Abstract Number: 1243 • 2019 ACR/ARP Annual Meeting
Anakinra Treatment in Recurrent Pericarditis: Single Center Experience
Background/Purpose: Recurrent pericarditis (RP), however the etiology is unknown in the majority, may be observed in autoinflammatory diseases such as familial Mediterranean fever (FMF) and…Abstract Number: 1244 • 2019 ACR/ARP Annual Meeting
Tocilizumab – An Effective Rescue Therapy for Refractory Unclassified Autoinflammatory Diseases in Children
Background/Purpose: Evidence based treatment options for children and adults with unclassified autoinflammatory diseases (AID) are limited. Frequently, IL-1-inhibition is primarily tried to control the severe…Abstract Number: 1245 • 2019 ACR/ARP Annual Meeting
Efficacy of Canakinumab Treatment in Adult-onset Still’s Disease
Background/Purpose: Adult-onset Still’s disease (AOSD) is a rare auto-inflammatory condition characterized by fever, arthritis, skin rash, and multi-organ inflammation. The pathogenesis of AOSD is centrally…Abstract Number: 1246 • 2019 ACR/ARP Annual Meeting
Using PROMIS Data to Assess Activity of Inflammatory Eye Disease
Background/Purpose: Diagnosing and managing patients with inflammatory eye disease (IED) requires close interdisciplinary care with rheumatologists adjusting systemic, potentially toxic, therapies based on exam findings…Abstract Number: 1247 • 2019 ACR/ARP Annual Meeting
Adult-Onset Still’s Disease and Spondyloarthritis: Overlapping Syndrome or Incidental Association? A Series of 5 Cases
Background/Purpose: Some forms of spondyloarthritis (SpA) such as SAPHO syndrome are classified as auto-inflammatory disorders. Association with adult onset Still’s disease (AoSD) has never been…Abstract Number: 1248 • 2019 ACR/ARP Annual Meeting
Clinical Manifestations and Management of US Patients with SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis) Syndrome, a Retrospective Study
Background/Purpose: The SAPHO syndrome is a rare inflammatory disorder of bones, joints and skin first coined by Chamot in 1987 characterized by synovitis, acne, pustulosis,…Abstract Number: 1249 • 2019 ACR/ARP Annual Meeting
Pelvic Congestion Syndrome, an Uncommon Cause of Osteoarticular Pain
Background/Purpose: Background: The pelvic congestion syndrome (PCS) is an under and often misdiagnosed entity that appears more frequently in premenopausal age and multiparous women. The…Abstract Number: 1250 • 2019 ACR/ARP Annual Meeting
Can Patient-Reported Outcomes and Disease Activity Scores Predict Patient Acceptable Symptom State in Adult-Onset Still’s Disease?
Background/Purpose: Adult-onset Still’s disease (AOSD) is a rare, chronic and auto-inflammatory disorder with multisystemic involvement. Patient Acceptable Symptom State (PASS) is the highest level of…Abstract Number: 1251 • 2019 ACR/ARP Annual Meeting
The Impact of Aging on Familial Mediterranean Fever Patients
Background/Purpose: Familial Mediterranean Fever (FMF) is a monogenic autoinflammatory disorder with innate immune activation with an onset before age 20 in approximately 90% of the…Abstract Number: 1252 • 2019 ACR/ARP Annual Meeting
Pattern of Arthropathy in Patients with Cystic Fibrosis
Background/Purpose: Arthropathy is a rare but debilitating manifestation of cystic fibrosis (CF) that has no formal definition. This study attempts to characterize the spectrum of…Abstract Number: 1253 • 2019 ACR/ARP Annual Meeting
Novel Nonsense Variant and Entire Deletion of TNFAIP3 Cause Haploinsufficiency of A20 Clinically Distinct from Behçet’s Disease
Background/Purpose: Haploinsufficiency of A20 (HA20) is caused by loss-of-function TNFAIP3 variants. Phenotypic and genetic features of HA20 remain uncertain; therefore, clinical distinction between HA20 and…Abstract Number: 1254 • 2019 ACR/ARP Annual Meeting
A Retrospective Medical Chart Review of Patients with Periodic Fever Syndromes Initiating Canakinumab in the United States
Background/Purpose: Periodic fever syndromes (PFS) is a group of rare autoinflammatory diseases that includes cryopyrin-associated periodic syndromes (CAPS), hyperimmunoglobulin D syndrome/mevalonate kinase deficiency (HIDS/MKD), TNF…Abstract Number: 1255 • 2019 ACR/ARP Annual Meeting
Is Exon 2 Associated with FMF or a New Disease?
Background/Purpose: In Familial Mediterranean Fever (FMF), patients having exon 10 mutations, specifically M694V, M680I, and V726A are more common and their disease profiles tend to…Abstract Number: 1256 • 2019 ACR/ARP Annual Meeting
Comparison of FMF Patients with Age of Onset Before 20 versus 40 Years and Over
Background/Purpose: Familial Mediterranean fever (FMF) is a disease with an onset before 20 years of age in 90% of the patients. However late onset FMF defined…Abstract Number: 1257 • 2019 ACR/ARP Annual Meeting
Recommendation on Colchicine Dosing and Definition of Colchicine Resistance/Intolerance in the Management of Familial Mediterranean Fever
Background/Purpose: Familial Mediterranean Fever (FMF) is the most common monogenic autoinflammatory disease and colchicine is the drug of choice for its treatment. However, about 5-10%…
