a broader population scale.
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Dermatomiosite , Miosite , Humanos , Estudos Retrospectivos , Miosite/tratamento farmacológico , Miosite/etiologia , Compostos Heterocíclicos com 3 Anéis/uso terapêuticoRESUMO
OBJECTIVES: To evaluate the efficacy and safety of tofacitinib in treatment-refractory inflammatory myositis in a real-world clinical setting. METHODS: All patients with refractory inflammatory myositis treated with tofacitinib from a single urban center in Vancouver, British Columbia, Canada, were included from June 2016 to December 2022. The medical records of these patients were retrospectively reviewed. RESULTS: A total 41 patients were included, 23 with classic dermatomyositis (DM), 12 with amyopathic DM (ADM) and 6 with polymyositis (PM) phenotype. The patients failed an average of 4-5 non-steroidal immunosuppressants before initiation of tofacitinib. In the classic DM and ADM group, tofacitinib offered clinically and statistically significant cutaneous improvement. In all myositis patients including the PM phenotype, no meaningful muscle strength response to tofacitinib was observed. 53.7% of the patients discontinued tofacitinib due to lack of benefit or death. Of the 19 patients who remained on tofacitinib at the conclusion of this study, tofacitinib demonstrated clinically and statistically significant improvement in cutaneous disease activity. CONCLUSION: Tofacitinib appears to be highly effective in targeting cutaneous manifestations in classic DM and ADM; however, minimal benefit in muscle strength in the DM or PM phenotype were observed.
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INTRODUCTION: Telehealth has the potential to address health disparities experienced by Indigenous people, especially in remote areas. This scoping review aims to map and characterize the existing evidence on telehealth use by Indigenous people and explore the key concepts for effective use, cultural safety, and building therapeutic relationships. METHODS: A search for published and gray literature, written in English, and published between 2000 and 2022 was completed in 17 electronic databases. Two reviewers independently screened retrieved records for eligibility. For included articles, data were extracted, categorized, and analyzed. Synthesis of findings was performed narratively. RESULTS: A total of 321 studies were included. The most popular type of telehealth used was mHealth (44%), and the most common health focuses of the telehealth interventions were mental health (26%) and diabetes/diabetic retinopathy (13%). Frequently described barriers to effective telehealth use included concerns about privacy/confidentiality and limited internet availability; meanwhile, telehealth-usage facilitators included cultural relevance and community engagement. Although working in collaboration with Indigenous communities was the most frequently reported way to achieve cultural safety, 40% of the studies did not report Indigenous involvement. Finally, difficulty to establish trusting therapeutic relationships was a major concern raised about telehealth, and evidence suggests that having the first visit-in-person is a potential way to address this issue. CONCLUSION: This comprehensive review identified critical factors to guide the development of culturally-informed telehealth services to meet the needs of Indigenous people and to achieve equitable access and positive health outcomes.
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BACKGROUND: To evaluate provider perspectives on the development and implementation of an inpatient Addiction Medicine Consult Service, including their awareness of the service, its perceived role in the continuum of care, and changes over time in their perceptions of care quality for inpatients with substance use disorders. METHODS: Repeated cross-sectional survey of hospital-based physicians, nurses and social workers performed at service launch (April-June, 2017) and 4 years later (March-June, 2021). RESULTS: Providers had generally positive perceptions of the service and its impact on care quality, but encountered significant barriers at both time points in meeting patient needs (related to high patient complexity and difficulty connecting patients with community services post-discharge). Relative to physicians and social workers, nurses were less likely to be familiar with the service or see it as beneficial. CONCLUSIONS: Findings indicate that the service fills a gap that existed previously in the local system of care; however, numerous opportunities exist to further strengthen the system beyond the hospital setting to promote longer-term health among people who use substances. For nurses in particular, outreach, education, and other resources (e.g., dedicated nursing role support, nurse liaison) are warranted to ensure that nurses feel supported and confident caring for this patient population.