Rheumatology Commons^™

A Canadian Advanced Physiotherapist Practitioner Shared-Care Model In Pediatric Rheumatology Offers Safe And Quality Care In The Management Of Juvenile Idiopathic Arthritis-Comparing Key Performance Indicators With The Pr-Coin Registry., Julie Herrington, Patrick Clarkin, Jade Singleton, Karen Beattie, Sheetal S. Vora, Katelyn Banschbach, Catherine A. Bingham, Tania Cellucci, Danielle Fair, Mileka Gilbert, Beth Gottlieb, Julia G. Harris, Liane Heale, Tzielan Lee, Melissa L. Mannion, Edward J. Oberle, Nancy Pan, Jonathan Park, Mary Toth, Jennifer E. Weiss, Michelle Batthish, Pediatric Rheumatology Care And Outcomes Improvement Network

Manuscripts, Articles, Book Chapters and Other Papers

Background/Objectives: Canadian Advanced Physiotherapist Practitioner (APP) roles have existed for over 25 years in pediatric rheumatology. The APP can manage many common pediatric rheumatic conditions most often in Shared-Care Models (SCMs) with pediatric rheumatologists (PRs). The quality of care children receive in an APP SCM compared to traditional care is unknown. The Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) tracks quality measures as Key Performance Indicators (KPIs) in juvenile idiopathic arthritis (JIA) care. This study aimed to analyze the frequency of KPIs documented in a pediatric rheumatology APP SCM from a single center and compare to PR-COIN's performance …

Eular/Acr Classification Criteria For Paediatric Chronic Nonbacterial Osteomyelitis (Cno)., Yongdong Zhao, Melissa S. Oliver, Anja Schnabel, Eveline Y. Wu, Zhaoyi Wang, Achille Marino, Cassyanne L. Aguiar, Jonathan D. Akikusa, Ummusen Kaya Akca, Beverley Almeida, Simone Appenzeller, Erin Balay-Dustrude, Ozge Basaran, Matthew L. Basiaga, Yelda Bilginer, David A. Cabral, Martina Capponi, Nathan Donaldson, Bugra Han Egeli, Emily Fox, Antonella Insalaco, Ramesh S. Iyer, Annette F. Jansson, Inna Kostik, Mikhail Kostik, Leonard K. Kovalick, Katia Tomie Kozu, Sivia K. Lapidus, Tzielan C. Lee, Aleksander Lenert, Kamran Mahmood, Edoardo Marrani, Doaa Mosad Mosa, Ian Muse, Alexander Mushkin, Katherine D. Nowicki, Farzana Nuruzzaman, Karen Onel, Manuela Pardeo, Trang Sophia Pham, Lauren Potts, Athimalaipet V. Ramanan, Angelo Ravelli, Nathan D. Rogers, Andrew W. Grim, Micol Romano, Natalie Rosenwasser, Takashi Shawn Sato, Gabriele Simonini, Jennifer B. Soep, Sara M. Stern, Timmy Strauss, Angela Taneja Kohli, Alexander C. Theos, Lori B. Tucker, Leslie F. Vogel, Shima Yasin, Stephen C. Wong, Katerina Bouchalova, Alison M. Hendry, Kevin C. Cain, Hermann J. Girschick, Fatma Dedeoglu, Christian M. Hedrich, Ronald M. Laxer, Polly J. Ferguson, Raymond Naden, Seza Ozen

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: To develop and validate classification criteria for paediatric chronic nonbacterial osteomyelitis (CNO) jointly supported by the European Alliance of Associations for Rheumatology (EULAR) and the American College of Rheumatology (ACR).

METHODS: This international initiative had 4 phases: (1) candidate items were proposed in a survey of paediatric rheumatologists, (2) criteria definition and reduction by Delphi and nominal group technique exercises, (3) criteria weighting using multicriteria decision analysis, and (4) refinement of weights and threshold score in a development cohort of 441 patients and validation in another cohort of 514 patients.

