Rheumatology Commons^™

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 …

Medical Student Competency In Wound Care Guidelines, Sophia Akhiyat, Sean Mcnish, Kara S. Couch, Victoria K. Shanmugam

GW Research Days 2016 - 2020

Chronic wounds that have failed to heal after 3 months of appropriate wound care affect approximately 6.5 million people in the US with a prevalence of 1% and costs estimated at $25 billion per year. Medical students currently receive limited wound care training, yet to effectively manage chronic wounds, providers must both understand the biology of healing, and also remain up-to-date with wound care guidelines published by the Agency for Healthcare Research and Quality (AHRQ). The purpose of this student-led project was to investigate medical students' knowledge and comfort with wound care guidelines.

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 …

Vitamin D Supplementation And Systemic Lupus Erythematosus, Pamela Rangen

Physician Assistant Scholarly Project Posters

• Patients with systemic lupus erythematosus (SLE) are prone to hypovitaminosis D because of their photosensitivity.

• Vitamin D has beneficial effects not only on bone metabolism but also on the function of the immune system.

• Vitamin D levels depend on many variables, including sun exposure, age, ethnicity, body mass index, use of medications and supplements.

• Patients with SLE avoid the sun because of photosensitive rashes and potential for disease flare, so adequate oral supplementation is critical.

• In addition, to the traditional known metabolic activities, vitamin D has been shown to modulate the immune system, and its …

Endothelial Cells Expressing Endothelial And Mesenchymal Cell Gene Products In Lung Tissue From Patients With Systemic Sclerosis-Associated Interstitial Lung Disease., Fabian A. Mendoza, Sonsoles Piera-Velazquez, John L. Farber, Carol Feghali-Bostwick, Sergio A. Jimenez

Department of Dermatology and Cutaneous Biology Faculty Papers

OBJECTIVE: To examine whether lung endothelial cells (ECs) from patients with systemic sclerosis (SSc)-associated interstitial lung disease (ILD) express mesenchymal cell-specific proteins and gene transcripts, indicative of the occurrence of endothelial-to-mesenchymal phenotypic transition (EndoMT).

METHODS: Lung tissue from 6 patients with SSc-associated pulmonary fibrosis was examined by histopathology and immunohistochemistry. Confocal laser microscopy was utilized to assess the simultaneous expression of EC and myofibroblast molecular markers. CD31+CD102+ ECs were isolated from the lung tissue of 2 patients with SSc-associated ILD and 2 normal control subjects, and the expression of EC and mesenchymal cell markers and other relevant genes was analyzed …

Stress Granules And Rna Processing Bodies Are Novel Autoantibody Targets In Systemic Sclerosis, Michael E. Johnson, Andrew V. Grassetti, Jaclyn N. Taroni, Shawn M. Lyons, Devin Schweppe, Jessica K. Gordon, Robert F. Speira, Robert Lafyatis, Paul J. Anderson, Scott A. Gerber, Michael L. Whitfield

Dartmouth Scholarship

Autoantibody profiles represent important patient stratification markers in systemic sclerosis (SSc). Here, we performed serum-immunoprecipitations with patient antibodies followed by mass spectrometry (LC-MS/MS) to obtain an unbiased view of all possible autoantibody targets and their associated molecular complexes recognized by SSc.

Effect Of Mycophenolate Mofetil On The White Blood Cell Count And The Frequency Of Infection In Systemic Lupus Erythematosus., Ananta Subedi, Laurence S. Magder, Michelle Petri

Department of Medicine Faculty Papers

Leukopenia is a common manifestation of SLE. Addition of immunosuppressive therapy in a SLE patient who is already leukopenic is a clinical concern. It could worsen leukopenia, increase the risk of infection, or both. The aim of this study was to analyze the immediate effect of mycophenolate mofetil on the white blood cell count and the rate of infection in SLE patients. Two hundred and forty-four patients within the Hopkins Lupus Cohort who were newly started on mycophenolate mofetil were included in the study. The white blood cell count and interval infection history on the day mycophenolate mofetil was started …

Use Of Dietary And Herbal Supplements In Older Adults With Osteoarthritis, Sharon Denise Mcdonald

