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The 73rd Annual Meeting of The Canadian Rheumatology Association was held at the Fairmont The Queen Elizabeth, Montreal, Quebec, Canada February 27 – March 2, 2019. The program consisted of presentations covering original research, symposia, awards, and lectures. Highlights of the meeting include the following 2019 Award Winners: Distinguished Rheumatologist, Edward Keystone; Distinguished Investigator, Diane Lacaille; Teacher-Educator, Shirley Tse; Emerging Investigator, Glen Hazlewood; Best Abstract on SLE Research by a Trainee – Ian Watson Award, Alexandra Legge; Best Abstract on Clinical or Epidemiology Research by a Trainee – Phil Rosen Award, Lauren King; Best Abstract on Basic Science Research by a Trainee, Remy Pollock; Best Abstract for Research by an Undergraduate Student, Andrea Carboni-Jimènez; Best Abstract on Research by a Rheumatology Resident, May Choi; Best Abstract by a Medical Student, Leonardo Calderon; Best Abstract by a Post-Graduate Research Trainee, Carolina Munoz-Grajales; Best Abstract by a Rheumatology Post-Graduate Research Trainee, Andre Luquini; Best Abstract on Quality Care Initiatives in Rheumatology, Cheryl Barnabe and Ines Colmegna; Best Abstract on Research by Young Faculty, Bindee Kuriya; Practice Reflection Award, Gold, Jason Kur; Practice Reflection Award, Silver, May Choi. Lectures and other events included Keynote Lecture by Andre Picard: Quirky Past, Uncertain Future: The State of Medicare in Canada; Keynote Address by Diane Lacaille, Distinguished Investigator Awardee: Time to Re-Label Comorbidities in RA – Coexisting or Complications; State of the Art Lecture by Mark Roberts: Myositis and its Mimics; Dunlop-Dottridge Lecture by Gilles Boire: The 4-H of Biomarkers in Arthritis: A lot of Help, Potential Harm, Some Hype, Increasing Hope; and the Great Debate: Be it Resolved that Competency-based Medical Education will Result in Improved Quality of Care for Patients vs the “Old Way” of Training Rheumatologists. Arguing for: Mercedes Chan and Marie-Paule Morin, and against: Beth Hazel and Heather McDonald-Blumer. Topics including rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, Sjögren syndrome, psoriatic arthritis, spondyloarthritis, vasculitis, osteoarthritis, fibromyalgia, and their respective diagnoses, treatments, and outcomes are reflected in the abstracts, which we are pleased to publish in this issue of The Journal.

Objective.Currently there are 2 different classification criteria for systemic lupus erythematosus (SLE): American College of Rheumatology (ACR) and Systemic Lupus International Collaborating Clinics (SLICC). The aim of this study was to compare the sensitivities of ACR and SLICC criteria in childhood-onset SLE (cSLE) using a large, multiethnic cohort.Methods.We conducted a retrospective study of 722 patients diagnosed with cSLE at The Hospital for Sick Children (SickKids). Prospectively collected data from SickKids’ Lupus Database were reviewed/validated against medical records prior to ACR and SLICC scoring based on cumulative symptoms up to the last visit. Sensitivities were compared using McNemar’s test. Descriptive statistics were used to identify SLE features unique to each set of criteria and autoantibodies not included in either.Results.ACR and SLICC sensitivities were as follows: 92.4% and 96.3% overall (p = 0.001); 82.5% and 91.3% (p = 0.01) in those scored ≤ 1 year from diagnosis; 92.7% and 97.9% (p = 0.02) in those scored 2–3 years from diagnosis. Forty-eight of 55 (87.3%) patients who did not meet ACR criteria met SLICC criteria through SLICC-specific criterion or renal biopsy. Twenty of 27 (74.1%) patients who did not meet SLICC criteria met ACR criteria as a result of photosensitivity (73.9%) and ACR lymphopenia criteria (26.1%). Six of 7 patients (85.7%) who were clinically diagnosed with cSLE but did not meet either SLICC or ACR criteria had anti-Ro antibodies.Conclusion.SLICC criteria were significantly more sensitive than ACR criteria in cSLE classification, especially early in the disease course. Because of the extreme rarity of primary Sjögren syndrome in children, one may consider adding anti-Ro antibodies to the classification criteria for cSLE because they are present in ∼40% of patents with cSLE.

ObjectiveTo evaluate the association between ethnicity and neonatal lupus erythematosus (NLE), as well as specific NLE manifestations in a large multiethnic population.MethodsWe conducted a cohort study of the children (≤ 1 yr of age) seen in the NLE clinic at The Hospital for Sick Children (SickKids), between January 2011 and April 2019. The cohort was divided into European, non-European, and mixed European–non-European groups according to parent-reported child’s ethnicity (Canada Census categories). Outcomes were NLE and specific NLE manifestations (cardiac, cutaneous, cytopenias, transaminitis, and macrocephaly). The frequency of NLE and specific manifestations were compared between ethnic groups (Fisher exact test). We tested the association between ethnicity and (1) NLE risk, and (2) specific NLE manifestations with logistic regression models, including covariates for child’s sex, maternal rheumatic disease status during pregnancy, and maternal use of antimalarials during pregnancy (multiple comparisons threshold P < 0.008).ResultsWe included 324 children born to 270 anti-Ro antibody–positive mothers. Median age at first visit was 1.8 (IQR 1.4–2.3) months, and median follow-up time was 12 (IQR 2–24) months. The majority was non-European (48%), with 34% European, and 18% mixed European–non-European. There was no significant association between non-European ethnicity (OR 1.18, 95% CI 0.71–1.94, P = 0.51), mixed European–non-European ethnicity (OR 1.13, 95% CI 0.59–2.16, P = 0.70), and NLE risk compared with European ethnicity. We also did not find an association between ethnicity and specific NLE manifestations in univariate or multivariable-adjusted models.ConclusionIn a large multiethnic cohort, there was no association between a child’s ethnicity and NLE risk or specific NLE manifestations.

