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Recent advances in Rosai-Dorfman-Destombes disease (RDD), notably molecular testing, targeted therapy, and PET-CT imaging, hold promise for better recognition and improved outcomes. This study presents patients diagnosed and treated in a "real world" setting, where navigating limited resources must be considered. This retrospective single-center review includes 15 adult patients diagnosed with RDD at Vancouver General Hospital between November 2015 and October 2023. The cohort comprised five males and ten females with a median age 53 years (range 19-80 years). All 15 patients had extra-nodal disease; 11 patients exclusively had extra-nodal disease, and four patients also had lymph node involvement. Seven patients had tissue next-generation sequencing, identifying MAP2K1 mutations in four cases and a KRAS p.K117N mutation in one case that was treated with targeted therapy using trametinib. PET-CT was used for disease staging in four cases. Six patients with refractory disease tolerated lenalidomide and dexamethasone without significant toxicity; three patients achieved complete response, and three had partial response. This study highlights RDD's diverse extra-nodal manifestations. Lenalidomide combined with dexamethasone is an effective and well-tolerated treatment option for select patients, especially those with refractory disease. Broad utilization of NGS and PET-CT can positively influence management decisions., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by ulcerative painful lesions with violaceous undermined borders. Up to 75% of PG cases develop in association with an underlying systemic disease. Monoclonal gammopathy is reportedly a concomitant condition with PG, with studies indicating immunoglobulin (Ig) A gammopathy as the most common. Whether gammopathy is associated with PG or is an incidental finding has been debated. We sought to investigate the association and characteristics of gammopathy in patients with PG. We retrospectively identified PG patients at our institution from 2010 to 2022 who were screened for plasma cell dyscrasia. Of 106 patients identified, 29 (27%) had a gammopathy; subtypes included IgA (41%), IgG (28%), and biclonal (IgA and IgG) (14%). Mean age was similar between those with and without gammopathy (60.7 vs. 55.9 years; P = .26). In addition, hematologic or solid organ cancer developed in significantly more patients with vs. without gammopathy (8/29 [28%] vs. 5/77 [6%]; P = .003). Among the subtypes of gammopathy, IgG monoclonal gammopathy had the highest proportion of patients with subsequent cancer development (4 of 8 patients, 50%). Study limitations include a retrospective, single-institution design with a limited number of patients. Overall, our data show a high prevalence of gammopathy in patients with PG; those patients additionally had an increased incidence of cancer, especially hematologic cancer., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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 centre 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 of 41 patients were included, 23 with classic DM, 12 with amyopathic DM (ADM) and six with PM phenotype. The patients failed an average of four to five 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., (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objective: Although patient outcomes in psoriatic arthritis (PsA) have improved with the advent of advanced therapies, there remains a high unmet need to treat residual disease activity. The objective of the current study was to quantify residual disease activity and burden of disease in Canadian patients with PsA., Methods: This was a multiregion, observational, retrospective analysis of patient data extracted from the Rhumadata and the International Psoriasis and Arthritis Research Team (IPART) registries, analyzing deidentified data from patients who had initiated advanced therapy for the treatment of PsA between January 2010 and December 2019. The primary endpoint was the proportion of patients failing to achieve minimal disease activity (MDA) within 6 months; secondary endpoints included clinical and patient-reported burden of disease. Descriptive statistics included summaries by region, treatment class, and number of prior advanced therapies., Results: One thousand five hundred ninety-six patients were included. The proportions of patients who failed to achieve MDA within 6 months of an advanced therapy were 64.8% in Ontario, 68.3% in Western Canada, 74.8% in Quebec, and 75% in the Atlantic/East region. Failure to achieve MDA was higher among patients receiving an IL-17i compared with a TNFi in all regions except the Atlantic/East. Between 73.2% and 78.6% of patients reported pain at 6 months, and continuing functional impairment varied from 24% in the West to 83.3% in the Atlantic/East., Conclusion: There is substantial burden and unmet need for improved therapies for Canadians with PsA. There is a wide regional variation in outcomes that requires further assessment., (Copyright © 2024 by the Journal of Rheumatology.)

