bluuebunny/crossref_metadata_2025_split huggingface dataset:
A64: Patient‐Reported Side Effects with Weekly Injections of Methotrexate in Tertiary Care Rheumatology Clinic
Authors: Bechard Melanie , Lemieux Julie , Roth Johannes , Watanabe Duffy Karen , Duffy Ciaran M. , Aglipay Mary , Barrowman Nick , Jurencak Roman 
Published: March 2014

Background/Purpose:Methotrexate is frequently used to treat a variety of paediatric rheumatologic conditions. We explored patient‐reported frequency of side effects and coping strategies for weekly injections of subcutaneous methotrexate.Methods:A prospective observational cohort study was conducted from June 2013 through August 2013. All patients with clinic appointments in rheumatology during this period were screened for eligibility. Patients aged 4–17 years who were currently receiving weekly subcutaneous methotrexate injections for at least 4 weeks were invited to participate in the study. A focused interview with the patient and family members, conducted by one interviewer, investigated their experiences with subcutaneous methotrexate. The interview consisted of 18 questions and required approximately 10 minutes to complete.Results:42 patients met inclusion criteria and 41 consented to participate in the study. The mean age was 11.2 years (SD = 3.9 yrs) and 68% of participants were female. Most of these patients were diagnosed with juvenile idiopathic arthritis (73%). Mean duration of therapy with subcutaneous injections of methotrexate was 2.5 years (SD = 2.1 yrs) and the dosing range was 0.3\N1 mg/kg/week (maximum 25 mg/week). Almost all patients (95%) reported excellent adherence to the treatment regimen, missing less than one dose per month. The two most commonly reported side effects were nausea and vomiting (56% and 34% of patients, respectively). Less frequently reported side effects included fatigue (29%), anorexia (24%), headache (15%), and recurrent oral ulcers (10%). Of patients who experienced nausea, 74% used dimenhydrinate while 48% used ondansetron to manage their symptoms. Similarly, many patients who experienced vomiting tried to alleviate their symptoms with dimenhydrinate (64%) or ondansetron (50%). While dimenhydrinate effectively treated nausea in only 42% of patients, most patients reported good efficacy of ondansetron (83%). A similar effect was observed for treatment of vomiting (efficacy of dimenhydrinate 12% versus ondansetron 75%). None of the patients tried ginger to alleviate nausea/vomiting. Except for oral ulcers, clinical side effects resolved within 24 hours in 88% of patients. Of patients who had used oral methotrexate in the past, only 26% felt that the oral form was better tolerated.Conclusion:More than half of the patients reported at least one side effect that could be attributed to methotrexate injections; the most frequent ones were nausea and vomiting. Importantly, most side effects resolved within 24hours and patients reported excellent compliance. In general, patients did not have a preference for oral methotrexate.
A71: Localized Myositis as a Manifestation of Systemic‐Onset Juvenile Idiopathic Arthritis: A Case Series From a Tertiary‐Care Hospital
Authors: Kitcharoensakkul Maleewan , White Andrew J. , French Anthony R. , Cooper Megan A. , Baszis Kevin W. 