ObjectiveMacrophage activation syndrome (MAS), a life-threatening inflammatory complication, is increasingly recognized in childhood-onset systemic lupus erythematosus (cSLE). It can be a challenge to differentiate active cSLE from MAS. We generated decision rules for discriminating MAS from active cSLE in newly diagnosed patients.MethodsWe conducted a retrospective cohort study of consecutive, newly diagnosed, active cSLE patients with fever, requiring hospital admission to The Hospital for Sick Children from January 2003 to December 2007 (cohort 1) and January 2008 to December 2013 (cohort 2). All patients met ≥ 4 American College of Rheumatology or Systemic Lupus International Collaborating Clinics criteria, and were steroid-naïve and infection-free. MAS was diagnosed based on expert opinion. Recursive partitioning was applied to each cohort to derive a decision rule based on clinical and laboratory features, distinguishing MAS from non-MAS cSLE. Each decision rule was applied to the alternate, independent cohort. Sensitivity and specificity of these decision rules were compared to existing criteria.ResultsCohort 1 (n = 34) and cohort 2 (n = 41) each had 10 patients with MAS. Recursive partitioning in cohort 1 identified ferritin ≥ 699 μg/L as the sole best discriminator between MAS and non-MAS patients (R2 = 0.48), and in cohort 2, ferritin ≥ 1107 μg/L was the best discriminator for MAS, followed by lymphocytes < 0.72 × 103/mm3 (R2 = 0.52). Cross-validation of our decision rules maintained 90–100% sensitivity and 65–85% specificity.ConclusionOur decision rule demonstrated improved performance compared to preliminary guidelines for MAS in cSLE from the Lupus Working Group of the Paediatric Rheumatology European Society and familial hemophagocytic lymphohistiocytosis diagnostic criteria. Validation in independent cohorts is required.

Objective.Persistent systemic lupus erythematosus (SLE) disease activity is associated with increased morbidity and mortality. In a multicenter cohort of patients with prevalent SLE, we described persistence, patterns, and predictors of change in disease activity over time.Methods.Based on SLE Disease Activity Index (SLEDAI)-2K scores at cohort entry, patients were classified into 4 groups: low (score < 4; LOW), moderate (4 to < 6; MOD), moderately high (6 to ≤ 10; MHIGH), and very high (> 10; VHIGH). Multivariable linear and longitudinal mixed linear regression models were used to identify predictors of change over time in SLEDAI-2K.Results.There were 2019 participants, with declining followup data over 5 years (1326, 580, 274, 186, and 148 patients, respectively). At cohort entry, mean (± SD) age was 42 (± 17) years, disease duration 11 (± 10) years, and 90% were female. The 4 groups included 44% LOW (n = 891), 20% MOD (n = 400), 22% MHIGH (n = 442), and 14% VHIGH (n = 286); therefore, 36% had clinically important SLE activity. The proportion of patients in the LOW group at entry who moved to a higher activity level varied from 30% (167/557) at 1 year, to 49% (41/83) at 3 years, and 54% (30/56) at 5 years. Among 181 patients with MOD to VHIGH entry activity and 3 years of followup, 116 (64.1%) remained active. In all analyses, only higher SLEDAI-2K at cohort entry remained a significant predictor of higher SLEDAI-2K in subsequent years.Conclusion.Higher SLEDAI-2K at study entry was the single major independent predictor of higher SLEDAI-2K over time, reflecting frequent persistence of active disease, even in patients with longstanding disease. This highlights gaps in the optimal treatment of SLE.

The 72nd Annual Meeting of The Canadian Rheumatology Association (CRA) was held at The Westin Ottawa, Ottawa, Ontario, Canada, February 8−11, 2017. The program consisted of presentations covering original research, symposia, awards, and lectures. Highlights of the meeting include the following 2017 award winners: Dr. Vinod Chandran, Young Investigator; Dr. Jacques P. Brown, Distinguished Investigator; Dr. David Robinson, Teacher-Educator; Dr. Michel Zummer, Distinguished Rheumatologist; Ms. Rebecca Gole, Best Abstract on SLE Research by a Trainee − Ian Watson Award; Ms. Bailey Russell, Best Abstract on Clinical or Epidemiology Research by a Trainee − Phil Rosen Award; Dr. Sahil Koppikar and Dr. Henry Averns, Practice Reflection Award; Dr. Shirine Usmani, Best Abstract on Basic Science Research by a Trainee; Ms. Carol Dou, Best Abstract for Research by an Undergraduate Student; Dr. Dania Basodan, Best Abstract on Research by a Rheumatology Resident; Dr. Claire Barber, Best Abstract on Adult Research by Young Faculty; Ms. Audrea Chen, Best Abstract by a Medical Student; Dr. Kun Huang, Best Abstract by a Post-Graduate Resident; and Dr. Ryan Lewinson, Best Abstract by a Post-Graduate Research Trainee. Lectures and other events included a Keynote Lecture by Jonathon Fowles: Exercise is Medicine: Is Exercise a Good or Bad Thing for People with Arthritis?; State of the Art Lecture by Matthew Warman: Insights into Bone Biology and Therapeutics Gleaned from the Sustained Investigation of Rare Diseases; Dunlop-Dottridge Lecture by Allen Steere: Lyme Disease: A New Problem for Rheumatologists in Canada; and the Great Debate: Be it Resolved that the Least Expensive Treatment Should be Chosen. Switch, Switch, Switch! Arguing for: Jonathan Chan and Antonio Avina, and against: Marinka Twilt and Glen Hazlewood. Topics such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, Sjogren syndrome, psoriatic arthritis, spondyloarthritis, vasculitis, osteoarthritis, fibromyalgia, pediatric rheumatology, and their respective diagnoses, treatments, and outcomes are reflected in the abstracts, which we are pleased to publish in this issue of The Journal.

