Abstract Number: 367 • 2017 ACR/ARHP Annual Meeting
Interleukin-1 Receptor Antagonist Is a Potential Treatment for Undifferentiated Autoinflammatory Syndromes
Background/Purpose: The autoinflammatory diseases (AIDs) are a group of disorders of the innate immune system characterized by seemingly unprovoked inflammation1. A variety of genetic alterations…Abstract Number: 368 • 2017 ACR/ARHP Annual Meeting
Evaluation of Efficacy and Safety of Opocalcium Colchicine, and Anti-IL1 Treatment in Childhood Colchicine-Resistant Familial Mediterranean Fever
Background/Purpose: The colchicine-resistant FMF (crFMF) is defined as 6 or more polyserositis attacks in the last year despite the regular usage of colchicine in the…Abstract Number: 369 • 2017 ACR/ARHP Annual Meeting
Hepatitis A Virus Vaccination in Juvenile-Onset Systemic Lupus Erythematosus
Background/Purpose: Various infections play significant roles in flares of systemic lupus erythematosus (SLE). Hepatitis A virus is one of these infectious agents that has high…Abstract Number: 370 • 2017 ACR/ARHP Annual Meeting
Validation of 2016 ACR/EULAR Classification Criteria of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis in Japanese Patients
Background/Purpose: To validate the 2016 ACR/EULAR classification criteria of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (s-JIA) in Japanese patients. Methods: A combination…Abstract Number: 371 • 2017 ACR/ARHP Annual Meeting
Longer Term Outcomes of Chronic Relapsing Multifocal Osteomyelitis in a UK Tertiary Adolescent and Young Adult Rheumatology Centre
Background/Purpose: Chronic relapsing multifocal osteomyelitis (CRMO) is a rare autoinflammatory bone condition presenting primarily in children & adolescents. It characteristically affects the epiphysis & metaphysis…Abstract Number: 372 • 2017 ACR/ARHP Annual Meeting
Phenotype of Chronic Recurrent Multifocal Osteomyelitis in a Tertiary Referral Centre: The Great Ormond Street Hospital Experience
Background/Purpose: Recurrent Multifocal Osteomyelitis (CRMO) is an aseptic inflammatory bone disease that typically affects the metaphases of the long bones. It affects children, adolescents and…Abstract Number: 373 • 2017 ACR/ARHP Annual Meeting
a Retrospective Study of Clinical Factors Influencing the Development of Overlapping Disease Features in Pediatric Patients with Chronic Recurrent Multifocal Osteomyelitis (CRMO) and Spondyloarthropathies (SpA)
Background/Purpose: Some studies have suggested that chronic recurrent multifocal osteomyelitis (CRMO) and spondyloarthropathies (SpA) fall on a spectrum of disease, as they have been noted…Abstract Number: 374 • 2017 ACR/ARHP Annual Meeting
A Pilot Study of Infrared Thermal Imaging to Detect Active Bone Lesions in Children with Chronic Nonbacterial Osteomyelitis
Background/Purpose: Chronic nonbacterial osteomyelitis (CNO) is an autoinflammatory bone disease. For detection of active bone lesions, bone scintigraphy and magnetic resonance imaging (MRI) are much…Abstract Number: 375 • 2017 ACR/ARHP Annual Meeting
Controlled Discontinuation of Colchicine Therapy in Familial Mediterranean Fever Patients with Single MEFV Mutation
Background/Purpose: Familial Mediterranean fever (FMF) traditionally has been considered an autosomal recessive disease; however, the diagnosis remains predominantly clinical, since mutations cannot always be identified…Abstract Number: 376 • 2017 ACR/ARHP Annual Meeting
Improvement of Disease Activity in Patients with Colchicine-Resistant FMF, Hids/Mkd and TRAPS Assessed By Autoinflammatory Disease Activity Index (AIDAI): Results from a Randomized Phase III Trial
Background/Purpose: AIDAI is a novel, validated tool for the assessment of disease activity across a wide spectrum of autoinflammatory diseases including recurrent fever syndromes such…Abstract Number: 377 • 2017 ACR/ARHP Annual Meeting
Novel Insights into Periodic Fever Syndromes
Background/Purpose: The Periodic Fever Syndromes (PFS) are a rapidly expanding group of disorders primarily of the innate immune system that often affect the inflammasome. In…Abstract Number: 378 • 2017 ACR/ARHP Annual Meeting
Musculoskeletal Features in Copa Syndrome
Background/Purpose: COPA syndrome is a newly discovered primary immunodeficiency resulting in immune dysregulation showing autosomal dominant inheritance with incomplete penetrance. Its name is derived from…Abstract Number: 379 • 2017 ACR/ARHP Annual Meeting
H Syndrome: Five New Cases from the United States with Novel Features and Responses to Therapy
Background/Purpose: H Syndrome is an autosomal recessive disorder characterized by cutaneous hyperpigmentation, hypertrichosis, and induration with numerous systemic manifestations. The syndrome is caused by mutations…Abstract Number: 380 • 2017 ACR/ARHP Annual Meeting
Treatment Outcomes of Down Syndrome Arthropathy
Background/Purpose: Crude prevalence estimates indicate Down syndrome arthropathy (DA) is 3-8 times more common than juvenile idiopathic arthritis (JIA), however, DA is still largely under…Abstract Number: 381 • 2017 ACR/ARHP Annual Meeting
Down’s Arthritis (DA) – Clinical and Radiological Features of Arthritis in Children with Trisomy 21
Background/Purpose: Down's Arthritis (DA) was first reported in the literature in 1984. Crude estimates suggest higher incidence and prevalence rates of DA compared with Juvenile…