Rheumatology Commons^™

Prosthetic Temporomandibular Joint Reconstruction In A Cohort Of Adolescent Females With Juvenile Idiopathic Arthritis., Michael Lypka, Karina Shah, Jordan T. Jones

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Temporomandibular joint (TMJ) arthritis and involvement is commonly seen in Juvenile Idiopathic Arthritis (JIA). Therapy includes conservative measures, but also includes intraarticular corticosteroid injections (IASI) and systemic immunosuppressive therapy. Despite aggressive medical therapy, some patients develop arthritic changes and frank TMJ ankylosis that can result in persistent pain and limitation in range of motion (ROM). A surgical option is prosthetic TMJ replacement with concurrent correction of dentofacial deformities, which can be performed simultaneously. The objective of this study was to evaluate the outcomes of prosthetic TMJ replacement in a cohort of adolescent females with JIA and severe TMJ involvement. …

Assessment And Treatment Of Down Syndrome-Associated Arthritis: A Survey Of Pediatric Rheumatologists., Anna Nicek, Nasreen Talib, Daniel Lovell, Chelsey Smith, Mara L. Becker, Jordan T. Jones

Manuscripts, Articles, Book Chapters and Other Papers

Background: Inflammatory arthritis in children with Down syndrome (DS) was first described in 1984 and is now termed Down syndrome-associated arthritis (DA). Studies have shown that DA is under-recognized with a 19-month average delay in diagnosis. Additionally, most patients present with polyarticular, rheumatoid factor (RF) and anti-nuclear antibody (ANA) negative disease. Current therapies for juvenile idiopathic arthritis (JIA) have been used, but appear to be poorly tolerated, more toxic and less effective in patients with DA. There is currently no standardized approach to the assessment or management of DA. The objective of this study was to describe provider perspectives toward …

Multifocal Bone Pain, Recurrent Fevers And Anemia In A 15-Year-Old, Anna M. Martens, Catherine Ezzio, Amanda Goddard, Leah Mallory

Journal of Maine Medical Center

Introduction: Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory bone disorder that primarily affects children with recurrent episodes of sterile osteomyelitis in one or more bones. Patients usually present with recurrent bone pain, though it is often a diagnosis of exclusion with most patients undergoing extensive work-up to arrive at a diagnosis.

Clinical Findings: This 15-year-old female presented with months of progressive multifocal bone pain, 22-pound weight loss and recurrent fevers. Work-up as an outpatient included a positive screening assay and IgM Western blot Panel positive for Lyme disease, treated with a 28-day course doxycycline without resolution of the symptoms, …

The Great Imitator Strikes Again: A Case Of A Lupus Flare-Up Presenting Like An Acute Abdomen, Emmanouil Kiriakopoulos, Vanessa Perez, Robyn Hoelle

HCA Healthcare Journal of Medicine

Systemic lupus erythematosus (SLE) is an autoimmune disease affecting all age groups and can manifest in various forms, often making the initial or successive presentations difficult to diagnose. Peritonitis secondary to lupus is a rare manifestation of this disease and the prevalence is said to be much lower in children. We present a case report of an adolescent male with a known history of lupus who presented to the emergency department with a clinical picture consistent with an acute surgical abdomen and underwent an appendectomy. Subsequent workup identified the culprit as a lupus-related peritonitis requiring corticosteroids for resolution.

Pain, Functional Disability, And Their Association In Juvenile Fibromyalgia Compared To Other Pediatric Rheumatic Diseases., Mark Connelly, Jennifer E Weiss, Carra Registry Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Severe pain and impairments in functioning are commonly reported for youth with juvenile fibromyalgia. The prevalence and impact of pain in other diseases commonly managed in pediatric rheumatology comparatively have been rarely systematically studied. The objective of the current study was to determine the extent to which high levels of pain and functional limitations, and the strength of their association, are unique to youth with juvenile primary fibromyalgia syndrome/JPFS) relative to other pediatric rheumatic diseases.