The 73rd Annual Meeting of The Canadian Rheumatology Association was held at the Fairmont The Queen Elizabeth, Montreal, Quebec, Canada February 27 - March 2, 2019. The program consisted of presentations covering original research, symposia, awards, and lectures. Highlights of the meeting include the following 2019 Award Winners: Distinguished Rheumatologist, Edward Keystone; Distinguished Investigator, Diane Lacaille; Teacher-Educator, Shirley Tse; Emerging Investigator, Glen Hazlewood; Best Abstract on SLE Research by a Trainee - Ian Watson Award, Alexandra Legge; Best Abstract on Clinical or Epidemiology Research by a Trainee - Phil Rosen Award, Lauren King; Best Abstract on Basic Science Research by a Trainee, Remy Pollock; Best Abstract for Research by an Undergraduate Student, Andrea Carboni-Jimènez; Best Abstract on Research by a Rheumatology Resident, May Choi; Best Abstract by a Medical Student, Leonardo Calderon; Best Abstract by a Post-Graduate Research Trainee, Carolina Munoz-Grajales; Best Abstract by a Rheumatology Post-Graduate Research Trainee, Andre Luquini; Best Abstract on Quality Care Initiatives in Rheumatology, Cheryl Barnabe and Ines Colmegna; Best Abstract on Research by Young Faculty, Bindee Kuriya; Practice Reflection Award, Gold, Jason Kur; Practice Reflection Award, Silver, May Choi. Lectures and other events included Keynote Lecture by Andre Picard: Quirky Past, Uncertain Future: The State of Medicare in Canada; Keynote Address by Diane Lacaille, Distinguished Investigator Awardee: Time to Re-Label Comorbidities in RA - Coexisting or Complications; State of the Art Lecture by Mark Roberts: Myositis and its Mimics; Dunlop-Dottridge Lecture by Gilles Boire: The 4-H of Biomarkers in Arthritis: A lot of Help, Potential Harm, Some Hype, Increasing Hope; and the Great Debate: Be it Resolved that Competency-based Medical Education will Result in Improved Quality of Care for Patients vs the "Old Way" of Training Rheumatologists. Arguing for: Mercedes Chan and Marie-Paule Morin, and against: Beth Hazel and Heather McDonald-Blumer. Topics including rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, Sjögren syndrome, psoriatic arthritis, spondyloarthritis, vasculitis, osteoarthritis, fibromyalgia, and their respective diagnoses, treatments, and outcomes are reflected in the abstracts, which we are pleased to publish in this issue of

Objective.To determine cancer incidence in a large pediatric-onset systemic lupus erythematosus (SLE) population.Methods.Data were examined from 12 pediatric SLE registries in North America. Patients were linked to their regional cancer registries to detect cancers observed after cohort entry, defined as date first seen in the clinic. The expected number of malignancies was obtained by multiplying the person-years in the cohort (defined from cohort entry to end of followup) by the geographically matched age-, sex-, and calendar year–specific cancer rates. The standardized incidence ratio (SIR; ratio of cancers observed to expected) was generated, with 95% CI.Results.A total of 1168 patients were identified from the registries. The mean age at cohort entry was 13 years (SD 3.3), and 83.7% of the subjects were female. The mean duration of followup was 7.6 years, resulting in a total observation period of 8839 years spanning the calendar period 1974–2009. During followup, fourteen invasive cancers occurred (1.6 cancers per 1000 person-yrs, SIR 4.13, 95% CI 2.26–6.93). Three of these were hematologic (all lymphomas), resulting in an SIR for hematologic cancers of 4.68 (95% CI 0.96–13.67). SIR were increased for both male and female patients, and across age groups.Conclusion.Although cancer remains a relatively rare outcome in pediatric-onset SLE, our data do suggest an increase in cancer for patients followed an average of 7.6 years. About one-fifth of the cancers were hematologic. Longer followup, and study of drug effects and disease activity, is warranted.

Objective.To examine the frequency and risk factors for symptomatic avascular necrosis (AVN) in childhood-onset systemic lupus erythematosus (cSLE).Methods.A single-center, nested, matched, case-control design was used. There were 617 patients with cSLE followed at the Hospital for Sick Children (SickKids) Lupus Clinic between July 1982 and June 2013 included in the study. The AVN cohort consisted of 37 patients identified with clinical findings of symptomatic AVN and diagnosis was confirmed by 1 or more imaging modalities. Three controls were matched to each patient with AVN by date and age at diagnosis. Baseline clinical, laboratory, and treatment characteristics were compared between patients with AVN and controls by univariable analyses and if statistically significant, were included in a multivariable logistic regression model.Results.A total of 37/617 patients (6%) developed symptomatic AVN in 91 joints during followup at SickKids. The mean duration to disease was 2.3 years. The hip was the most commonly involved joint (26/37, 70%). Compared with the matched non-AVN cohort, patients with AVN had a higher incidence of central nervous system (CNS) involvement and nephritis, required greater cumulative prednisone (PRED) from cSLE diagnosis to AVN, received a greater maximal daily PRED dose, and had more frequent use of pulse methylprednisolone therapy. Multivariable regression analysis confirmed major organ involvement (CNS disease and/or nephritis) and maximal daily PRED dose as significant predictors of symptomatic AVN development.Conclusion.Patients with cSLE with severe organ involvement including nephritis and CNS disease and higher maximal daily dose of PRED are more likely to develop symptomatic AVN.