Dissertations

Over the past several decades, older adults with Osteoarthritis (OA) have increasingly used more natural products in an effort to manage chronic pain. The theoretical framework for this study was the Lazarus and Folkman transactional theory of stress and coping. This framework was chosen based on the belief that older adults with OA are taking natural products as an active problem solving coping strategy to manage the pain, disability, and alterations they experience in their health related quality life including functional status, physical symptoms, emotional health, and social functioning. Older adults also take prescription medications for Osteoarthritis and a variety …

Libman-Sacks Endocarditis “A Wolf In Sheep’S Skin”, Philip Dunn Do, Ryan Rogers Do, James Ross Md

Department of Medicine

No abstract provided.

Prevalence Of Rheumatoid Arthritis In Low-And Middle-Income Countries: A Systematic Review And Analysis, Igor Rudan, Simrita Sidhu, Angeliki Papana, Shi–Jiao Meng, Yu Xin-Wei, Wei Wang, Ruth M. Campbell–Page, Alessandro Rhyll Demaio, Harish Nair, Devi Sridhar, Evropi Theodoratou, Ben Dowman, Davies Adeloye, Azeem Majeed, Josip Car, Harry Campbell, Wei Wang, Kit Yee Chan

Research outputs 2014 to 2021

Background: Rheumatoid arthritis (RA) is an autoimmune disorder that affects the small joints of the body. It is one of the leading causes of chronic morbidity in high–income countries, but little is known about the burden of this disease in low– and middle–income countries (LMIC).

Methods: The aim of this study was to estimate the prevalence of RA in six of the World Health Organization's (WHO) regions that harbour LMIC by identifying all relevant studies in those regions. To accomplish this aim various bibliographic databases were searched: PubMed, EMBASE, Global Health, LILACS and the Chinese databases CNKI and WanFang. Studies …

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 …

Safety And Efficacy Of Belimumab To Treat Systemic Lupus Erythematosus In Academic Clinical Practices, J. S. Hui-Yuen, A. Reddy, J. Taylor, X. Li, A. H. Eichenfield, L. M. Bermudez, A. J. Starr, L. F. Imundo, R. A. Furie, A. Askanase, +7 Additional Authors

Journal Articles

No abstract provided.

Mechanisms Of Endothelial Dysfunction In Systemic Lupus Erythematosus, Joy N. Jones Buie

MUSC Theses and Dissertations

Patients with systemic lupus erythematosus (SLE) have an increased propensity for developing endothelial dysfunction. Declines in endothelial derived NO production as evidenced by decreases in brachial artery flow mediated dilation, suggest that inadequate NO levels may be one mechanism whereby vascular homeostasis is perturbed in SLE patients. NO modifies progression to atherogenesis through inhibition of platelet aggregation, smooth muscle cell proliferation, adhesion molecule expression, and prevention of oxLDL. Recent studies suggest that Type I interferons and oxidative stress, leading to endothelial nitric oxide synthase uncoupling, are critical for accelerated vasculopathies observed in SLE. We postulate that two potential mechanisms are …

The Role Of Occupational Therapy In Primary Care, Priti Patel

Occupational Therapy Doctorate Capstone Projects

The Capstone Project is focused on communicating, influencing and educating other health care professionals regarding the role of occupational therapy in the future model of primary care delivery initiated by the adoption of the Affordable Care Act and the Triple Aim Initiative. Currently, primary care, the largest health care platform in United States, is not inclusive of occupational therapy services. Occupational therapists have the scope, knowledge and understanding to be part of the redesigned team model of primary care. Educating those currently working in primary care about adding occupational therapy services can have a significant impact on the profession of …

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 …

Analysis Of Humira, Electro-Acupuncture, And Pulsatile Dry Cupping On Reducing Joint Inflammation In Patients With Rheumatoid Arthritis, Natalie Noll

