Abstract Number: 2383 • 2016 ACR/ARHP Annual Meeting
Analysis of the Use of Anticoagulants and Antiplatelet Agents in Strokes Caused By the Deficiency of Adenosine Deaminase 2
Background/Purpose: Deficiency of adenosine deaminase 2 (DADA2) is a recessive genetic condition in which children develop recurrent strokes, intermittent fevers, elevated acute-phase reactants, livedoid rash,…Abstract Number: 2384 • 2016 ACR/ARHP Annual Meeting
Variable Clinical Phenotypes and Relation of Interferon Signature with Disease Activity in ADA 2 Deficiency
Background/Purpose: The deficiency of adenosindeaminase 2 (DADA2) is a recently described autosomal recessive autoinflammatory disease, caused by mutations of CECR1 and characterized by early onset…Abstract Number: 2385 • 2016 ACR/ARHP Annual Meeting
Validation of Diary Score for the Assessment of Disease Activity in Candle (Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature) and SAVI (STING associated vasculopathy with onset in Infancy) Patients
Background/Purpose: CANDLE and SAVI are two rare autoinflammatory interferonopathies without validated outcome measures to assess disease activity; however, daily diaries of prominent disease symptoms have…Abstract Number: 2386 • 2016 ACR/ARHP Annual Meeting
Role of Pentraxin 3 in Patients with Juvenile Scleroderma
Background/Purpose: Juvenile scleroderma (JS) is a rarely seen chronic connective tissue disorder. According to organ involvement, the disease is divided into two main forms: systemic…Abstract Number: 2387 • 2016 ACR/ARHP Annual Meeting
Response to Pamidronate Treatment Assessed By Whole Body Magnetic Resonance Imaging in Pediatric Chronic Non-Bacterial Osteomyelitis
Background/Purpose: Pamidronate (PAM) may be effective in diminishing pain and permanent bone deformities in chronic non-bacterial osteomyelitis (CNO). Whole body magnetic resonance imaging (WB MRI)…Abstract Number: 2388 • 2016 ACR/ARHP Annual Meeting
Clinical Characterization and Outcomes of Pediatric Chronic Nonbacterial Osteomyelitis
Background/Purpose: Chronic Nonbacterial Osteomyelitis (CNO) is a rare condition of largely unknown etiology with variable clinical and radiological features. It is an aseptic auto-inflammatory bone…Abstract Number: 2389 • 2016 ACR/ARHP Annual Meeting
Is There a Difference in the Presentation of Diffuse and Limited Subtype in Childhood? Results from the Juvenile Scleroderma Inception Cohort
Background/Purpose: Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Several adult publications looked at the differences between limited (ljSSc) and diffuse subtype (djSSc). There…Abstract Number: 2390 • 2016 ACR/ARHP Annual Meeting
Proposal of Assessment of the Activity of Juvenile Localised Scleroderma. Results of the Consensus Meeting in Hamburg, Germany December 2015
Background/Purpose: Juvenile Localised Scleroderma (JLS) is an orphan disease which is complicated by difficulties in robust measurement of disease activity. Several outcome measures to assess…Abstract Number: 2391 • 2016 ACR/ARHP Annual Meeting
Update on the Juvenile Systemic Sclerosis Inception Cohort Project. Characteristics of the First 80 Patients at First Assessment
Background/Purpose: Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Currently just retrospective data exist regarding evolvement of organ involvement. In the retrospective studies assessment…Abstract Number: 2392 • 2016 ACR/ARHP Annual Meeting
Establishing Quality of Life Content Domains in Pediatric Localized Scleroderma
Background/Purpose: Localized scleroderma (LS) can affect patients' physical function and psychosocial well-being, but, published studies of the impact of pediatric LS on health-related quality of…Abstract Number: 2393 • 2016 ACR/ARHP Annual Meeting
Medication Use in the Juvenile Systemic Sclerosis Inception Cohort. ARE There Differences in the Diffuse and Limited Subset Patients?
Background/Purpose: Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Currently there is no data regarding use of medication in jSSc patients. Our project is…Abstract Number: 2394 • 2016 ACR/ARHP Annual Meeting
Patient and Parent Global Assessments of Disease Impact in Pediatric Localized Scleroderma: Correlates of Patient Reported Health-Related Quality of Life and Parent Reported Family Impact Domains
Background/Purpose: Pediatric localized sclerodermaÕs (LS) effects on patient/family well-being are underexplored. Contributions of HRQoL domains and family dynamics to LSÕs impact on patients has not…Abstract Number: 2395 • 2016 ACR/ARHP Annual Meeting
The Performance of the Kobayashi and Egami Scores in Detecting IVIG Resistance in Kawasaki Disease in a Single Centre Canadian Cohort Treated with IVIG and Low Dose Aspirin
Background/Purpose: Patients with Kawasaki disease (KD) resistant to IVIG remain a challenge. IVIG resistance has been recognised as a risk factor for the development of…Abstract Number: 2396 • 2016 ACR/ARHP Annual Meeting
Initiation of Dialysis in Pediatric ANCA-Associated Vasculitis
Background/Purpose: ANCA-associated vasculitis (AAV) is a group of rare systemic vasculitidies with significant morbidity and mortality. There have been no comparative effectiveness studies of AAV…Abstract Number: 2397 • 2016 ACR/ARHP Annual Meeting
Developing a Role for 18f-Flurodeoxyglucose PET/MRI in Pediatric Takayasu Arteritis
Background/Purpose: Assessing disease activity is often challenging in pediatric Takayasu Arteritis (TA). TA inflammation may be clinically silent and not always reflected on laboratory or…
ACR Meeting Abstracts