The 72nd Annual Meeting of The Canadian Rheumatology Association (CRA) was held at The Westin Ottawa, Ottawa, Ontario, Canada, February 8-11, 2017. The program consisted of presentations covering original research, symposia, awards, and lectures. Highlights of the meeting include the following 2017 award winners: Dr. Vinod Chandran, Young Investigator; Dr. Jacques P. Brown, Distinguished Investigator; Dr. David Robinson, Teacher-Educator; Dr. Michel Zummer, Distinguished Rheumatologist; Ms. Rebecca Gole, Best Abstract on SLE Research by a Trainee - Ian Watson Award; Ms. Bailey Russell, Best Abstract on Clinical or Epidemiology Research by a Trainee - Phil Rosen Award; Dr. Sahil Koppikar and Dr. Henry Averns, Practice Reflection Award; Dr. Shirine Usmani, Best Abstract on Basic Science Research by a Trainee; Ms. Carol Dou, Best Abstract for Research by an Undergraduate Student; Dr. Dania Basodan, Best Abstract on Research by a Rheumatology Resident; Dr. Claire Barber, Best Abstract on Adult Research by Young Faculty; Ms. Audrea Chen, Best Abstract by a Medical Student; Dr. Kun Huang, Best Abstract by a Post-Graduate Resident; and Dr. Ryan Lewinson, Best Abstract by a Post-Graduate Research Trainee. Lectures and other events included a Keynote Lecture by Jonathon Fowles: Exercise is Medicine: Is Exercise a Good or Bad Thing for People with Arthritis?; State of the Art Lecture by Matthew Warman: Insights into Bone Biology and Therapeutics Gleaned from the Sustained Investigation of Rare Diseases; Dunlop-Dottridge Lecture by Allen Steere: Lyme Disease: A New Problem for Rheumatologists in Canada; and the Great Debate: Be it Resolved that the Least Expensive Treatment Should be Chosen. Switch, Switch, Switch! Arguing for: Jonathan Chan and Antonio Avina, and against: Marinka Twilt and Glen Hazlewood. Topics such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, Sjögren syndrome, psoriatic arthritis, spondyloarthritis, vasculitis, osteoarthritis, fibromyalgia, pediatric rheumatology, and their respective diagnoses, treatments, and outcomes are reflected in the abstracts, which we are pleased to publish in this issue of

The medical cycle of discovery from researcher to editor to reviewer to publisher to researcher and back again — often re-engaging the same players in different roles — is a model for vetting and sharing medical information that inspired longtime Journal Editor Duncan Gordon1 (Figure 1). Figure 1. The medical discovery cycle; adapted with permission1. Little did anyone know at that time — not Duncan Gordon, the associate editors, reviewers, and authors — to what extent the discovery process would speed up and multiply itself, in particular with the emergence of electronic resource networks on the Internet. Today, electronic resources are apparent … Address reprint requests to Dr. E.D. Silverman; e-mail: jrheum{at}jrheum.com

The 71st Annual Meeting of The Canadian Rheumatology Association (CRA) was held at the Fairmont Chateau Lake Louise, Lake Louise, Alberta, Canada, February 17-20, 2016. The program consisted of presentations covering original research, symposia, awards, and lectures. Highlights of the meeting include the following 2016 Award Winners: Distinguished Rheumatologist, Ronald Laxer; Distinguished Investigator, Proton Rahman; Teacher-Educator, Lori Albert; Young Investigator, Nigil Haroon; Best Abstract on Basic Science Research by a Trainee, Liam O'Neil; Best Abstract on Research by a Rheumatology Resident, Valérie Leclair; Best Abstract by a Medical Student, Matthew Jessome; Best Abstract by a Post-Graduate Resident, Hyein Kim; CRA/Arthritis Research Foundation (ARF) Best Epidemiology/Health Services Research Award, Cheryl Barnabe; Summer Studentship Mentor Award, Ines Colmegna; CRA/ARF Best Paediatric Research Award, Lily Lim; CRA/ARF Best Clinical Research Award, Zahi Touma; CRA/ARF Best Basic Science Research Award, Nigil Haroon; Best Abstract on SLE Research by a Trainee - Ian Watson Award, Stephanie Nantes.

Objective.Pleuritis is a common manifestation and independent predictor of mortality in systemic lupus erythematosus (SLE). We examined the prevalence of pleuritis and factors associated with pleuritis in a multicenter Canadian SLE cohort.Methods.We studied consecutive adults satisfying the American College of Rheumatology (ACR) classification criteria for SLE who had a completed Systemic Lupus International Collaborating Clinics/ACR Damage Index (SDI) score, at least 1 evaluable extractable nuclear antigen assay, and either a SLE Disease Activity Index (SLEDAI) or a SLE Activity Measure score. Pleuritis was defined as having pleuritis by satisfying the ACR criteria or the SLEDAI. Factors related to pleuritis were examined using univariate and multivariate logistic regression.Results.In our cohort of 876 patients, 91% were women, 65% Caucasian, mean age (± SD) was 46.8 ± 13.5 years, and disease duration at study entry was 12.1 ± 9.9 years; the prevalence of pleuritis was 34% (n = 296). Notably, greater disease duration (p = 0.002), higher SDI score (p ≤ 0.0001), age at SLE diagnosis (p = 0.009), and anti-Sm (p = 0.002) and anti-RNP (p = 0.002) seropositivity were significantly associated with pleuritis. In multivariate analysis with adjustment for disease duration, age at diagnosis, and SDI score, concomitant seropositivity for RNP and Sm were related to a nearly 2-fold greater prevalence of pleuritis (OR 1.98, 95% CI 1.31–2.82).Conclusion.Pleuritis occurred in one-third of this Canadian cohort. Concomitant Sm and RNP seropositivity, greater cumulative damage, longer disease duration, and younger age at SLE disease onset were related to a higher rate of SLE pleural disease.