AUCTUS: The Journal of Undergraduate Research and Creative Scholarship

Humira, an anti-TNF drug aimed at decreasing inflammation in Rheumatoid Arthritis patients, can cause skin diseases from rashes to skin cancer. Humira works by blocking the chemical receptor RANKL which inhibits the production of osteoclasts. Osteoclasts are cells that attack and eat bone and cartilage therefore an inhibitory mechanism would cause inflammation.. By analyzing Humira’s effect on the human body, Humira can be compared to other treatments such as electro-acupuncture and pulsatile dry cupping to determine the viability of these alternative treatment methods in regards to their abilities to decrease inflammation in Rheumatoid Arthritis patients through blocking RANKL. An analysis …

Dengue Hemorrhagic Fever Complicated By Intercostal Artery Hemorrhage., Syed Ahsan Ali, Sara Ahmed, Mehmood Riaz

Department of Medicine

Hemorrhagic manifestations are fairly common in Dengue hemorrhagic fever and are associated with increased mortality. During last few decades there have been increasing reports of Dengue infection with unusual manifestations. Here we present a case of dengue hemorrhagic fever complicated by spontaneous rupture of an intercostal artery leading to a large hematoma which was treated successfully with angio-embolization. To the authors' knowledge this is a first case of dengue hemorrhagic fever complicated by spontaneous intercostal artery hemorrhage.

Role Of Cellular Senescence And Nox4-Mediated Oxidative Stress In Systemic Sclerosis Pathogenesis., Sonsoles Piera-Velazquez, Sergio A. Jimenez

Department of Dermatology and Cutaneous Biology Faculty Papers

Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by progressive fibrosis of skin and numerous internal organs and a severe fibroproliferative vasculopathy resulting frequently in severe disability and high mortality. Although the etiology of SSc is unknown and the detailed mechanisms responsible for the fibrotic process have not been fully elucidated, one important observation from a large US population study was the demonstration of a late onset of SSc with a peak incidence between 45 and 54 years of age in African-American females and between 65 and 74 years of age in white females. Although it is not appropriate …

Gene Expression Changes Reflect Clinical Response In A Placebo-Controlled Randomized Trial Of Abatacept In Patients With Diffuse Cutaneous Systemic Sclerosis, Eliza F. Chakravarty, Viktor Martyanov, David Fiorentino, Tammara A. Wood, David J. Haddon, Justin A. Jarrell, Paul Utz, Mark Genovese, Michael Whitfield, Lorinda Chung

Dartmouth Scholarship

Systemic sclerosis is an autoimmune disease characterized by inflammation and fibrosis of the skin and internal organs. We sought to assess the clinical and molecular effects associated with response to intravenous abatacept in patients with diffuse cutaneous systemic.

The Impact Of Waist Circumference On Function And Physical Activity In Older Adults: Longitudinal Observational Data From The Osteoarthritis Initiative, John A. Batsis, Alicia J. Zbehlik, Laura K. Barre, Todd A. Mackenzie, Stephen J. Bartels

Dartmouth Scholarship

Background: We previously demonstrated that BMI is associated with functional decline and reduced quality of life. While BMI in older adults is fraught with challenges, waist circumference (WC) is a marker of visceral adiposity that can also predict mortality. However, its association with function and quality of life in older adults is not well understood and hence we sought to examine the impact of WC on six-year outcomes.

Methods: We identified adults aged ≥60 years from the longitudinal Osteoarthritis Initiative and stratified the cohort into quartiles based on WC. Our primary outcome measures of function at six year follow-up included: …

Blau Syndrome, The Prototypic Auto-Inflammatory Granulomatous Disease., Carine H Wouters, Anne Maes, Kevin P Foley, John Bertin, Carlos D Rose

Department of Medicine Faculty Papers

Blau syndrome is a monogenic disease resulting from mutations in the pattern recognition receptor NOD2, and is phenotypically characterized by the triad of granulomatous polyarthritis, dermatitis and uveitis. This paper reviews briefly the classical clinical features of the disease, as well as more recently described extra-triad symptoms. From an ongoing prospective multicenter study, we provide new data on the natural history of Blau syndrome, focusing on functional status and visual outcome. We also present an update of the range of different NOD2 mutations found in Blau syndrome as well as recent data on morphologic and immunohistochemical characteristics of the Blau …