RESULTS: The new EULAR/ACR classification criteria for CNO require …

American College Of Rheumatology Guidance Statements For Addressing Mental Health Concerns In Youth With Pediatric Rheumatologic Diseases., Natoshia R. Cunningham, Ashley N. Danguecan, Samantha L. Ely, Yaa Amponsah, Alaina Davis, Suzanne Edison, Julia G. Harris, Jordan T. Jones, Alana Goldstein-Leever, Alison Manning, Anne Mchugh, Crystal Mui, Ekemini Ogbu, Nikki Reitz, Martha Rodriguez, Natalie Rosenwasser, Alyse Tankanow, Erin Treemarcki, Katherine Winner, Tamar B. Rubinstein, Andrea M. Knight, Carra Mental Health Workgroup

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: Pediatric rheumatologic diseases (PRDs) are characterized by high rates of anxiety and depression known to impact health-related outcomes. We present guidance statements to assess and manage mental health concerns for youth with PRDs in pediatric rheumatology practice.

METHODS: Development of the guidance statements was initiated in 2019 and concluded in November 2023. It included (1) the formation of a task force (including pediatric rheumatologists, pediatric behavioral health providers, patients, and parents) led by two licensed pediatric psychologists and two board-certified pediatric rheumatologists, (2) iterative drafting of statements and rating of evidence based on the Oxford Centre for Evidence-Based Medicine …

Prevalence Of Psychiatric And Sleep Disorders And Their Impact On Quality Of Life In Children With Hypermobile Ehlers-Danlos Syndrome: An Observational Study., Amanda K. Hertel, Jordan T. Jones, Ashley Lytch, Emily Cramer, Ariana Schroeder, William R. Black

Manuscripts, Articles, Book Chapters and Other Papers

Hypermobile Ehlers-Danlos Syndrome (hEDS) is the most common connective tissue disorder. However, few studies exist on psychiatric and sleep disorders in pediatric patients with hEDS. This study aims to describe psychiatric and sleep disorders and evaluate their impact on health-related quality of life (HRQoL) in pediatric patients with hEDS. As part of a longitudinal study, a convenience sample of 123 pediatric patients with hEDS, were recruited at a hEDS multidisciplinary clinic in sequential order over a seven-month period. Patient-reported outcomes were completed (Patient Reported Outcomes Measurement Information System Pediatric Profile Version 2 [PROMIS], Generalized Anxiety Disorder-7 [GAD-7], Adolescent Sleep Wake …

Prevalence Of Pain And Effect Of Joint Strength On Perceived Physical Function In Pediatric Patients With Ehlers–Danlos Syndrome, Nathaniel Garcia, Ashley Lytch, Emily Cramer, William R. Black, Jordan T. Jones

Manuscripts, Articles, Book Chapters and Other Papers

Objective

To identify how pediatric patients with hypermobile-type Ehlers–Danlos syndrome (hEDS) present to physical therapy (PT) and better understand their musculoskeletal dysfunction.

Design

Patients were included in the study if they had a diagnosis of hEDS and at least 1 completed PT evaluation after diagnosis. A chart review was conducted to assess joint strength and pain characteristics at the initial PT assessment. Physical Function Mobility (PF-Mobility; 4 domains with 1-5 scale with higher score representative of better physical function) as part of the Patient-Reported Outcome Measurement Information System and pain intensity score (visual analog scale, 0-10 scale where a lower …

Patient And Parent Knowledge, Understanding, And Concerns After A New Diagnosis Of Ehlers Danlos Syndrome., Jordan T. Jones, Lora L. Black, William R. Black

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: After diagnosis of Ehlers Danlos Syndrome (EDS), it is unclear what information patients and parents need and understand about EDS. The objective of this study is to characterize patient and parent knowledge and concerns about EDS after a diagnosis of EDS is made to determine patient and parent concerns and identify barriers that cause discomfort with the diagnosis.6 METHODS: A convenience sample of patient and parent dyads were recruited after new diagnosis of EDS. Patients and parents completed questionnaires that assessed knowledge, comfort, and barriers of EDS before and after diagnosis, EDS education materials accessed, and additional clinical needs …