Objective.Prevalence and severity of systemic lupus erythematosus (SLE) in adults is suggested to be distinctly different between ethnic groups. The impact of ethnicity is not as well delineated in pediatric SLE (pSLE). We compared prevalence and extent of major organ involvement, disease activity, and damage in pSLE between different ethnic groups.Methods.Ethnic demographic profiles of an inception cohort of 265 patients with pSLE followed at Sick Kids Hospital in Toronto were determined and compared to the Metropolitan Toronto at-risk population. Patients were categorized into ethnic subsets based on self-designated ethnic origins. Disease characteristics including major organ involvement, disease activity, and damage measures were longitudinally determined and compared among ethnic groups.Results.Ethnicity data were available on 259/265 pSLE patients (99.6%); the majority were non-Caucasian (60%) compared to the Metropolitan Toronto at-risk population (40%) (p < 0.0001). Non-Caucasian patients were younger at diagnosis than Caucasian patients, Black patients being the youngest at diagnosis (12.6 vs 14.6 yrs; p = 0.007). Renal disease was significantly more common in non-Caucasian than in Caucasian pSLE patients (62% vs 45%; p = 0.01). There was a trend toward increased prevalence of central nervous system disease in Black patients compared to Asian patients (p = 0.108). There was no difference in gender ratio, SLE Disease Activity Index, or damage scores between ethnic groups.Conclusion.Non-Caucasian ethnicity is associated with increased pSLE disease prevalence. Non-Caucasian pSLE patients were significantly younger and more likely to have nephritis. However, disease activity and damage were strongly associated with major organ disease independent of the patient’s ethnicity.

Objective.Autoantibodies to the dense fine speckled 70 kDa antigen (DFS70) are reported to be more common in individuals who do not have an antinuclear antibody (ANA)-associated rheumatic disease (AARD) than in patients with AARD. The frequency of anti-DFS70 antibodies has been thoroughly studied in adult but not in pediatric populations. The primary objective of this observational study was to determine the frequency of anti-DFS70 in pediatric AARD and reference cohorts.Methods.Sera from 743 children with AARD and related conditions, and 345 samples from reference cohorts (healthy children and those being investigated for AARD) were studied for anti-DFS70 autoantibodies as measured by a chemiluminescence immunoassay. A de-identified administrative database was used to retrieve demographic, serologic, and clinical data.Results.Anti-DFS70 antibodies were seen in 2.1% of healthy children and in 4.5% of sera from pediatric individuals referred for ANA testing. The frequency of anti-DFS70 was highest in juvenile localized scleroderma (LS; 4/29, 13.8%), juvenile dermatomyositis (JDM; 2/11, 18.2%), childhood systemic lupus erythematosus (cSLE; 19/331, 5.7%), diffuse cutaneous systemic sclerosis (1/22, 4.5%), celiac disease (2/49, 4.1%), and juvenile idiopathic arthritis (JIA; 5/202, 2.5%). Of note, anti-DFS70 antibodies were observed in 3/26 children (11.5%) with uveitis and JIA-associated uveitis.Conclusion.The frequency of anti-DFS70 autoantibodies in healthy pediatric subjects is within the lower range of that reported in adults. Anti-DFS70 antibodies can be found in childhood SSc and cSLE, but has a remarkably high frequency in children with LS, JDM, and uveitis.

The 70th Annual Meeting of The Canadian Rheumatology Association (CRA) was held at the Fairmont Chateau Frontenac, Quebec City, Quebec, Canada, February 4-7, 2015. The program consisted of presentations covering original research, symposia, awards, and lectures. Highlights of the meeting include 2015 Award Winners: Distinguished Rheumatologist Award: Carter Thorne; Distinguished Investigator Award: Hani El-Gabalawy; Teacher-Educator Award: Andrew E. Thompson; Young Investigator Award: Sindhu Johnson; Summer Studentship Mentor Award: Lori Albert; Innovation in Education Award: Henry Averns; Best Abstract for Basic Science Research by a Trainee: Sina Rusta-Sallehy; Best Abstract for Research by an Undergraduate Student: Tristan Kerr; Best Abstract for Research by a Rheumatology Resident: Claire Barber; Best Poster by a Medical Student: Dennis Wong; Best Poster by a Post-Graduate Resident: Zainab Alabdurubalnabi; CRA/ARF Best Epidemiology/Health Services Research Award: Evelyn Vinet; CRA/ARF Best Clinical Research Award: Glen Hazelwood; CRA/ARF Best Basic Science Research Award: Carolina Landolt-Marticorena; Ian Watson Award for Best Abstract for SLE Research by a Trainee: Ripneet Puar; Phil Rosen Award for Best Abstract for Clinical or Epidemiology Research by a Trainee: Liam O'Neil.

Objective.To determine the most effective immunosuppressive therapy for the longterm management of proliferative lupus nephritis (PLN) based on the outcome of renal failure.Methods.A systematic review of randomized controlled trials (RCT) was conducted. MEDLINE and EMBASE were searched. RCT designed to examine the maintenance treatment effectiveness of immunosuppressive agents for PLN were included. A Bayesian network metaanalysis of 2-arm and 3-arm trials was used. A skeptical prior assumption was used in sensitivity analysis. Four immunosuppressive agents were evaluated: cyclophosphamide (CYC), azathioprine (AZA), mycophenolate mofetil (MMF), and prednisone alone. The outcome of interest was renal failure during the study period, defined by serum creatinine (sCr) > 256µmol/l, doubling of sCr from baseline, and/or endstage renal disease.Results.The OR (95% credible interval) of developing renal failure at 2–3 years was 0.72 (0.11, 4.49) for AZA versus CYC, 0.32 (0.04, 2.25) for MMF versus CYC, 2.40 (0.22, 36.94) for prednisone alone versus CYC, and 0.45 (0.11, 1.48) for MMF versus AZA. The probability (95% credible interval) of developing renal failure at 2 years as expected for each agent was 6% (0.7%, 24%) for MMF, 12% (2%, 37%) for AZA, 16% (5%, 33%) for CYC, and 31% (5%, 81%) for prednisone alone. After applying a skeptical prior in the Bayesian analysis, there was no evidence of benefit for 1 therapy over another.Conclusion.Although the data suggest that MMF may be superior to other treatments for the maintenance treatment of PLN, the evidence is not conclusive.