Published: March 2014

Background/Purpose:Localized myositis is rarely reported in patients with systemic‐onset JIA (sJIA), despite common symptoms of myalgia. We describe sJIA patients with the manifestation of localized myositis.Methods:We reviewed the charts of 6 pediatric patients with sJIA who developed localized myositis during their disease course.Results:The age at presentation with myositis ranged from 5 to 19 years. 4 were males and 2 were females. Races included Caucasian (4), Caucasian/Mayan (1), and African‐American (1). Three patients had localized myositis with initial presentation of sJIA, and 3 patients developed localized myositis during disease flares. For the disease flare group, the time from diagnosis of sJIA until development of myositis ranged from 3 to 6 years, and 2 of 3 patients were on immunosuppressive agents: methotrexate (MTX) (1), anakinra and MTX (1). All 6 patients complained of localized swelling and pain in a localized muscle group. Accompanying symptoms included arthralgia, arthritis, fever and rash. Three patients had localized erythema of the skin overlying myositis areas on exam. Sites of myositis included unilateral biceps (3), bilateral biceps (1), quadriceps (1), and flexor pollicis longus and flexor digitorum superficialis and profundus (1). All patients had at least one muscle enzyme obtained, and all were normal. Five patients had imaging studies demonstrating evidence of myositis: 2 by ultrasound, 2 by ultrasound and MRI, and 1 by MRI. All 6 patients had laboratory evidence of systemic inflammation, including elevated ESR (41–172 mm/hr) and CRP (54–269 mg/L). Four patients had thrombocytosis (462,000–834,000/μL). Two patients transferred care to another hospital, thus we had no information on follow‐up. The remaining 4 patients responded well to prednisone, with resolution of their symptoms within 4 weeks. One patient underwent incision and drainage for a fluid collection and myositis of biceps muscle. He was initially treated with antibiotics and started on anakinra after negative cultures. Duration of follow‐up since onset of myositis was 6–14 months, and no patients had recurrent myositis. The current treatment of the 4 patients includes infliximab (1), MTX (1), canakinumab and leflunomide (1), and canakinumab and MTX (1).Conclusion:Localized myositis is a rare extraarticular manifestation of sJIA and can present either as an initial manifestation or during a disease flare. Patients usually present with unilateral or bilateral localized swelling, overlying warmth and erythema, and pain of one muscle group, most commonly the biceps in our series of patients. Our subjects' muscle enzymes were normal, likely secondary to the focal nature of muscle inflammation. Imaging studies are helpful in establishing the diagnosis. To our knowledge, this study represents the largest series to date of sJIA patients with localized myositis.
A79: Pulmonary Hemorrhage in Pediatric Systemic Lupus Erythematous: Clinical Course and Outcomes
Authors: Singla Saimun , Muscal Eyal , Canter Debra , DeGuzman Marietta , Vece Timothy 
Published: March 2014

Background/Purpose:Pulmonary hemorrhage (PH) is a rare and devastating manifestation of SLE, occurring in 1–5% of adult cohorts with mortality rates ranging from 70%‐90%. The majority of PH reported in SLE refers to the adult population, and there is a paucity of data in pediatrics. Therefore, we describe our experience with PH in pediatric SLE, outlining disease presentation, treatment, and outcomes.Methods:We reviewed records of pediatric SLE patients who presented with pulmonary involvement diagnosed between January 2000 and August 2010. Inclusion criteria included > 4 SLE classification criteria and PH based on pulmonary evaluation by BAL, lung biopsy and/or characteristic imaging with worsening anemia. We describe our pediatric cohort, presenting symptoms, diagnostic modalities, various interventions and overall outcomes.Results:Seven of 401 (1.7%) SLE patients met inclusion criteria for PH. Patients were predominantly male (71.4%) and Hispanic (57.1%). Median age of PH presentation was 13.5 years (range 2.0–15.2). Episodes were primarily within the first 3 months of SLE diagnosis (71.4%) and were a presenting manifestation for SLE in 28.6% of our patients. All patients presented with a new cough, dyspnea, hypoxemia, anemia and abnormal CXR. Hemoptysis was present in 28.6%. Four out of 6 children had chest CT findings with diffuse ground glass opacities (66.6%) and 3 with nodules (50%). Additional non‐pulmonary features included fever (71.4%), renal disease (57.1%), cytopenias (71.4%), cardiac disease (50.0%), and positive antiphospholipid antibodies (85.7%). Eighty‐five percent required supplemental oxygen and 57.1% required mechanical ventilation. Hemosiderin‐laden macrophages without infection were observed in 3 BAL's. Out of the two lung biopsies performed, both revealed diffuse hemorrhage and one demonstrated immunoglobulin and complement deposition. All were treated with pulse doses of methylprednisolone infusions. Other therapeutic interventions included cyclophosphamide (42.8%), plasma exchange (42.8%), rituximab (85.7%) and IVIG (14.2%). Survival rate was 87.5%. All patients were maintained on corticosteroids and 66.6% received monthly cyclophosphamide infusions. Subsequent outpatient therapies included IVIG (33.3%), azathioprine (16.6%) or rituximab (16.6%). No patients had recurrences of PH. No survivors required supplemental oxygen nor developed interstitial lung disease (median follow‐up 2.6 years, range 1.5–5.0).Conclusion:In our review of pSLE patients with PH, the prevalence of this complication approximated that of adult SLE cohorts. However, in contrast to the adult population, PH presented earlier in the course of SLE in children. Additionally, outcomes were favorable with initiation of aggressive, multi‐modal immunosuppressive therapy with no recurrences. The mortality rate was much lower than described in adult cohorts.