Influenza Knowledge And Barriers To Vaccination In Immunosuppressed Patients In The Pediatric Rheumatology Clinic., Julia G. Harris, Leslie Favier, Jordan T. Jones, Maria Ibarra, Michael J. Holland, Emily Fox, Kelly Jensen, Ashley K. Sherman, Ashley M. Cooper

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Most patients with a pediatric rheumatic disease are at increased risk of influenza due to immunosuppressive medication use. Despite initial quality improvement efforts, our influenza vaccination rate plateaued at 72%, which prompted a survey of patients and families to assess provider vaccine recommendations, influenza knowledge, and barriers to influenza vaccination.

METHODS: Patients on immunosuppressive medication or their parent were eligible to complete a survey between July 2019 and January 2020. Survey questions assessed demographics, rheumatology diagnosis, immunosuppressive medication(s), influenza vaccination recommendation, patient/parent influenza knowledge, and barriers to influenza vaccination. Influenza vaccination rates for immunosuppressed patients were acquired each influenza …

Key Data Elements For A Successful Pediatric Rheumatology Virtual Visit: A Survey Within The Pr-Coin Network., Y Ingrid Goh, Meghan E. Ryan, Shoghik Akoghlanian, Rajdeep Pooni, Julia G. Harris, Danielle R. Bullock, Sheetal S. Vora, Tzielan C. Lee, Shirley M L Tse, Fatima Barbar-Smiley

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: Juvenile idiopathic arthritis (JIA) is the most common childhood rheumatic disease which is commonly monitored by a combination of history, physical examination, bloodwork, and imaging. The COVID-19 pandemic prompted a rapid shift to telemedicine to ensure that patients continued to receive healthcare. The shift to telemedicine changed the methodology and ability of healthcare providers to monitor their patients' progress, as they were unable to perform direct hands-on assessments. The following survey sought to understand the impact of switching pediatric rheumatology healthcare delivery from in-person to telemedicine modality. Specifically, it sought to examine the rate of collection of critical data …

Cardiovascular, Autonomic Symptoms And Quality Of Life In Children With Hypermobile Ehlers-Danlos Syndrome., Amanda K. Hertel, William R. Black, Ashley Lytch, Emily Cramer, Lindsey Malloy-Walton, Jordan T. Jones

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: Hypermobile Ehlers-Danlos syndrome is a connective tissue disorder characterized by joint hypermobility and other systemic manifestations. Cardiovascular, autonomic symptoms and dysautonomia are frequently reported in adults with hypermobile Ehlers-Danlos syndrome and have been shown to have a negative impact on quality of life. However, there is scant literature on autonomic symptoms in pediatric patients with hypermobile Ehlers-Danlos syndrome. This study aims to characterize cardiovascular symptoms and diagnoses in pediatric patients with hypermobile Ehlers-Danlos syndrome and evaluate the impact of autonomic symptoms on quality of life.

METHODS: As part of a longitudinal study, a consecutive sample of 70 patients with …

Implementation Study Of The Carra Uveitis Consensus Treatment Plans: Feasibility For Clinical Practice And Applicability For Research., Margaret H. Chang, Fatima Barbar-Smiley, Shoghik Akoghlanian, Joanne Drew, Sheila T. Angeles-Han, Megan Quinlan-Waters, John F. Bohnsack, Ashley M. Cooper, Barbara Edelheit, Jennifer Twachtman-Bassett, Melissa A. Lerman, Kabita Nanda, C Egla Rabinovich, Mindy S. Lo, Carra Uveitis Workgroup And The Carra Registry Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Chronic anterior uveitis (CAU) carries a significant risk for eye complications and vision loss. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) introduced consensus treatment plans (CTPs) to standardize treatment for CAU and facilitate future comparative effectiveness studies. Two CTPs were developed to address: 1) initiation of methotrexate (MTX) in patients with CAU naïve to steroid-sparing therapy, and 2) initiation of a TNF inhibitor (TNFi) in patients with severe uveitis or uveitis refractory to MTX. We evaluated implementation of the uveitis CTPs using existing CARRA Registry infrastructure and assessed feasibility of the CTPs for comparative effectiveness research.