Objective.To evaluate and determine the most effective immunosuppressive therapy for the induction treatment of proliferative lupus nephritis (PLN) based on renal remission.Methods.A systematic review of randomized controlled trials was conducted. The outcomes were renal remission at 6 months: (1) normalization of serum creatinine [(sCr), or within 15% of the normal range, i.e., sCr < 132µmol/l — creatinine remission]; and (2) proteinuric remission (prU < 0.5 g/day/1.73m2). A Bayesian network metaanalysis was used.Results.The OR (95% credible interval) of inducing an sCr remission at 6 months was 1.70 (0.51, 6.87) for mycophenolate mofetil (MMF) versus cyclophosphamide (CYC); 2.16 (0.38, 13.36) for tacrolimus (Tac) versus CYC; and 1.25 (0.13, 10.51) for Tac versus MMF. For proteinuric remission the OR was 1.46 (0.81, 3.04) for MMF versus CYC; 1.96 (0.80, 5.11) for Tac versus CYC; and 1.34 (0.43, 3.90) for Tac versus MMF. The probability (95% credible interval) of inducing a creatinine remission at 6 months was Tac 56% (19%, 88%); MMF 51% (23%, 79%); and CYC 37% (28%, 47%). The probability of inducing a proteinuric remission was Tac 41% (23%, 63%); MMF 34% (23%, 50%); CYC 26% (20%, 32%); azathioprine 10% (1%, 55%); prednisone 11% (2%, 38%). None of the results were conclusive when examined in a sensitivity analysis.Conclusion.There is currently insufficient evidence to determine which of these immunosuppressive agents is superior. The probability of renal remission is 50% or lower at 6 months.

Earl D. Silverman, Editor-in-chief In the summer of 2011, Duncan Gordon first announced his plans to retire as The Journal ’s Editor-in-Chief, a position he had held since 1979. The news was received with mixed emotions: We all knew he needed more free time to enjoy other aspects of life, but it was also clear that the new editor was going to have very large shoes to fill. When I was asked to consider becoming editor-in-chief and when the position was ultimately offered to me, an important part of my decision to accept was based on Duncan’s ready availability as a mentor. Sadly for Duncan, as well as for all of us, he soon developed acute myelogenous leukemia. To watch such a great, ‘young,’ passionate man become ill and succumb to this terrible illness was truly sad. Despite the shortened mentorship and guidance time, I am grateful to have had the opportunity to observe how to balance the many aspects required to run a leading rheumatology journal. Those who know me, however, will be the first to confirm that subtlety, mediation, and conciliation are not my forte . Nevertheless, such characteristics are required of a journal editor, and certainly they were Duncan’s strong points. I hope that my short time as co-editor-in-chief with Duncan, as well as more than 20 years on the editorial committee, will have allowed me, if only by osmosis, to acquire some of Duncan’s virtues, both as a person and as a journal editor. Duncan showed true leadership in rheumatology, including disseminating new knowledge in the field, as he successfully guided The Journal from infancy and childhood to adulthood; he was also an outstanding human being. I am sure that all those whose lives he touched will remember him as fondly and for as long as …

Objective.(1) To describe the clinical course and response to treatment; and (2) to evaluate and compare damage accrual of distinct phenotypic subgroups of patients with clinically important psychiatric illness of pediatric systemic lupus erythematosus (pSLE).Methods.A single-center cohort study of patients with pSLE followed at a pediatric lupus clinic from 1985 to July 2009. Clinical course and response to treatment were studied. Remission was defined by absence of psychiatric/cognitive symptoms while receiving minimal doses of prednisone. Disease activity and damage were measured using SLE Disease Activity Index and SLE Damage Index.Results.Fifty-three children were included: 40 with psychosis and cognitive dysfunction (PSYC group) and 13 with isolated cognitive dysfunction (COG group). All received immunosuppressive treatment. Eighteen of 32 treated with azathioprine required a change to cyclophosphamide for poor response but none on cyclophosphamide required a change. The median times to remission were 72 weeks (PSYC) and 70 weeks (COG). Eight patients (7 PSYC, 1 COG) experienced flare following response/remission. New damage was noted in 50% of children at a median of 11 months: 57% of PSYC group, 31% of COG group. Persistent cognitive dysfunction was seen in 16% of PSYC patients and 15% of COG patients.Conclusion.Most patients responded to immunosuppressive treatment, although median time to remission was > 1 year. Roughly half the patients acquired a new damage item, most of which did not interfere with functional abilities. Fewer than 20% of patients developed neuropsychiatric damage. Both phenotypes of psychiatric pSLE responded equally well to current treatment.