Competing Interests: Conflicts of interest P.L. has received grants from the Lady Davis Institute for Medical Research, from the Jewish General Hospital Foundation and from the Jewish General Hospital Department of Medicine for this work. R.M. is the executive director of the Canadian Skin Patient Alliance, and receives funding from governments, individuals and corporations, including pharmaceutical companies. A full list of sponsors can be viewed here: https://www.canadianskin.ca/about-us/sponsors. A.P. is an inventor on two patents covering the use of peptidic transforming growth factor-β antagonists as antifibrotic agents.

Competing Interests: Conflicts of interest I.S. has received honoraria from Sanofi-Genzyme. R.M. is the executive director of the Canadian Skin Patient Alliance, which receives funding from governments, individuals and corporations, including pharmaceutical companies. A full list of sponsors can be viewed here: https://www.canadianskin.ca/about-us/sponsors. A.P. is an inventor on two patents covering the use of peptidic transforming growth factor-β antagonists as antifibrotic agents.

Competing Interests: Conflicts of interest R.M. is the executive director of the Canadian Skin Patient Alliance receiving funding from governments, individuals and corporations, including ­pharmaceutical ­companies. A full list of sponsors can be viewed here https://www.canadianskin.ca/about-us/sponsors. C.N. has received honoraria for advisory boards and talks by Merck, EMD Sorono, Novartis and Sanofi. A.B. is a coinventor on a patent covering the use of neddylation activating enzyme inhibitors as enhancers of RNA viruses. A.P. is an inventor on two patents covering the use of peptidic transforming growth factor-β antagonists as antifibrotic agents.

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., Competing Interests: Competing interests: JD received speaker’s honoraria from AbbVie, Amgen, Bausch, Leo, Janssen, Novartis, Sanofi and Pfizer; he participated a Data Safety Monitoring Board for Bristol Myers Squib and is on the advisory board for Solius and Boehringer Ingelheim. He performs clinical research with Corbus, Eli Lilly and company. MB and KH have no conflict of interest to declare., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Chilblain-like lesions (CLL) coinciding with Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) infection have been described in the literature. Available reviews of the literature suggest that CLL are associated with younger age, an equal sex ratio, negative testing for SARS-CoV-2, and mild to no extracutaneous manifestations (ECM) associated with COVID-19 infection. This systematic review aims to provide a summary of reports of CLL associated with the early SARS-CoV-2 pandemic in children to clarify the prevalence, clinical characteristics, and resolution outcomes of these skin findings. Sixty-nine studies, published between May 2020 and January 2022, met inclusion criteria and were summarized in this review, representing 1,119 cases of CLL. Available data showed a slight male predominance (591/1002, 59%). Mean age was 13 years, ranging from 0 to 18 years. Most cases had no ECM (682/978, 70%). Overall, 70/507 (14%) of patients tested positive for COVID-19 using PCR and/or serology. In the majority the clinical course was benign with 355/415 (86%) of cases resolving, and 97/269 (36%) resolving without any treatment. This comprehensive summary of pediatric CLL suggests these lesions are rarely associated with COVID-19 symptoms or test positivity.

Background: The Skin Investigation Network of Canada (SkIN Canada) is a new national skin research network. To shape the research landscape and ensure its value to patient care, research priorities that are important to patients, caregivers, and health care providers must be identified., Objectives: To identify the Top Ten research priorities for 9 key skin conditions., Methods: We first surveyed health care providers and researchers to select the top skin conditions for future research within the categories of inflammatory skin disease, skin cancers (other than melanoma), and wound healing. For those selected skin conditions, we conducted scoping reviews to identify previous priority setting exercises. We combined the results of those scoping reviews with a survey of patients, health care providers, and researchers to generate lists of knowledge gaps for each condition. We then surveyed patients and health care providers to create preliminary rankings to prioritize those knowledge gaps. Finally, we conducted workshops of patients and health care providers to create the final Top Ten lists of research priorities for each condition., Results: Overall, 538 patients, health care providers, and researchers participated in at least one survey or workshop. Psoriasis, atopic dermatitis and hidradenitis suppurativa (inflammatory skin disease); chronic wounds, burns and scars (wound healing); and basal cell, squamous cell and Merkel cell carcinoma (skin cancer) were selected as priority skin conditions. Top Ten lists of knowledge gaps for inflammatory skin conditions encompassed a range of issues relevant to patient care, including questions on pathogenesis, prevention, non-pharmacologic and pharmacologic management., Conclusions: Research priorities derived from patients and health care providers should be used to guide multidisciplinary research networks, funders, and policymakers in Canada and internationally.