A90: Pediatric Henoch‐NSchöAdonlein Purpura: Can Length of Hospital Stay Be Predicted?
Authors: Cohen Neta , Mimouni Francis , Freidel Nadav , Amarilyo Gil 
Published: March 2014

Background/Purpose:Henoch Schonlein purpura (HSP) is a common, usually benign, arthritis in childhood. However, at times, it follows a protracted course involving prolonged length of hospital stay (LOS).Objective:To determine clinical and laboratory characteristics that allow prediction of prolonged LOS in children with HSP.Methods:We retrospectively collected all consecutive cases of HSP that were admitted to the Dana‐Duek Children's hospital between Jan. 1, 2000 and Sep. 30, 2013. All charts were thoroughly reviewed for demographic characteristics (e.g. age, gender), LOS (in days by date), presenting and later symptoms (abdominal pain, vomiting, GI bleeding, palpable purpura, arthralgia or periarthritis, fever) and laboratory data on admission and discharge (CBC, CRP, ESR, serum creatinine, urinalysis). In univariate analysis we studied the variables that were statistically related to LOS. Variables that significantly predicted LOS in univariate analysis or that were clinically relevant (i.e. Proteinuria (defined as positive protein in urinalysis in 2 consecutive tests), systemic fever (defined as rectal temperature ≥38.0°C), gastrointestinal (GI) bleeding, arthralgia, CRP concentration, age (years) and abdominal pain as sole presenting symptom on admission) were analyzed in stepwise backward multiple regression analysis. Variables that remained significant in the final analysis were correlated with LOS, were each given specific weight (according to their contribution to the final R square) and were used to assemble an HSP severity score. Discriminate analysis was used to determine the score best discriminating patients with prolonged LOS (4 or more days) from the others.Results:Eighty nine children (aged 9 months–17 years) were retrieved for analysis. In univariate analysis, variables that were associated with prolonged LOS were: abdominal pain as initial sole presentation (P < 0.001), CRP (P = 0.001) and systemic fever (P = 0.018). Four variables remained significant in the final stepwise multiple regression analysis, and were used as parameters in assembling the HSP severity score. The HSP severity score consisted of sole abdominal pain at presentation (0 or 3 points), Fever (0 or 2 points), CRP ≥45 (0 or 1 point) and Age ≥6 (0 or 0.5 points). A score of ≥3 predicted a prolonged LOS (4 or more days) with a sensitivity of 76%, a specificity of 79% and a positive predictive value of 93%.Conclusion:Four selected variables available on the day of admission allow predicting the severity (in terms of LOS) of HSP. This score will be validated in an ongoing prospective study.