METHODS: Using data from 7753 patients enrolled in the multinational Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry, we compared the levels and …

Implementing Treat To Target Approach In The Care Of Children With Juvenile Idiopathic Arthritis, Julia G. Harris, Jamie Holland, Emily Fox, Leslie Favier, Ashley M. Cooper

Posters

Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory disease seen in pediatric rheumatology. Despite advances in biologic therapy, many children with JIA will experience morbidity. Treat to Target is a treatment paradigm that involves setting a target of disease activity, using shared decision making, and incorporating clinical decision support. Our clinic aimed to set a treatment target on 50% of eligible JIA patients by December 31, 2019.

Collaboration Between Rheumatology Clinic And Specialty Pharmacy To Positively Impact Patient Experience And Hospital Stewardship, Alaina Linafelter, Julia G. Harris, Robert Herr, Stephanie Quinn, Ashley M. Cooper

Posters

Biologic medications are commonly utilized to treat pediatric rheumatic diseases. Being high-cost, most third-party payers require dispensing through a specialty pharmacy. Children's Mercy Hospital Specialty Pharmacy (CMH SP) started acceting patients in March 2015.

The number of patients benefited by the superior service of CMH SP has risen steadily, leading to high patient satisfaction and financial benefits for the hospital.

Improving Pneumococcal Vaccination Rates In High Risk Patients Across Multiple Specialty Divisions, Rachel Moran, Julia G. Harris, Claire Olsen, Rana El Feghaly, Liset Olarte, Douglas Blowey, Luke A. Harris

Posters

Pediatric patients with deficient immune systems or certain chronic medical conditions have an increased risk of acquiring invasive pneumococcal disease.

The 23-valent pneumococcal (PPSV23) vaccine provides protection against 23 pneumococcal serotypes and is recommended for patients aged 2 years or older who are high-risk for invasive pneumococcal disease.

Unfortunately, many high-risk patients are not properly vaccinated due to lack of provider knowledge or understanding of accountability between primary care and specialty providers.

The goal of this project was to improve PPSV23 vaccination rates by 10-20% across multiple Children's Mercy Kansas City specialty divisions.

Demographic, Clinical, And Treatment Characteristics Of The Juvenile Primary Fibromyalgia Syndrome Cohort Enrolled In The Childhood Arthritis And Rheumatology Research Alliance Legacy Registry., Jennifer E. Weiss, Kenneth N. Schikler, Alexis D. Boneparth, Mark Connelly, Carra Registry Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: To describe the demographic, clinical, and treatment characteristics of youth diagnosed with juvenile primary fibromyalgia syndrome (JPFS) who are seen in pediatric rheumatology clinics.

METHODS: Information on demographics, symptoms, functioning, and treatments recommended and tried were obtained on patients with JPFS as part of a multi-site patient registry (the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry). Data were summarized using descriptive statistics. In a subset of patients completing registry follow-up visits, changes in symptoms, pain, and functioning were evaluated using growth modeling.

RESULTS: Of the 201 patients with JPFS enrolled in the registry, most were Caucasian/White (85%), non-Hispanic …

Physician Practices For Withdrawal Of Medications In Inactive Systemic Juvenile Arthritis, Childhood Arthritis And Rheumatology Research Alliance (Carra) Survey., Susan Shenoi, Kabita Nanda, Grant S. Schulert, John F. Bohnsack, Ashley M. Cooper, Bridget Edghill, Miriah C. Gillispie-Taylor, Baruch Goldberg, Olha Halyabar, Thomas G. Mason, Tova Ronis, Rayfel Schneider, Richard K. Vehe, Karen Onel, Childhood Arthritis And Rheumatology Research Alliance Systemic Juvenile Idiopathic Arthritis Workgroup

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: We describe a Childhood Arthritis and Rheumatology Research Alliance (CARRA) survey of North American pediatric rheumatologists that assesses physician attitudes on withdrawal of medications in systemic juvenile idiopathic arthritis (SJIA).

METHODS: A REDCap anonymous electronic survey was distributed to 100 random CARRA JIA workgroup physician-voting members. The survey had three broad sections including: A) demographic information; B) physicians' opinions on clinical inactive disease (CID) in SJIA and C) existing practices for withdrawing medications in SJIA.