METHODS: This …

14-Month-Old Female With Anti-Mda5 Juvenile Dermatomyositis Complicated By Liver Disease: A Case Report., Mitch Kinkor, Sameena Hameed, Alexander Kats, Voytek Slowik, Emily Fox, Maria Ibarra

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Juvenile Dermatomyositis (JDM) is a rare disorder with subtypes associated with different myositis-specific antibodies (MSAs) including anti-MDA5. Hepatic involvement in JDM is rare and has not previously been documented in anti-MDA5 JDM. There is a lack of formal research on treatment protocols for anti-MDA5 JDM, though tofacitinib is a highly regarded emerging therapy.

CASE PRESENTATION: A previously healthy 14-month-old Hispanic female presented to a pediatric rheumatology clinic with eight months of worsening rash, weakness, periorbital edema, intermittent fevers, and weight loss. Her physical exam was notable for fever, thinning of hair, heliotrope rash, periorbital edema, violaceous macules on her …

Using A Collaborative Learning Health System Approach To Improve Disease Activity Outcomes In Children With Juvenile Idiopathic Arthritis In The Pediatric Rheumatology Care And Outcomes Improvement Network., Julia G. Harris, Catherine A. Bingham, Sheetal S. Vora, Cagri Yildirim-Toruner, Michelle Batthish, Danielle R. Bullock, Jon M. Burnham, Danielle C. Fair, Kerry Ferraro, Suhas Ganguli, Mileka Gilbert, Beth S. Gottlieb, Olha Halyabar, Melissa M. Hazen, Ronald M. Laxer, Tzielan C. Lee, Alice Liu, Daniel J. Lovell, Melissa L. Mannion, Edward J. Oberle, Nancy Pan, Michael Shishov, Jennifer E. Weiss, Esi M. Morgan

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: The Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) is a North American learning health network focused on improving outcomes of children with juvenile idiopathic arthritis (JIA). JIA is a chronic autoimmune disease that can lead to morbidity related to persistent joint and ocular inflammation. PR-COIN has a shared patient registry that tracks twenty quality measures including ten outcome measures of which six are related to disease activity. The network's global aim, set in 2021, was to increase the percent of patients with oligoarticular or polyarticular JIA that had an inactive or low disease activity state from 76% to …

Improving Pneumococcal Vaccination Rates In Immunosuppressed Pediatric Patients With Rheumatic Disease., Julia G. Harris, Jordan T. Jones, Leslie Favier, Emily Fox, Michael J. Holland, Amy Ivy, Cara M. Hoffart, Maria Ibarra, Ashley M. Cooper

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Patients with rheumatic diseases are at a high risk of invasive pneumococcal disease due to immunosuppression. We conducted a quality improvement project, and the first aim was to increase the percentage of patients with systemic lupus erythematosus and mixed connective tissue disease that is up to date on pneumococcal vaccinations from 9.6% to 80% within one year. Subsequently, the second aim was to increase the percentage of patients on immunosuppression with systemic lupus erythematosus, mixed connective tissue disease, juvenile dermatomyositis and systemic vasculitis that is up to date on pneumococcal vaccinations from 62.6% to 80% within one year.

METHODS: …

Achieving Reliable Patient Reported Outcomes Collection To Measure Health Care Improvement In A Learning Health Network: Lessons From Pediatric Rheumatology Care And Outcomes Improvement Network., Nancy Pan, Esi M. Morgan, Meghan Ryan, Beth Gottlieb, Julia G. Harris, Tzielan Lee, Y Ingrid Goh

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: Data from the Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) registry suggests that reliable collection of patient-reported outcomes (PROs) varies across sites. The objective of this study was to better understand the practices of collecting PROs at PR-COIN sites.