A100: Predictors of Involvement in Leisure Activities Among Children and Youth With Juvenile Idiopathic Arthritis
Authors: Cavallo Sabrina , Majnemer Annette , Duffy Ciarán M. , Feldman Debbie Ehrmann 
Published: March 2014

Background/Purpose:Children with juvenile idiopathic arthritis may be less involved in leisure activities compared to their healthy peers in part due to disease related factors such as pain, joint stiffness and tenderness. The main purpose of our study was to describe involvement in leisure activities, as well as to identify potential socio‐demographic, disease‐related, personal and environmental determinants.Methods:Ninety three children and youth aged 8 to 18 years diagnosed with juvenile idiopathic arthritis (JIA) and their families participated in this cross‐sectional study. Children and youth were administered the Children's Assessment of Participation and Enjoyment (CAPE), which measures involvement in leisure activities (recreation, physical, social, skill‐based, self‐improvement). The disease characteristics were abstracted from the child's medical file (JIA sub‐type, active joint count, age of diagnosis), pain perception, functional status, motivation, self‐perception and perceived social support were obtained through self‐report. Parents completed questionnaires on socio‐demographic data, as well as on family function and environmental barriers. Multiple linear regression was used for data analysis.Results:The leisure activities completed by most participants (>90%) were playing computer/video games, watching television, hanging out with friends and doing homework. Least popular activities were (h10%) martial arts, gymnastics and horseback riding. The level of enjoyment was highest for social activities (mean: 4.2+0.5). Involvement in active physical activities was negatively associated with the presence of active disease (β = −0.385, p = 0.028). However a higher level of perceived athletic competence was associated with greater intensity in active physical activities (β = 0.261, p = 0.037). Higher environmental barrier scores were negatively associated with involvement in social (β = −0.222, p = 0.023) leisure activities.Conclusion:Disease activity may dissuade children and youth from participating in more active pursuits, which places them at greater risk for adopting sedentary lifestyles. There is evidence that contextual factors (personal and environmental) influence leisure participation. The identification of determinants of leisure activities in children and youth with arthritis may allow healthcare professionals to assess children's health needs with more precision and promote a healthier lifestyle.
A101: The Impact of Sleep and Cytokine Levels on Pain Perception in JIA
Authors: De Ranieri Deirdre I. , Wagner‐Weiner Linda , Tesher Melissa , Bandla Hari , Onel Karen 
Published: March 2014

Background/Purpose:Children with JIA experience more pain and disordered sleep than healthy children. JIA and poor sleep are associated with particular cytokine abnormalities, and the correlation between the two and how they relate to a patient's pain perception have not been studied. Elevation in TNF‐a, IL‐6, IL‐17, and IFN‐g have been found in the serum of children with JIA during active disease. Levels of proinflammatory cytokines are also increased during sleep loss. Indeed, blocking TNF‐a has been shown in adults with RA to improve sleep the night after the first dose. We hypothesize that dysfunctional sleep enhances pain in our patients and may be associated with abnormal cytokine profiles. In this pilot study, we hope to identify a subgroup of children with JIA that could benefit from intervention in improving sleep patterns.Methods:We plan to enroll 60 patients with JIA. Sleep is assessed by the sleep self‐report (SSR), the children's sleep habits questionnaire (CSHQ), and actigraphy. Good concordance in measurements of sleep duration has been established between PSG and actigraphy. Patients wear an actigraphy watch and keep a daily sleep and pain diary for 14 days. Disease activity is assessed using ESR, sum of active joints, patient/parent and physician global assessment of disease activity. Serum cytokine testing will be performed by flow cytometry.Results:31 patients are enrolled. We identified 7 children with disordered sleep using CSHQ/ SSR. These patients report having pain on most days, and they trend toward having elevated disease activity (increased physician and patient global assessments as well as number of active joints, ESR was non‐contributory). To date, 9 of the 31 patients underwent actigraphy. Preliminary data does not indicate an association between disease activity and objective sleep dysfunction. There is not yet enough data to comment on the association of pain and disordered sleep in the absence of disease activity. Serum from 22 patients has been collected for cytokine studies.Conclusion:There is a trend toward an association of perceived poor sleep (as reported subjectively by SSR/CSHQ) with increased pain and disease activity in our patient population. Increased disease activity does not appear to be objectively related to disordered sleep using actigraphy, however. So far, actigraphy has been conducted on patients without subjective complaints of sleep dysfunction. We plan to collect more objective data (via actigraphy) on patients with subjective sleep complaints (with and without active disease) to better characterize the relationship of pain and sleep. When this has been accomplished, we plan to investigate cytokine profiles in these groups.