Chilblain-like lesions (CLL) coinciding with SARS-CoV-2 infection have been described. Previous systematic reviews suggest CLL are associated with younger age, an equal sex ratio, negative testing for SARS-CoV-2, and mild to no extracutaneous symptoms. A systematic review was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines on CLL coinciding with SARS-CoV-2 to clarify the demographic characteristics, clinical features, and resolution outcomes of these skin findings. One hundred twenty-eight studies, published between March 2020 and January 2022, met inclusion criteria and were summarized in this review, representing 4,982 cases of CLL. Available data showed a slight female predominance (55%, n = 2,471 of 4,472). The mean age was 25 years, ranging from 0 to 95 years. Most cases were not associated with extracutaneous signs and symptoms (63%, n = 1,649 of 2,636). Overall, 19% (n = 347 of 1,838) of patients tested positive for SARS-CoV-2 using polymerase chain reaction, serology, or tissue biopsy. Clinical course was generally benign with 80% (n = 979 of 1,224) of cases resolving and 47% (n = 204 of 432) resolving without receiving treatment. This review provides a comprehensive summary of CLL associated with SARS-CoV-2. CLL occurred at a mean age of 25 years with a slight female predominance. The majority had negative COVID-19 testing, no extracutaneous signs and symptoms, and resolution without recurrence., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Extracorporeal photopheresis (ECP) is an immunomodulatory therapy that has been used for over 35 years to treat numerous conditions. ECP was initially approved by the US FDA in 1988 for the treatment of Sézary syndrome, a leukemic form of cutaneous T-cell lymphoma (CTCL). Although CTCL remains the only FDA-approved indication, ECP has since been used off-label for numerous other conditions, including graft-versus-host disease (GvHD), systemic sclerosis, autoimmune bullous dermatoses, Crohn's disease, and prevention of solid organ transplant rejection. In Canada, ECP is mainly used to treat CTCL, acute and chronic GvHD, and in some instances systemic sclerosis. Herein, we review the current concepts regarding ECP mechanism of action, treatment considerations and protocols, and efficacy., Competing Interests: Elena Netchiporouk has received grants, research support from Novartis, Sanofi, Sun Pharma, AbbVie, Biersdorf, Leo Pharma, Eli Lilly; speaker fees/ honoraria from Bausch Health, Novartis, Sun Pharma, Eli Lilly, Sanofi Genzyme, AbbVie, Galderma, Novartis, Sanofi Genzyme, Sun Pharma, Bausch Health and Leo Pharma and consulting fees from Bausch Health, Novartis, Sun Pharma, Eli Lilly, Sanofi Genzyme, AbbVie, Galderma, Novartis, Sanofi Genzyme, Sun Pharma, Bausch Health and Leo Pharma. Irina Turchin served as advisory board member, consultant, speaker and/or investigator for AbbVie, Amgen, Arcutis, Aristea, Bausch Health, Boehringer Ingelheim, Celgene, Eli Lilly, Galderma, Incyte, Janssen, Kiniksa, Leo Pharma, Mallinckrodt, Novartis, Pfizer, Sanofi, UCB. Wayne Gulliver received grants/research support from AbbVie, Amgen, Eli Lilly, Novartis and Pfizer; honoraria for advisory boards/invited talks from AbbVie, Actelion, Amgen, Arylide, Bausch Health, Boehringer, Celgene, Cipher, Eli Lilly, Galderma, Janssen, Leo Pharma, Merck, Novartis, PeerVoice, Pfizer, Sanofi-Genzyme, Tribute, UCB, Valeant and clinical trial (study fees) from AbbVie, Asana Biosciences, Astellas, Boehringer-Ingelheim, Celgene, Corrona/National Psoriasis Foundation, Devonian, Eli Lilly, Galapagos, Galderma, Janssen, Leo Pharma, Novartis, Pfizer, Regeneron, UCB. Gizelle Popradi has received honoraria or speaker fees from Jazz Pharma, Seattle Genetics, Abbvie, Kite Gilead, Pfizer, Taiho, Servier, Novartis, Merck, Kyowa Kirin, Abbvie, Avir Pharma, Mallinckrodt. Robert Gniadecki reports carrying out clinical trials for Bausch Health, AbbVie and Janssen and has received honoraria as consultant and/or speaker from AbbVie, Bausch Health, Eli Lilly, Janssen, Mallincrodt, Novartis, Kyowa Kirin, Sun Pharma and Sanofi. Charles Lynde was a consultant, speaker, and advisory board member for Amgen, Pfizer, AbbVie, Janssen, Novartis, Mallincrodt, and Celgene, and was an investigator for Amgen, Pfizer, AbbVie, Janssen, Lilly, Novartis, and Celgene. Ivan V. Litvinov received research grant funding from Novartis, Merck, AbbVie and Bristol Myers Squibb and honoraria from Janssen, Bausch Health, Galderma, Novartis, Pfizer, Sun Pharma, Johnson & Johnson and Actelion. Topics included in this article were based on, but not limited to, broad discussions at an advisory board meeting, which was sponsored and funded by Mallinckrodt, Inc. Consultancy fees were paid to meeting participants (EN, IT, WG, JD, MK, RG, CL and IVL). All other authors declare no existing competing interests., (Copyright 2022 by SkinCareGuide.com Ltd. Skin Therapy Letter© is published 6 times annually by SkinCareGuide.com Ltd, 1003 - 1166 Alberni Street, Vancouver, British Columbia, Canada, V6E 3Z3. All rights reserved. Reproduction in whole or in part by any process is strictly forbidden without prior consent of the publisher in writing.)