RESULTS: The survey had an 86% response rate. 88 and 93% of participants agreed with the current criteria for CID and clinical remission on …

Developing Comparative Effectiveness Studies For A Rare, Understudied Pediatric Disease: Lessons Learned From The Carra Juvenile Localized Scleroderma Consensus Treatment Plan Pilot Study., Suzanne C. Li, Robert C. Fuhlbrigge, Ronald M. Laxer, Elena Pope, Maria Ibarra, Katie Stewart, Thomas Mason, Mara L. Becker, Sandy Hong, Fatma Dedeoglu, Kathryn S. Torok, C Egla Rabinovich, Polly J. Ferguson, Marilynn Punaro, Brian M. Feldman, Tracy Andrews, Gloria C. Higgins, Carra Registry Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: We designed and initiated a pilot comparative effectiveness study for juvenile localized scleroderma (jLS), for which there is limited evidence on best therapy. We evaluated the process we used, in relation to the specific protocol and to the general task of identifying strategies for implementing studies in rare pediatric diseases.

METHODS: This was a prospective, multi-center, observational cohort study of 50 jLS patients initiating treatment, designed and conducted by the jLS group of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) from 2012 to 2015. A series of virtual and physical meetings were held to design the study, standardize …

Association Of Slco1b1 *14 Allele With Poor Response To Methotrexate In Juvenile Idiopathic Arthritis Patients, Laura B. Ramsey, Halima Moncrieffe, Chelsey Smith, Marc Sudman, Miranda C. Marion, Carl D. Langefeld, Mara L. Becker, Susan D. Thompson

Manuscripts, Articles, Book Chapters and Other Papers

Objective: Variants in the SLCO1B1 gene, encoding a hepatic methotrexate (MTX) transporter, affect clearance of high-dose MTX. We tested whether in the *14 and *15 alleles of SLCO1B1 influenced the response to low-dose MTX in juvenile idiopathic arthritis (JIA) patients.

Methods: The study included 310 JIA patients genotyped for three single nucleotide polymorphisms (SNPs) in SLCO1B1 (rs4149056, rs2306283, and rs11045819). A patient's SLCO1B1 diplotype was determined by combining the SNPs into the *1a, *1b, *4, *5, *14, and *15 alleles. Number of active joints at follow-up (visit closest to 6 months of treatment and prior …

Frequency Of Cd19, Qianzi Zhao, Lawrence K Jung

Medicine Faculty Publications

BACKGROUND: To understand the relationship between regulatory B cells (Bregs) and juvenile idiopathic arthritis (JIA), we analyzed the percentages of Bregs and their function in peripheral blood (PB) and synovial fluid (SF) of JIA patients.

METHODS: Twenty-one JIA patients and 11 children with growing pain but without known rheumatic diseases as controls were included. The B cell phenotype and intracellular production of IL-10 of Bregs were assessed by flow cytometry. Mononuclear cells from PB and SF were stimulated to produce IL-10 in vitro for the identification of IL-10- producing regulatory B cells.

RESULTS: The percentage of CD24

CONCLUSIONS: The inability …

A 6-Month, Multicenter, Open-Label Study Of Fixed Dose Naproxen/Esomeprazole In Adolescent Patients With Juvenile Idiopathic Arthritis, D. J. Lovell, J. A. Dare, M. Francis-Sedlak, J. Ball, B. D. Lamoreaux, E. Von Scheven, A. Reinhardt, R. Jerath, B. Gottlieb, R. J. Holt, +6 Additional Authors

Journal Articles

© 2018 The Author(s). Background: Juvenile idiopathic arthritis (JIA) is an inflammatory arthritis of unknown etiology, which lasts for greater than 6 weeks with onset before 16 years of age. JIA is the most common chronic rheumatic disease in children. NSAIDs have been the mainstay of initial management with naproxen (NAP) being commonly used, but they may cause serious side effects such as gastric ulcers which can be reduced by concomitant administration of proton pump inhibitors, such as esomeprazole (ESO). Methods: Primary objective was to evaluate the safety and tolerability of 3 fixed doses of NAP/ESO in JIA patients aged …