A111‐a: Setting Up of Japanese Online Registry of Pediatric Rheumatic Disease
Authors: Yamaguchi Ken‐ichi , Nerome Yasuhito , Imagawa Tomoyuki 
Published: March 2014

Background/Purpose:Performing quality clinical and translational research in pediatric rheumatic diseases (eg. Juvenile Idiopathic Arthritis (JIA), Child onset Systemic Lupus Erythematosus (cSLE) and Juvenile Dermatomyositis (JDM)) had been difficult due the rarity of these diseases.Methods:Professor Takei, the former chairman of Pediatric Rheumatology Association of Japan (PRAJ), had ordered his working group to set up a new registry system in 2012. We, working group, have set up Japanese online registry for pediatric rheumatic disease in 2014 and have named it as Pediatric Rheumatology International Collaboration Unit Registry (PRICURE).Results:Under this registry, PRAJ will collect basic information (e.g. age of onset, sex, symptoms, major complication, main treatment) about these diseases (JIA, SLE, JDM, Sjogren syndrome, Mixed Connective Tissue Disease, Systemic Sclerosis, Behcet's disease, Vasculitis, Autoinflammatory syndromes, Fibromyalgia).Conclusion:We had set up new registry of pediatric rheumatic diseases. We will show initial data from this registry here.
A113: TCR‐independent Activation of T‐cells Through CD31 Triggering as an Etiology of Cytokine Production in Juvenile Idiopathic Arthritis
Authors: Ferguson Ian D. , Griffin Patricia , Michel Joshua , Kietz Daniel , Rosenkranz Margalit , Vallejo Abbe N. 
Published: March 2014

Background/Purpose:JIA is the banner diagnosis for a heterogenous group of rheumatic conditions characterized by local inflammation and destruction of the joint space and systemic manifestations. Pro‐inflammatory cytokines derived from T cells are considered effectors of disease. Recent data has shown that some of these cytokines come directly from a distinct subset of T cells expressing CD31, an adhesion molecule expressed by granulocytes and endothelial cells. In the present work, we examined whether the array of cytokines in blood and synovial fluid of patients are the same array produced by T cells activation through CD31 ligation independent of the TCR. We propose that CD31‐driven cytokine production by T cells is a self‐perpetuating mechanism of local and systemic inflammation in JIA.Methods:Following informed consent/assent, blood and/or synovial fluid were collected from patients with oligoarticular and polyarticular JIA. Similar blood samples were also collected from healthy subjects. Cytokine composition of the plasma and/or fluid were subjected to multiplex analysis using the Luminex platform. Phenotypes of mononuclear cells in blood and synovial fluid were examined by multicolor flow cytometry. Drawing from the results of the Luminex assay, receptor crosslinking bioassays using primary CD31+ T cells (in blood and/or synovial fluid) and CD31+ T cell lines were conducted. Cytokine production by these cells were assessed by flow cytometry and/or by Luminex.Results:The overall cytokine signature of JIA characterized by the dominance of IL‐6, IL‐10 and TNFα. Unexpectedly, patients with oligoarticular disease showed higher levels of IFNγ and IL‐17 family cytokines than those with polyarticular disease. Both types of patients however showed a similar cellular signature characterized by the preponderance of T cells deficient in CD4, CD8, and CD28, but expressing CD31. Results of CD31 cross‐linking bioassays, using either specific antibody or its recombinant ligand CD38, showed high levels of induction of IL‐2, IL‐17 and IFNγ. Specificity of CD31‐driven, TCR‐dependent cytokine production was verified by similar bioassays using somatic somatic T cell line mutants Loucy and JRT3 that lack TCR and TCR/CD3, respectively, but are both CD31+. The observed cytokine production was associated with the phosphorylation of the signaling substrates ZAP70, cAbl, and p65‐NFKB.Conclusion:Our data show that the cytokine signature of oligoarticular JIA appears distinct with the dominance of IFNγ and IL17. TCR independent, CD31‐driven induction of these cytokines suggests maladaptive T cell function in JIA. Further investigation on the relevance of IFNγ and IL‐17 to disease activity/clinical outcomes, and the CD31 signaling pathway will pave way to innovations in immunotherapy in JIA.