The design of good notation is a cause that was dear to Charles Babbage's heart throughout his career. He was convinced of the "immense power of signs" (1864, 364), both to rigorously express complex ideas and to facilitate the discovery of new ones. As a young man, he promoted the Leibnizian notation for the calculus in England, and later he developed a Mechanical Notation for designing his computational engines. In addition, he reflected on the principles that underlie the design of good mathematical notations. In this paper, we discuss these reflections, which can be found somewhat scattered in Babbage's writings, for the first time in a systematic way. Babbage's desiderata for mathematical notations are presented as ten guidelines pertinent to notational design and its application to both individual symbols and complex expressions. To illustrate the applicability of these guidelines in non-mathematical domains, some aspects of his Mechanical Notation are also discussed., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Introduction: An estimated 2-4% of Western populations are thought to have psoriasis, with a regional incidence ranging from 0.09% to 11.43%. Variance in estimates is a result of differences in study populations, methodology, regional differences, and definitions of disease. Reliable prevalence estimates of plaque psoriasis are challenging to establish. Further, the distribution of psoriasis severity in the population is unknown. This study aims to establish the utility of expert elicitation (EE) as a method for estimating unknown parameters in dermatology by (1) estimating the prevalence of psoriasis in the adult population, and (2) estimating previously unknown disease severity distribution., Methods: An expert panel of 11 Canadian dermatologists with demonstrated expertise in psoriasis was formed. A proof-of-concept EE exercise estimated psoriasis prevalence in the general population in Canada, followed by estimation of psoriasis disease severity distribution by body surface area (BSA). Expert estimates were consolidated using Bayesian methods to statistically model the data and represent uncertainty., Results: The median prevalence of psoriasis in the adult population using the Bayesian estimate was 3.0% (95% credibility interval, 2.7-3.3%), compared with the estimated mean prevalence of 3.4% (95% confidence interval, 2.2-4.9%). By EE, the estimated cumulative distribution of disease severity assessed by BSA suggests that approximately 50% of patients have a BSA of < 3% and 78% of patients have a BSA of < 10%, with only 2% having a BSA of > 50%., Conclusion: The EE approach resulted in prevalence estimates that had a narrow distribution and were consistent with published literature, supporting its value in dermatology as a complementary method to help guide decision-making in areas where evidence is scarce or uncertain.