Evaluation Of Performance, Acceptance, And Compliance Of An Auto-Injector In Healthy And Rheumatoid Arthritic Subjects Measured By A Motion Capture System., Xiao Xiao, Wei Li, Corbin Clawson, David Karvani, Perceval Sondag, James K Hahn

Pediatrics Faculty Publications

Purpose: The study aimed to develop a motion capture system that can track, visualize, and analyze the entire performance of self-injection with the auto-injector.

Methods: Each of nine healthy subjects and 29 rheumatoid arthritic (RA) patients with different degrees of hand disability performed two simulated injections into an injection pad while six degrees of freedom (DOF) motions of the auto-injector and the injection pad were captured. We quantitatively measured the performance of the injection by calculating needle displacement from the motion trajectories. The max, mean, and SD of needle displacement were analyzed. Assessments of device acceptance and usability were evaluated …

Pediatric Rheumatology: Addressing The Transition To Adult-Orientated Health Care., Sara Sabbagh, Tova Ronis, Patience H White

Medicine Faculty Publications

The transition from pediatric to adult health care is often a challenging process due to multiple interwoven complexities, especially for children with chronic medical conditions. Health care transition (HCT) is a process of moving from a pediatric to an adult model of health care with or without a transfer to a new clinician. This paper focuses on what is known about HCT for youth and young adults (Y/YA) with rheumatic diseases within a larger context of HCT recommendations. HCT barriers for youth, families, and providers and current evidence for a structured HCT processes are reviewed. Practical advice is offered on …

Medication Education In The Pediatric Rheumatology Clinic, Ashley M. Cooper, Julia G. Harris

Posters

No abstract provided.

Pilot Study Comparing The Childhood Arthritis & Rheumatology Research Alliance (Carra) Systemic Juvenile Idiopathic Arthritis Consensus Treatment Plans., Yukiko Kimura, Sriharsha Grevich, Timothy Beukelman, Esi Morgan, Peter A. Nigrovic, Kelly Mieszkalski, T Brent Graham, Maria Ibarra, Norman Ilowite, Marisa Klein-Gitelman, Karen Onel, Sampath Prahalad, Marilynn Punaro, Sarah Ringold, Dana Toib, Heather Van Mater, Jennifer E. Weiss, Pamela F. Weiss, Laura E. Schanberg, Carra Registry Investigators

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: To assess the feasibility of studying the comparative effectiveness of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) consensus treatment plans (CTPs) for systemic Juvenile Idiopathic Arthritis (JIA) using an observational registry.

METHODS: Untreated systemic JIA patients enrolled in the CARRA Registry were begun on one of 4 CTPs chosen by the treating physician and patient/family (glucocorticoid [GC] alone; methotrexate [MTX] ± GC; IL1 inhibitor [IL1i] ± GC; IL6 inhibitor [IL6i] ± GC). The primary outcome of clinical inactive disease (CID) without current GC use was assessed at 9 months.

TRIAL REGISTRATION: clinicaltrials.gov NCT01697254; first registered 9/28/12 (retrospectively enrolled). …

Fine-Mapping The Mhc Locus In Juvenile Idiopathic Arthritis (Jia) Reveals Genetic Heterogeneity Corresponding To Distinct Adult Inflammatory Arthritic Diseases., A Hinks, J Bowes, J Cobb, H C. Ainsworth, M C. Marion, M E. Comeau, M Sudman, B Han, Juvenile Arthritis Consortium For Immunochip, Mara L. Becker, J F. Bohnsack, P I W De Bakker, J P. Haas, M Hazen, D J. Lovell, P A. Nigrovic, E Nordal, M Punnaro, A M. Rosenberg, M Rygg, S L. Smith, C A. Wise, V Videm, L R. Wedderburn, A Yarwood, R S M Yeung, S Prahalad, C D. Langefeld, S Raychaudhuri, S D. Thompson, W Thomson

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: Juvenile idiopathic arthritis (JIA) is a heterogeneous group of diseases, comprising seven categories. Genetic data could potentially be used to help redefine JIA categories and improve the current classification system. The human leucocyte antigen (HLA) region is strongly associated with JIA. Fine-mapping of the region was performed to look for similarities and differences in HLA associations between the JIA categories and define correspondences with adult inflammatory arthritides.