METHODS: A REDCap survey was sent to the lead representative for each PR-COIN site. Registry data were analyzed to better understand the completion rates of PROs. Interviews of physician leaders of high performing sites were conducted by videoconference, audiotranscribed and themes were summarized. Quantitative data were analyzed using descriptive statistics and qualitative data were thematically analyzed.

RESULTS: All 23 …

Barriers To The Diagnosis, Care, And Management Of Pediatric Patients With Ehlers-Danlos Syndrome In The United States: A Qualitative Analysis., William R. Black, Lora L. Black, Jordan T. Jones

Manuscripts, Articles, Book Chapters and Other Papers

Objectives: Ehlers-Danlos Syndromes (EDS) are a family of heritable connective tissue diseases. Primary practitioners are capable of diagnosing and managing EDS; however, few are knowledgeable and comfortable enough to see patients with EDS, resulting in delays in diagnosis and care. This study explores the barriers physicians experience with diagnosing, managing, and caring for patients with EDS, and potential resolutions to those barriers. Methods: As part of a larger online study, providers (n = 107) in the United States were asked to specify "What information would improve (their) comfort" in diagnosing, caring for, and managing EDS via open-ended questions. Results …

Gender Dysphoria In Adolescents With Ehlers-Danlos Syndrome., Jordan T. Jones, William R. Black, Christine Moser, Eric T. Rush, Lindsey Malloy-Walton

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: Ehlers-Danlos Syndrome represents a family of heritable connective tissue disorders that include joint hypermobility, tissue fragility, and skin hyperextensibility. Ehlers-Danlos Syndrome presents with clinical sequela across multiple body systems that require multidisciplinary care. Little is known about adolescents with Ehlers-Danlos Syndrome who are transgender and gender diverse. To date, there have been no reports of transgender and gender diverse youth in pediatric patients with Ehlers-Danlos Syndrome. The objective of this study was to characterize transgender and gender diverse adolescents with Ehlers-Danlos Syndrome seen in a pediatric multidisciplinary specialty clinic.

METHODS: A retrospective chart review was performed and it was …

Down Syndrome-Associated Arthritis (Da): Diagnostic And Management Challenges., Jordan T. Jones, Jacqueline Kitchen, Nasreen Talib

Manuscripts, Articles, Book Chapters and Other Papers

Down syndrome (DS) is one of the most common birth defects in the United States, the most common genomic disorder of intellectual disability, and results from trisomy 21. This chromosome disorder causes an extensive, heterogenous phenotype that results in a broad presentation of symptoms that includes atlantoaxial instability, congenital heart defects, muscle hypotonia, hypothyroidism, hematologic disorders, recurrent infections, and autoimmune diseases. The autoimmune diseases are caused by immune system dysregulation that results in increased pro-inflammatory cytokines, along with other innate and adaptive immune system dysregulation. This is the likely cause of the increased risk of inflammatory arthritis or Down syndrome-associated …

Knowledge About Juvenile Idiopathic Arthritis-Associated Uveitis: More Frequent Reminders Are Associated With Higher Patient And Family Uveitis Knowledge., Ashley M. Cooper, Elaine R. Flanagan, Tova Ronis, Baruch Goldberg, Ashley K. Sherman, Chelsey Smith, Gary N. Holland, Carra Uveitis Workgroup

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Chronic anterior uveitis is a sight-threatening complication of juvenile idiopathic arthritis (JIA) and a primary contributor to long-term morbidity in people with JIA. Levels of knowledge about uveitis among JIA patients and their parents are unknown. A survey of JIA patients and parents was conducted to assess knowledge about uveitis complications and recommended screening.

METHODS: A survey was developed consisting of six demographic questions, six arthritis/uveitis history questions, and nine uveitis knowledge questions. The survey was administered to JIA patients age 14 and older and parents of patients with JIA at three pediatric rheumatology practices and online through the …

Brief Musculoskeletal Screen And Patient Education For Down Syndrome-Associated Arthritis., Jordan T. Jones, Chelsey Smith, Nasreen Talib

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Identifying The Content And Context Of Pain Within Paediatric Rheumatology Healthcare Professional Curricula In The Uk: A Summative Content Analysis., Rebecca Rachael Lee, Janet E. Mcdonagh, Mark Connelly, Sarah Peters, Lis Cordingley

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The curriculum for professionals working in paediatric rheumatology should include pain but it is unclear to what extent this currently occurs. The aim of this study was to identify pain-related curriculum content and the context in which pain is presented in educational and training documentation for healthcare professionals in this clinical speciality.