Immune-mediated diseases and immunotherapeutics can negatively affect normal immune functioning and, consequently, vaccine safety and response. The COVID-19 pandemic has incited research aimed at developing a novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccine. As SARS-CoV-2 vaccines are developed and made available, the assessment of anticipated safety and efficacy in patients with immune-mediated dermatologic diseases and requiring immunosuppressive and/or immunomodulatory therapy is particularly important. A review of the literature was conducted by a multidisciplinary committee to provide guidance on the safety and efficacy of SARS-CoV-2 vaccination for dermatologists and other clinicians when prescribing immunotherapeutics. The vaccine platforms being used to develop SARS-CoV-2 vaccines are expected to be safe and potentially effective for dermatology patients on immunotherapeutics. Current guidelines for the vaccination of an immunocompromised host remain appropriate when considering future administration of SARS-CoV-2 vaccines., Competing Interests: Conflicts of interest None disclosed., (Copyright © 2021 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Objective: To conduct a systematic review and meta-analysis of the prevalence, incidence, and medication treatment of depression and anxiety among patients with psoriatic arthritis (PsA)., Methods: We searched Medline, Embase, Cochrane Database of Systematic Reviews, CINAHL and PsycINFO for full-length studies that: utilized an observational design; included patients with PsA with/without a comparator group; evaluated depression and/or anxiety as an outcome, comorbidity, or predictor of a health outcome; and reported relevant estimates. We pooled estimates using random effects models., Results: Of 683 titles identified in our search, 18 studies met inclusion criteria. The pooled prevalence proportion for depression based on 11 studies was 17% (95% confidence interval [CI], 13% to 21%). In addition, a meta-analysis of four studies comparing the prevalence of depression in patients with PsA to those without PsA yielded a pooled odds ratio (OR) of 1.68 (95% CI, 1.37 to 2.08). The incidence of depression in PsA patients compared to the general population yielded a pooled incidence rate of 21.27 (95% CI, 16.28 to 26.27) per 1,000 person-years and a pooled incidence rate ratio of 1.44 (95% CI, 1.20 to 1.73). The pooled prevalence proportion of anxiety based on seven studies was 19% (95% CI, 11% to 29%) and a meta-analysis of two studies comparing the prevalence of anxiety in patients with PsA to those without PsA yielded a pooled OR of 1.49 (95% CI, 1.39 to 1.59). Only a small proportion of patients, between 2.4% and 13.5%, were reported to be taking antidepressant or antianxiety medications., Conclusions: We identified substantial prevalence of depression and anxiety as well as elevated incidence of depression among patients with PsA. These findings should raise awareness of the importance of mental health care in this population., Competing Interests: Declaration of competing interest None., (Copyright © 2020 Elsevier Inc. All rights reserved.)

The fitness and recruitment of fish stocks can be markedly affected by environmental disturbances including global warming, eutrophication and contamination. Understanding the effects of environmental stressors on salmon physiology during marine residence is of a global concern as marine survival has decreased. We present a unique combination of physiological responses - antioxidant defence and oxidative damage biomarkers, stable isotopes and contaminant exposure biomarkers - measured from adult Atlantic salmon (Salmo salar) collected at the Baltic Sea and studied in relation to environmental variables and fitness estimates. The results demonstrate that feeding populations of salmon display marked temporal and spatial variation in oxidative status. Better oxidative status of salmon was characterized by a higher amount of reduced glutathione (GSH) and decreased lipid peroxidation (LPX), when the weight-at-age of 3-4-year old sprats was higher and contaminant exposure biomarker (EROD) was lower. Summer season conditions, which included cooler sea surface temperature (SST), higher bottom O 2 and less cyanobacteria also indicated conditions for better oxidative status. Summer SST was additionally shown to affected glutathione metabolism enzyme activities. Oxidative status was associated with stable isotopes δ 13 C and δ 15 N indicating indirect effect of abiotic conditions and lower levels of the food web. Differences in condition factor and growth were associated with oxidative status in one and two sea winter salmon, respectively. Wild salmon survival was higher in years when they had higher GSH and catalase activity and lower LPX. Enhanced glutathione metabolism and increased protein carbonyls were associated with higher occurrence of yolk-sac fry mortality (M74). Our results show that oxidative status can provide information on exposure to complex combinations of environmental conditions and stressors in the wild and provide a link of physiological function to individual and population level fitness effects., (Copyright © 2020. Published by Elsevier B.V.)