METHODS: Dense genotype data from the HLA region, from the Immunochip array for 5043 JIA cases and 14 390 controls, were used to impute single-nucleotide polymorphisms, HLA classical alleles and amino acids. …

Validation Of Patient-Reported Outcomes Measurement Information System Short Forms For Use In Childhood-Onset Systemic Lupus Erythematosus., Jordan T. Jones, Adam C. Carle, Janet Wootton, Brianna Liberio, Jiha Lee, Laura E. Schanberg, Jun Ying, Esi Morgan Dewitt, Hermine I. Brunner

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: To validate the pediatric Patient-Reported Outcomes Measurement Information System short forms (PROMIS-SFs) in childhood-onset systemic lupus erythematosus (SLE) in a clinical setting.

METHODS: At 3 study visits, childhood-onset SLE patients completed the PROMIS-SFs (anger, anxiety, depressive symptoms, fatigue, physical function-mobility, physical function-upper extremity, pain interference, and peer relationships) using the PROMIS assessment center, and health-related quality of life (HRQoL) legacy measures (Pediatric Quality of Life Inventory, Childhood Health Assessment Questionnaire, Simple Measure of Impact of Lupus Erythematosus in Youngsters [SMILEY], and visual analog scales [VAS] of pain and well-being). Physicians rated childhood-onset SLE activity on a VAS and completed …

Evidence-Based Decision Support For Pediatric Rheumatology Reduces Diagnostic Errors., Michael M. Segal, Balu Athreya, Mary Beth F. Son, Irit Tirosh, Jonathan S. Hausmann, Elizabeth Yn. Ang, David Zurakowski, Lynn K. Feldman, Robert P. Sundel

Department of Pediatrics Faculty Papers

BACKGROUND: The number of trained specialists world-wide is insufficient to serve all children with pediatric rheumatologic disorders, even in the countries with robust medical resources. We evaluated the potential of diagnostic decision support software (DDSS) to alleviate this shortage by assessing the ability of such software to improve the diagnostic accuracy of non-specialists.

METHODS: Using vignettes of actual clinical cases, clinician testers generated a differential diagnosis before and after using diagnostic decision support software. The evaluation used the SimulConsult® DDSS tool, based on Bayesian pattern matching with temporal onset of each finding in each disease. The tool covered 5405 diseases …

Monitoring Change In Volume Of Calcifications In Juvenile Idiopathic Inflammatory Myopathy: A Pilot Study Using Low Dose Computed Tomography., Maria Ibarra, Cynthia Rigsby, Gabrielle A. Morgan, Christina L. Sammet, Chiang-Ching Huang, Dong Xu, Ira N. Targoff, Lauren M. Pachman

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Dystrophic calcifications may occur in patients with J uvenile Idiopathic Inflammatory Myopathy (JIIM) as well as other connective tissue and metabolic diseases, but a reliable method of measuring the volume of these calcifications has not been established. The purpose of this study is to determine the feasibility of low dose, limited slice, Computed Tomography (CT) to measure objectively in-situ calcification volumes in patients with JIIM over time.

METHODS: Ten JIIM patients (eight JDM, two Overlap) with calcifications were prospectively recruited over a 2-year period to undergo two limited, low dose, four-slice CT scans. Calculation of the volume of calcifications …

Decreasing Patient Cost And Travel Time Through Pediatric Rheumatology Telemedicine Visits., Elizabeth A. Kessler, Ashley K. Sherman, Mara L. Becker

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: There is a critical shortage of pediatric rheumatologists in the US. Substantial travel to clinics can impose time and monetary burdens on families. The aim of this study was to evaluate the cost of in-person pediatric rheumatology visits for families and determine if telemedicine clinics resulted in time and cost savings. Factors associated with interest in telemedicine were also explored.