Division Of Rheumatology New Frontiers, Debra Bergman, John Keenan

New Frontiers

Table of Contents

• Nebraska Arthritis Outcomes Research Center puts UNMC on cutting edge
• Medical education: A fresh perspective
• The reader
• Lupus study becomes international affair
• The puzzle of ‘the loss of self-tolerance’
• An icon in rheumatology
• Registry, research offer insights into RA risk factors
• On the RISE
• RAIN man
• A top-tier team player
• Faculty Publications & Research Awards

Safety Of Infusing Rituximab At A More Rapid Rate In Patients With Rheumatoid Arthritis: Results From The Rate-Ra Study, Charles H. Pritchard, Maria W. Greenwald, Joel M. Kremer, Norman B. Gaylis, William Rigby

Dartmouth Scholarship

As recommended in the current prescribing information, rituximab infusions in patients with rheumatoid arthritis (RA) take 4.25hours for the first infusion and 3.25hours for subsequent infusions, which is a burden on patients and the health care system. We therefore evaluated the safety of infusing rituximab at a faster rate for an infusion period of 2hours in patients with RA.

Inborn Error Of Metabolism Is A Disease Of Any Age; Carniting Palmitoyltransferase Ii Deficiency And Its Manifestations, Mira Mitry Md, James Ross Md, Facp

Department of Medicine

No abstract provided.

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 …

Safety Of Celecoxib And Nonselective Nonsteroidal Anti-Inflammatory Drugs In Juvenile Idiopathic Arthritis: Results Of The Phase 4 Registry, R. E. Sobel, D. J. Lovell, H. I. Brunner, J. E. Weiss, P. W. Morris, B. S. Gottlieb, E. C. Chalom, L. K. Jung, K. B. Onel, E. H. Giannini, +6 Additional Authors

Journal Articles

BACKGROUND: This study aimed to assess long-term safety and developmental data on juvenile idiopathic arthritis (JIA) patients treated in routine clinical practice with celecoxib or nonselective nonsteroidal anti-inflammatory drugs (nsNSAIDs). METHODS: Children aged >/=2 to-negative polyarthritis, persistent or extended oligoarthritis, or systemic arthritis were enrolled into this prospective, observational, multicenter standard-of-care registry. Eligible patients were newly or recently prescribed (

Efficacy And Safety Of Abatacept In Lupus Nephritis A Twelve-Month, Randomized, Double-Blind Study, R. Furie, K. Nicholls, T. T. Cheng, F. Houssiau, R. Burgos-Vargas, S. L. Chen, J. L. Hillson, S. Meadows-Shropshire, M. Kinaszczuk, J. T. Merrill

Journal Articles

Objective. To compare the efficacy and safety of intravenous (IV) abatacept, a selective T cell costimulation modulator, versus placebo for the treatment of active class III or IV lupus nephritis, when used on a background of mycophenolate mofetil and glucocorticoids. Methods. This was a 12-month, randomized, phase II/III, multicenter, international, double-blind study. A total of 298 patients were treated in 1 of 3 IV treatment arms: placebo, abatacept at the standard weight-tiered dose (approximating 10 mg/kg), or abatacept at 30 mg/kg for 3 months, followed by the standard weight-tiered dose (abatacept 30/10). The primary end point, time to confirmed complete …

A Comparison Of The Malignancy Incidence Among Patients With Psoriatic Arthritis And Patients With Rheumatoid Arthritis In A Large Us Cohort, R. L. Gross, J. S. Schwartzman-Morris, M. Krathen, G. Reed, H. Chang, K. C. Saunders, M. C. Fisher, J. D. Greenberg, C. Putterman, A. Broder, +3 Additional Authors

Journal Articles

OBJECTIVE: To compare the incidence rates of malignancy among patients with psoriatic arthritis (PsA) and patients with rheumatoid arthritis (RA) in the Consortium of Rheumatology Researchers of North America (CORRONA) registry. METHODS: We analyzed 2,970 patients with PsA (7,133 patient-years of followup) and 19,260 patients with RA (53,864 patient-years of followup). Using a standardized adjudication process, we identified 40 confirmed malignancies in the patients with PsA and 307 confirmed malignancies in those with RA. Incidence rates were calculated per 100 patient-years. Incidence rate ratios were estimated, with adjustment for age, sex, disease duration, body mass index, disease activity, year of …