Background: The European League Against Rheumatism/American College of Rheumatology classification criteria for inflammatory myopathies are able to classify patients with skin-predominant dermatomyositis (DM). However, approximately 25% of patients with skin-predominant DM do not meet two of the three hallmark skin signs and fail to meet the criteria., Objectives: To develop a set of skin-focused classification criteria that will distinguish cutaneous DM from mimickers and allow a more inclusive definition of skin-predominant disease., Methods: An extensive literature review was done to generate items for the Delphi process. Items were grouped into categories of distribution, morphology, symptoms, antibodies, histology and contextual factors. Using REDCap™, participants rated these items in terms of appropriateness and distinguishing ability from mimickers. The relevance score ranged from 1 to 100, and the median score determined a rank-ordered list. A prespecified median score cut-off was decided by the steering committee and the participants. There was a pre-Delphi and two rounds of actual Delphi., Results: There were 50 participating dermatologists and rheumatologists from North America, South America, Europe and Asia. After a cut-off score of 70 during the first round, 37 of the initial 54 items were retained and carried over to the next round. The cut-off was raised to 80 during round two and a list of 25 items was generated., Conclusions: This project is a key step in the development of prospectively validated classification criteria that will create a more inclusive population of patients with DM for clinical research. What's already known about this topic? Proper classification of patients with skin-predominant dermatomyositis (DM) is indispensable in the appropriate conduct of clinical/translational research in the field. The only validated European League Against Rheumatism/American College of Rheumatology criteria for idiopathic inflammatory myopathies are able to classify skin-predominant DM. However, a quarter of amyopathic patients still fail the criteria and does not meet the disease classification. What does this study add? A list of 25 potential criteria divided into categories of distribution, morphology, symptomatology, pathology and contextual factors has been generated after several rounds of consensus exercise among experts in the field of DM. This Delphi project is a prerequisite to the development of a validated classification criteria set for skin-predominant DM., (Published 2019. This article is a U.S. Government work and is in the public domain in the USA.)

Increasing sea surface temperatures (SST) and blooms of lipid-poor, filamentous cyanobacteria can change mesozooplankton metabolism and foraging strategies in marine systems. Lipid shortage and imbalanced diet may challenge the build-up of energy pools of lipids and proteins, and access to essential fatty acids (FAs) and amino acids (AAs) by copepods. The impact of cyanobacterial blooms on individual energy pools was assessed for key species temperate Temora longicornis and boreal Pseudo-/Paracalanus spp. that dominated field mesozooplankton communities isolated by seasonal stratification in the central Baltic Sea during the hot and the cold summer. We looked at (a) total lipid and protein levels, (b) FA trophic markers and AA composition, and (c) compound-specific stable carbon isotopes (δ 13 C) in bulk mesozooplankton and in a subset of parameters in particulate organic matter. Despite lipid-poor cyanobacterial blooms, the key species were largely able to cover both energy pools, yet a tendency of lipid reduction was observed in surface animals. Omni- and carnivory feeding modes, FA trophic makers, and δ 13 C patterns in essential compounds emphasized that cyanobacterial FAs and AAs have been incorporated into mesozooplankton mainly via feeding on mixo- and heterotrophic (dino-) flagellates and detrital complexes during summer. Foraging for essential highly unsaturated FAs from (dino-) flagellates may have caused night migration of Pseudo-/Paracalanus spp. from the deep subhalocline waters into the upper waters. Only in the hot summer (SST>19.0°C) was T. longicornis submerged in the colder subthermocline water (~4°C). Thus, the continuous warming trend and simultaneous feeding can eventually lead to competition on the preferred diet by key copepod species below the thermocline in stratified systems. A comparison of δ 13 C patterns of essential AAs in surface mesozooplankton across sub-basins of low and high cyanobacterial biomasses revealed the potential of δ 13 C-AA isoscapes for studies of commercial fish feeding trails across the Baltic Sea food webs., Competing Interests: All authors declare no conflict of interest.

Background:: The treat-to-target (T2T) strategy has become established in several medical specialties as a key guidance to optimal therapeutic decision making. T2T may be effective in the assessment of the biologic class of agents called interleukin (IL)-17 inhibitors, which are emerging as a safe and effective treatment option for autoimmune inflammatory conditions such as plaque psoriasis, psoriatic arthritis (PsA), and ankylosing spondylitis (AS)., Objective:: The objective of this article is to use a T2T approach for the evaluation of the effectiveness and safety of IL-17 inhibitors in the management of patients with plaque psoriasis, PsA, and AS., Methods:: Following a comprehensive literature search, a full-day meeting was convened to discuss and identify the T2T targets for psoriasis, PsA, and AS. Clinical trial evidence was presented for the approved IL-17 inhibitors-secukinumab, ixekizumab, and brodalumab-to assess whether these data meet T2T safety and efficacy targets., Results:: All 3 approved agents were significantly superior to placebo and active controls in the achievement of T2T targets for psoriasis. Secukinumab and ixekizumab were likewise associated with significantly better outcomes than controls in the PsA targets, and secukinumab resulted in significant AS target improvements vs placebo. The IL-17 inhibitors were also associated with low rates of serious adverse events and exacerbations of common comorbid conditions., Conclusion:: Phase III trial results support the T2T benefit and safety of IL-17 inhibitors according to their specific indications for the management of patients with plaque psoriasis, PsA, and AS.