METHODS: Surveys were offered to parents and guardians of patients in Pediatric Rheumatology follow-up clinics in Kansas City, Missouri, the primary site of in-person care, and at a telemedicine outreach site 160 miles away, in Joplin, Missouri. Survey questions …

Variants In Cxcr4 Associate With Juvenile Idiopathic Arthritis Susceptibility., Terri H. Finkel, Jin Li, Zhi Wei, Wei Wang, Haitao Zhang, Edward M. Behrens, Emma L. Reuschel, Sophie Limou, Carol Wise, Marilynn Punaro, Mara L. Becker, Jane E. Munro, Berit Flatø, Øystein Førre, Susan D. Thompson, Carl D. Langefeld, David N. Glass, Joseph T. Glessner, Cecilia E. Kim, Edward Frackelton, Debra K. Shivers, Kelly A. Thomas, Rosetta M. Chiavacci, Cuiping Hou, Kexiang Xu, James Snyder, Haijun Qiu, Frank Mentch, Kai Wang, Cheryl A. Winkler, Benedicte A. Lie, Justine A. Ellis, Hakon Hakonarson

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease among children, the etiology of which involves a strong genetic component, but much of the underlying genetic determinants still remain unknown. Our aim was to identify novel genetic variants that predispose to JIA.

METHODS: We performed a genome-wide association study (GWAS) and replication in a total of 1166 JIA cases and 9500 unrelated controls of European ancestry. Correlation of SNP genotype and gene expression was investigated. Then we conducted targeted resequencing of a candidate locus, among a subset of 480 cases and 480 controls. SUM test was performed …

Initial Benchmarking Of The Quality Of Medical Care In Childhood-Onset Systemic Lupus Erythematosus., Rina Mina, Julia G. Harris, Marisa S. Klein-Gitelman, Simone Appenzeller, Maraisa Centeville, Diane Eskra, Jennifer L. Huggins, Anne L. Johnson, Raju Khubchandani, Prachi Khandekar, Jiha Lee, Hai Mei Liu, Joshua D. Pendl, Clovis A. Silva, Marco F. Silva, Ahmad I. Zaal, Esi Morgan Dewitt, Stacy P. Ardoin, Hermine I. Brunner

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: To assess the quality of medical care in childhood-onset systemic lupus erythematosus (SLE) at tertiary pediatric rheumatology centers as measured by observance of SLE quality indicators (SLE-QIs).

METHODS: International consensus has been achieved for childhood-onset SLE-QIs capturing medical care provision in 9 domains: diagnostic testing, education of cardiovascular (CV) risk and lifestyles, lupus nephritis (LN), medication management, bone health, ophthalmologic surveillance, transition, pregnancy, and vaccination. Using medical record information, the level of performance of these childhood-onset SLE-QIs was assessed in childhood-onset SLE populations treated at 4 tertiary pediatric rheumatology centers in the US, 2 in Brazil, and 1 center …

Validation Of The Lupus Nephritis Clinical Indices In Childhood-Onset Systemic Lupus Erythematosus, R. Mina, K. Abulaban, M. Klein-Gitelman, A. Eberhard, S. Ardoin, N. Singer, K. Onel, L. Tucker, K. O'Neil, H. I. Brunner, +8 Additional Authors

Journal Articles

OBJECTIVE: To validate clinical indices of lupus nephritis (LN) activity and damage when used in children against the criterion standard of kidney biopsy findings. METHODS: In 83 children requiring kidney biopsy the SLE Disease Activity Index Renal Domain (SLEDAI-R); British Isles Lupus Assessment Group index Renal Domain (BILAG-R), Systemic Lupus International Collaborating Clinics Renal Activity (SLICC-RAS) and Damage Index Renal Domain (SDI-R) were measured. Fixed effect and logistic models were done to predict International Society of Nephrology/Renal Pathology Society (ISN/RPS) class; low/moderate vs. high LN-activity [NIH Activity Index (NIH-AI) score:10; Tubulointerstitial Activity Index (TIAI) score:5) or the absence vs. presence …