This is my first editorial as the new editor of The Journal of Rheumatology . It is an honor to be only the third editor of one of the first journals in the field of Rheumatology. I am following the founding editor, Dr. Metro Ogryzlo, and the retiring editor, Dr. Duncan Gordon. It is also fitting that The Journal of Rheumatology , which has been a leader in rheumatology and a source of knowledge and investigation in all fields of this subspecialty, including pediatric rheumatology, should now have a pediatric rheumatologist as the editor. When it became apparent that The Journal was looking for a new editor, and when I considered applying for the position, I thought about how to build on the current strengths of The Journal and look to the future. This becomes particularly important in … Address correspondence to Dr. Silverman; E-mail: esilverman{at}jrheum.com; earl.silverman{at}sickkids.ca

Objective.To determine the spectrum of manifestations in clinically important (i.e., requiring alterations of immunosuppressive therapy) psychiatric illness of pediatric systemic lupus erythematosus (pSLE) and to describe the laboratory and imaging features associated with psychiatric illness of pSLE (psySLE).Methods.This was a single-center cohort study of patients with pSLE followed at a pediatric SLE clinic from August 1985 to July 2009. Patients with organic psychiatric disease due to SLE were included. Data regarding psychiatric features at initial presentation and during followup were obtained from psychiatry and rheumatology visits. Data regarding concomitant SLE disease activity and laboratory results were obtained from the institutional SLE database. Information from imaging studies was abstracted from patients' charts.Results.Our cohort consisted of 53 pediatric patients (87% female) diagnosed with psySLE, representing 12% of the total pSLE cohort of 447 in the same time period. The median age at diagnosis of pSLE was 15.0 years and 16.1 years for psySLE. All patients reported symptoms of cognitive dysfunction and 75% of patients had additional psychotic features. Insight was preserved in 64% of patients with psychosis at diagnosis of psySLE. Visual distortion was observed among 32% of children with psySLE. Eighty-two percent of patients demonstrated clinical response to the institutional protocol of immunosuppression.Conclusion.Cognitive dysfunction was present in all and additional psychosis present in 75% of pediatric patients with psySLE. Visual distortion and early preservation of insight were unique features of psychosis observed in this cohort of children/adolescents with psySLE.

Objective.To compare the clinical features and outcome between patients with juvenile psoriatic arthritis (JPsA) and non-JPsA juvenile idiopathic arthritis (JIA).Methods.Fifty-three children with JPsA, 32 with < 5 joints in the first 6 months of disease (oligo–JPsA) and 21 (≥ 5 joints) polyarticular-onset (poly-JPsA) were compared to 53 patients with JIA who were matched by sex, age, date of diagnosis, and articular onset pattern.Results.There was no difference in the percentage of patients between the oligoarticular groups who developed extended oligoarthritis or in the percentage of patients who were positive for antinuclear antibodies. The only differences were that 25% of patients with oligo-JPsA had dactylitis compared to 0% of patients with oligo-JIA (p < 0.01) and 50% had nail pitting as compared to 18.7% (p < 0.05). In polyarticular patients the percentages with dactylitis were similar (19% vs 38%; p = 0.25). The frequency of uveitis was identical in the oligoarticular patients but a higher rate was seen in poly-JPsA compared to poly-JIA (23.8% vs 0%; p = 0.02), while contractures were more frequent in poly-JIA compared to poly-JPsA during the course of the illness (47.6% vs 14.3%; p = 0.03) but not at last followup (14.3% vs 4.7%; p = 0.6). At last followup the mean Childhood Health Assessment Questionnaire scores were similar in both the polyarticular and oligoarticular groups.Conclusion.There were only a few differences between patients with JPsA and JIA regarding disease onset, disease course, and outcome. We suggest that large, longterm prospective studies are required to accurately determine whether subdividing JIA according to psoriasis is worthwhile.

Objective.(1) To evaluate the spectrum of serum autoantibodies in pediatric-onset systemic lupus erythematosus (pSLE) with a focus on ethnic differences; (2) using cluster analysis, to identify patients with similar autoantibody patterns and to determine their clinical associations.Methods.A single-center cohort study of all patients with newly diagnosed pSLE seen over an 8-year period was performed. Ethnicity, clinical, and serological data were prospectively collected from 156/169 patients (92%). The frequencies of 10 selected autoantibodies among ethnic groups were compared. Cluster analysis identified groups of patients with similar autoantibody profiles. Associations of these groups with clinical and laboratory features of pSLE were examined.Results.Among our 5 ethnic groups, there were differences only in the prevalence of anti-U1RNP and anti-Sm antibodies, which occurred more frequently in non-Caucasian patients (p < 0.0001, p < 0.01, respectively). Cluster analysis revealed 3 autoantibody clusters. Cluster 1 consisted of anti-dsDNA antibodies. Cluster 2 consisted of anti-dsDNA, antichromatin, antiribosomal P, anti-U1RNP, anti-Sm, anti-Ro and anti-La autoantibody. Cluster 3 consisted of anti-dsDNA, anti-RNP, and anti-Sm autoantibody. The highest proportion of Caucasians was in cluster 1 (p < 0.05), which was characterized by a mild disease with infrequent major organ involvement compared to cluster 2, which had the highest frequency of nephritis, renal failure, serositis, and hemolytic anemia, or cluster 3, which was characterized by frequent neuropsychiatric disease and nephritis.Conclusion.We observed ethnic differences in autoantibody profiles in pSLE. Autoantibodies tended to cluster together and these clusters were associated with different clinical courses.

Lipid abnormalities in patients with systemic lupus erythematosus (SLE) are common and likely are one of the causes of premature atherosclerosis in these patients. Our aims were to determine the frequency and pattern of dyslipoproteinemia at presentation of pediatric SLE; and to determine the association between dyslipoproteinemia and markers of disease activity and inflammatory markers at presentation of pediatric SLE.Serum lipid measurements were obtained at diagnosis before corticosteroid treatment for an inception cohort of 54 patients. Total cholesterol, triglyceride, LDL-C, and HDL-C levels were regressed on measures of inflammation, disease activity, and disease symptoms.At least one lipid abnormality was present in the majority of patients (63%), an elevated triglyceride level being the most common lipid abnormality (62%). Triglycerides were best predicted by fibrinogen, nephritis, and pleuritis (model R2 = 0.6). Albumin, C4, and white blood cell count were found to predict HDL-C (model R2 = 0.6). Erythrocyte sedimentation rate, central nervous system involvement, nasal ulcers, and nephritis were found as predictors for LDL-C:HDL-C (model R2 = 0.5). No significant predictors were found for total cholesterol or LDL-C. The European Consensus Lupus Activity Measure disease activity score best predicted abnormal triglyceride and HDL-C levels (OR 1.7, 95% CI 1.2-2.3).Children with newly diagnosed SLE exhibited the distinct pattern of dyslipoprotein of increased triglycerides and depressed HDL-C that was twice as common in the presence of kidney disease. This lipid profile puts them at risk for premature atherosclerosis. Good disease control and individualized use of lipid-lowering agents based on the observed pattern of lipid abnormalities may lower the risk of premature atherosclerosis in these patients.