Objective: To develop recommendations for the assessment of people with systemic lupus erythematosus (SLE) in Canada., Methods: Recommendations were developed using the GRADE (Grading of Recommendations Assessment, Development, and Evaluation) approach. The Canadian SLE Working Group (panel of Canadian rheumatologists and a patient representative from Canadian Arthritis Patient Alliance) was created. Questions for recommendation development were identified based on the results of a previous survey of SLE practice patterns of members of the Canadian Rheumatology Association. Systematic literature reviews of randomized trials and observational studies were conducted. Evidence to Decision tables were prepared and presented to the panel at 2 face-to-face meetings and online., Results: There are 15 recommendations for assessing and monitoring SLE, with varying applicability to adult and pediatric patients. Three recommendations focus on diagnosis, disease activity, and damage assessment, suggesting the use of a validated disease activity score per visit and annual damage score. Strong recommendations were made for cardiovascular risk assessment and measuring anti-Ro and anti-La antibodies in the peripartum period and conditional recommendations for osteoporosis and osteonecrosis. Two conditional recommendations were made for peripartum assessments, 1 for cervical cancer screening and 2 for hepatitis B and C screening. A strong recommendation was made for annual influenza vaccination., Conclusion: These are considered the first guidelines using the GRADE method for the monitoring of SLE. Existing evidence is largely of low to moderate quality, resulting in more conditional than strong recommendations. Additional rigorous studies and special attention to pediatric SLE populations and patient preferences are needed.

Background: Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are mucocutaneous hypersensitivity reactions, usually to drugs or their metabolites. TEN is the most severe involving greater than 30% of the total body surface area (TBSA). Management of these patients usually benefits from a large multidisciplinary team for both wound and medical management. Treatment of these patients varies between centers and physicians and there is lack of a standardized treatment protocol in the medical literature., Objectives: To review the literature and complete a retrospective review of patients treated at Vancouver General Hospital over a 11-year period., Methods: A retrospective chart review of all patients diagnosed with SJS/TEN and treated at Vancouver General Hospital from 2001 to 2011 was completed. Data collected include patient demographics, time to transfer to a burn center, SCORTEN calculation, suspected cause of TEN, %TBSA involved, length of stay in hospital and ICU, medications, dressings, infections/cultures, fluids, mucosal involvement, teams involved, associated complications, morbidity and mortality. Data is reported quantitatively., Results: A total of 67 patients were identified (28 SJS, 21 SJS/TEN overlap, 18 TEN). In SJS/TEN overlap and TEN patients, oral mucosa and trunk were the primary sites involved. SCORTEN calculations were highest in the TEN group. Plastic surgery was consulted in 53% of TEN cases, 52% of SJS/TEN cases and 25% of SJS cases. Patients were admitted to a burn unit in 74% of TEN cases, 57% of TEN/SJS cases and 21% of SJS cases. Time from symptoms to diagnosis and transfer to a burn unit was highest for TEN patients. Time from presentation to diagnosis was highest in SJS/TEN overlap. Triggers were identified in 67-82% of cases. Treatment varied widely. Patients were treated conservatively, with steroids, IVIg, and cyclosporine alone or in combination. Observed mortality was higher than predicted by SCORTEN for patients treated with IVIg and lower for those treated with Cyclosporin. Dressings varied greatly and were often changed throughout a patients stay. Total mortality was 20.9% being the highest in the TEN group (35%)., Conclusions: SJS and TEN are a spectrum of severe mucocutaneous reactions that have unclear treatment recommendations within the literature and within our Level 1 hospital. Information gleaned from this research will help educate physicians involved in the treatment and management of patients with these diagnoses and has resulted in development of treatment guidelines in our hospital., (Copyright © 2017 Elsevier Ltd and ISBI. All rights reserved.)