Development Of A Novel Renal Activity Index Of Lupus Nephritis In Children & Young Adults, H. I. Brunner, M. Bennett, K. Abulaban, M. Klein-Gitelman, K. O'Neil, L. Tucker, S. Ardoin, K. Rouster-Stevens, B. A. Eberhard, P. Devarajan, +8 Additional Authors

Journal Articles

BACKGROUND: Noninvasive estimation of the degree of inflammation seen on kidney biopsy with lupus nephritis (LN) remains difficult. The objective of this study was to develop a Renal Activity Index for Lupus (RAIL) that, based solely on laboratory measures, accurately reflects histological LN activity. METHODS: We assayed traditional LN laboratory tests and 16 urine biomarkers (UBMs) in children (n=47) at the time of kidney biopsy. Histological LN activity was measured by the NIH Activity Index (NIH-AI) and the Tubulointerstitial Activity Index (TIAI). High LN-activity status (vs. moderate/low) was defined as NIH-AI scores > 10 (vs.5 (vs.92% accuracy and LN-activityTIAI status with …

Effects Of Obesity On Health-Related Quality Of Life In Juvenile-Onset Systemic Lupus Erythematosus, R. Mina, M. S. Klein-Gitelman, S. Nelson, B. A. Eberhard, G. Higgins, N. G. Singer, K. Onel, L. Tucker, K. M. O'Neil, H. I. Brunner, +4 Additional Authors

Journal Articles

OBJECTIVE: This study evaluated the effects of obesity on health-related quality of life (HRQOL) measures in juvenile-onset systemic lupus erythematosus (jSLE). METHODS: Obesity was defined as a body mass index (BMI) >/=95th percentile according to the Sex-specific Center for Disease Control BMI-For-Age Charts and determined in a multicenter cohort of jSLE patients. In this secondary analysis, the domain and summary scores of the Pediatric Quality of Life (PedsQL) Inventory and the Child Health Questionnaire (CHQ) of obese jSLE patients were compared to those of non-obese jSLE patients as well as historical obese and non-obese healthy controls. Mixed-effects modeling was performed …

Pfapa: A Periodic Febrile Syndrome Afflicting Children, Jessica Lahti

Physician Assistant Scholarly Project Posters

• Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA).

• Auto-inflammatory disorder occurring in affected children every 3-8 weeks.

• Onset around age 2; lasting into second decade of life.

• A current pathophysiologic theory is PFAPA is a disorder of the innate immune system involving interleukins, chemoattractants, and cytokines that stimulating the inflammatory response causing symptoms associated with PFAPA.

• Early diagnosis of PFAPA may help prevent the unnecessary use of antibiotics.

• Biomarker CD64, galectin-3 and the MPV may be useful in the diagnosis of PFAPA along with other laboratory values during the febrile attack period …

High Levels Of Dek Autoantibodies May Predict Early Flare Following Cessation Of Anti-Tnf Therapy In Juvenile Idiopathic Arthritis, Nirit Mor-Vaknin, Miguel Rivas, Maureen Legendre, Y. Yuanfang, Anne Johnson, Lawrence K. Jung, +12 Additional Authors

Pediatrics Faculty Publications

Introduction The nuclear oncoprotein DEK is a biochemically distinct, pro-inflammatory protein that is a chemoattractant for neutrophils and T-cells. High levels of DEK autoantibodies have been found in several autoimmune diseases including juvenile idiopathic arthritis (JIA), but their role in disease pathogenesis is unclear.

Objectives Since DEK and DEK autoantibodies can contribute to the development of immune complexes and joint inflammation, we suggest that DEK antibody levels can predict disease flare with discontinuation of anti-TNF therapy. Methods In 16 pediatric rheumatology centers, sera samples were collected from 137 children with polyarticular JIA on anti-TNF therapy. Therapy was stopped after 6 …