METHODS: Core curricula documents from UK based professional organisations were identified in partnership with healthcare professionals. Documents were analysed using a summative content analysis approach. Key pain terms were quantified and weighted frequencies were used to explore narrative pain themes. Latent content was interpreted qualitatively to explore …

Atypical Presentation Of Systemic Arthritis In A Toddler With Down Syndrome., Emily Worley, Weijie Li, Jordan T. Jones

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Systemic juvenile idiopathic arthritis (sJIA) is a chronic, inflammatory disease of childhood, which is characterized by the combination of arthritis, serositis, daily, high-spiking fevers, and evanescent macular rash and can present with the life-threatening complication of macrophage activation syndrome (MAS). Children with Down syndrome (DS) have complex medical challenges related to abnormalities in their immune system, which can cause a broad spectrum of disease manifestations, which can occur atypically. Children with DS are at increased risk for arthritis and interstitial lung disease (ILD) associated with sJIA that has high mortality. This case report outlines an atypical presentation of sJIA in …

Assessment Of Down Syndrome-Associated Arthritis: A Survey Of Down Syndrome Clinic Providers., Jordan T. Jones, Chelsey Smith, Nasreen Talib

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Exploring Pediatric Tele-Rheumatology Practices During Covid-19: A Survey Of The Prcoin Network., Y Ingrid Goh, Danielle R. Bullock, Janalee Taylor, Rajdeep Pooni, Tzielan C. Lee, Sheetal S. Vora, Cagri Yildirim-Toruner, Esi M. Morgan, Nancy Pan, Julia G. Harris, Andrew Warmin, Kendra Wiegand, Jon M. Burnham, Fatima Barbar-Smiley

Manuscripts, Articles, Book Chapters and Other Papers

Healthcare providers were rapidly forced to modify the way they practiced medicine during the coronavirus disease 2019 (COVID-19) pandemic. Many providers transitioned from seeing their patients in person to virtually using telemedicine platforms with limited training and experience using this medium. In pediatric rheumatology, this was further complicated as musculoskeletal exams typically require hands-on assessment of patients. The objective of this study was to examine the adoption of telemedicine into pediatric rheumatology practices, to assess its benefits and challenges, and to gather opinions on its continued use. A survey was sent to the lead representatives of each Pediatric Rheumatology Care …

Engaging Patients And Parents To Improve Mental Health Intervention For Youth With Rheumatological Disease., Oluwatunmise A. Fawole, Michelle V. Reed, Julia G. Harris, Aimee Hersh, Martha Rodriguez, Karen Onel, Erica Lawson, Tamar Rubinstein, Kaveh Ardalan, Esi Morgan, Anne Paul, Judy Barlin, R Paola Daly, Mitali Dave, Shannon Malloy, Shari Hume, Suzanne Schrandt, Laura Marrow, Angela Chapson, Donna Napoli, Michael Napoli, Miranda Moyer, Vincent Delgaizo, Ashley Danguecan, Emily Von Scheven, Andrea Knight, Carra Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Mental health disorders are common in youth with rheumatological disease yet optimal intervention strategies are understudied in this population. We examined patient and parent perspectives on mental health intervention for youth with rheumatological disease.

METHODS: We conducted a mixed methods cross-sectional study, via anonymous online survey, developed by researchers together with patient/parent partners, to quantitatively and qualitatively examine youth experiences with mental health services and resources in North America. Patients ages 14-24 years with juvenile idiopathic arthritis, juvenile dermatomyositis, or systemic lupus erythematous, and parents of patients ages 8-24 with these diseases were eligible (not required to participate in …

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 …

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 …

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 …