Nasal septal perforation has been well recognized in patients with various rheumatic diseases. To our knowledge, this condition has not been reported in children with systemic juvenile idiopathic arthritis (SJIA) or patients with adult onset Still's disease (AOSD). We describe 3 patients with persistent SJIA/AOSD who developed nasal septal perforation during the course of their disease. As illustrated by these cases, nasal septal perforation may develop as a rare complication of SJIA/AOSD and can be considered as part of the clinical spectrum of the disease. In one case the nasal septal perforation was associated with vasculitis.

To determine whether the presence of hypothyroidism is associated with an increased incidence of neonatal lupus erythematosus (NLE) in infants of mothers with anti-Ro autoantibodies.The cohort consisted of 87 women with anti-Ro antibodies who delivered 102 infants. All infants had a full history and physical examination within 12 weeks of birth, electrocardiogram (echocardiogram when clinically indicated), complete blood cell count with differential, liver function tests, and autoantibody profile. Nine women had hypothyroidism and 78 had normal thyroid function.At least one manifestation of NLE was seen in 7/9 (78%) infants born to mothers with hypothyroidism and 45/78 (58%) infants born to mothers with normal thyroid function. Complete congenital heart block (CCHB) was seen in 5/9 (55%) in the hypothyroid group and 10/78 (13%) of the normal thyroid function group (p0.005). Mothers with hypothyroidism had a 9-fold increased risk over women with normal thyroid function of having a child with CCHB (odds ratio 8.63). Twenty-seven (31%) of the women were healthy: 4/9 of the hypothyroid and 23/78 of the normal thyroid function group. Of the 23 infants born to healthy mothers with normal thyroid function, 15/23 (65%) had NLE and 8/23 (35%) of these had CCHB.Women with hypothyroidism and anti-Ro antibodies were at increased risk for delivering a child with CCHB compared to women with antibodies alone, irrespective of maternal health. This study indicates that women with hypothyroidism should be tested for anti-Ro antibodies, as they may be at risk to deliver a child with CCHB.

Kawasaki disease (KD) has potentially serious cardiac complications including coronary artery aneurysms. Children who develop giant aneurysms (GA) are at increased risk of thrombosis and ischemia, and although longterm oral anticoagulation with warfarin is recommended, its efficacy has not been studied. We examined the longterm outcome of patients with GA secondary to KD, to determine if anticoagulation with warfarin aids in the prevention of myocardial ischemia.We studied patients with KD followed between May 1990 and April 2000.Thirty-nine GA occurred in 2.2% of patients with KD (22/997 patients), and 33 non-GA were also identified in these patients. Patients were divided into 2 groups, those taking warfarin and no warfarin. Most patients in both groups were also taking antiplatelet agents. The demographics of the 2 groups were statistically similar, except the median duration of followup was significantly longer for patients in the no-warfarin group (6.9 vs 13.3 yrs; p = 0.008). Four early ischemic events (1 year after KD diagnosis) occurred (3 myocardial infarctions and one stroke). Screening for late ischemic events by stress nuclear medicine myocardial perfusion imaging revealed only one patient, in the no-warfarin group, with reversible perfusion defects. No patient had clinical signs or symptoms of late myocardial ischemia. Echocardiographic regression of aneurysms was observed in both groups. In the warfarin vs no-warfarin group, the diameters of the GA regressed a median 22% vs 32% (p = 0.27), and non-GA regressed a median of 30% vs 25% (p = 0.61). Compliance with anticoagulation was good, and no major bleeding complication of anticoagulation occurred.Regression of GA occurred in most of our patients, and minimal late ischemia was observed. Further studies are required to evaluate the use of oral anticoagulation in patients with GA secondary to KD.

To evaluate the efficacy of eutectic lidocaine/prilocaine cream (EMLA) in reducing the pain associated with steroid joint injection in children with juvenile arthritis.A randomized, double blind, placebo controlled parallel group trial. Thirty-one children (ages 8-18 yrs) scheduled for steroid injection into a knee were randomized into groups having either 2.5 g lidocaine/prilocaine cream or placebo cream applied to the injection site 60-90 min before the procedure. Patients assessed the pain associated with initial needle insertion and subsequent steroid injection using a 10 cm visual analog scale.No significant difference was found in the pain reported after needle insertion or steroid injection between the lidocaine/prilocaine cream group (n = 17) and the placebo group (n = 14). There was a trend toward an association of lower median scores with the pain of steroid injection in the lidocaine/prilocaine group (6 mm) compared with the placebo group (22 mm).Application of 2.5 g lidocaine/prilocaine cream for 60-90 min had no statistically significant analgesic effect on pain associated with injections of steroids into the knees of children with juvenile arthritis.

Transient lesions involving the splenium of the corpus callosum — reversible splenial lesion syndrome (RESLES) or mild encephalitis/encephalopathy with a reversible isolated splenium of the corpus callosum lesion (MERS) — have been described in patients with encephalopathy of various etiologies but rarely in systemic lupus erythematosus (SLE), limited to a case series of 3 patients1,2,3 …