Abstracts tagged "Autoinflammatory Disease"

Abstract Number: 2750 • 2013 ACR/ARHP Annual Meeting
Long-Term Efficacy and Safety Of IL1 Receptor Antagonist In Schnitzler’s Syndrome : A French Multicenter Study
Antoine Néel¹, Agathe Masseau¹, Sebastien Barbarot², Benoit Henry³, Pierre-Jean Weiller⁴, Olivier Decaux⁵, Xavier Kyndt⁶, Xavier Puechal⁷, Arnaud Hot⁸, Pierre Pottier¹, Amar Smail⁹, David Launay¹⁰, Jean-Marie Berthelot¹¹, Eric Hachulla¹², Leonardo Astudillo¹³, Pierre-Yves Hatron¹², Laurent Sailler¹³, Aurelien Lorleac'h¹⁴, Achille Aouba¹⁵, Bérangère Cador¹⁶, Renato Fior¹⁷, Robin Dhote¹⁸, Fabrice Bonnet¹⁹, Jean-Dominique de Korwin²⁰ and Mohamed Hamidou¹, ¹Internal Medicine Department, Nantes University Hospital, Nantes, France, ²Dermatology, Nantes University Hospital, Nantes, France, ³Infectious and Tropical Diseases, Pitié-Salpêtrière University Hospital, Paris, France, ⁴Internal Medicine Department, La Timone University Hospital, Marseille, France, ⁵Department of Internal Medicine, Rennes University Hospital, Rennes, France, ⁶Department of Nephrology and Internal Medicine, CH, Valenciennes, Valenciennes, France, ⁷Internal Medicine, Hôpital Cochin, Paris, France, ⁸Internal Medicine, Edouard Herriot University Hospital, Lyon, France, ⁹Internal Medicine Department, CHU Amiens Nord, Amiens, France, ¹⁰Internal Medicine, Claude Huriez University Hospital, Lille, France, ¹¹Rheumatology Unit, Nantes University Hospital, Nantes, France, ¹²Department of Internal Medicine, Claude Huriez University Hospital, Lille, France, ¹³Department of Internal Medicine, Toulouse University Hospital, Toulouse, France, ¹⁴Internal Medicine Department, Lorient Hospital, Lorient, France, ¹⁵Hematology, Necker University Hospital, Paris, France, ¹⁶Internal Medicine Department, Rennes University Hospital, Rennes, France, ¹⁷Internal Medicine Department, Antoine Béclère University Hospital, Clamart, France, ¹⁸Internal Medicine, Avicenne University Hospital, Bobigny, France, ¹⁹Internal Medicine, Bordeaux University Hospital, Bordeaux, France, ²⁰Internal Medicine Department, Nancy University Hospital, Nancy, France
Background/Purpose: Schnitzler's syndrome is a rare late onset auto-inflammatory disease which associates a chronic/recurrent urticarial skin rash, a monoclonal gammopathy (mostly IgM kappa), and a…
Abstract Number: 2613 • 2013 ACR/ARHP Annual Meeting
NF-κB Pathway Is Depleted In Phagocytes From Behçet´s Disease Patients Secondarily To Constitutive Phosphorylation Of The p65 Subunit
Sandro F. Perazzio^1,2, Paulo Vitor Soeiro Pereira³, Alexandre W.S. Souza^4,5, Antonio Condino-Neto³ and Luis Eduardo C. Andrade⁶, ¹Rheumatology, Escola Paulista de Medicina - Universidade Federal de São Paulo, Sao Paulo, Brazil, ²Fleury Medicine and Health, Sao Paulo, Brazil, ³Immunology, ICB IV - Universidade de São Paulo, São Paulo, Brazil, ⁴University Medical Center Groningen, Groningen, Netherlands, ⁵Rheumatology Div/Dept of Med, Escola Paulista de Medicina - Universidade Federal de São Paulo, Sao Paulo, Brazil, ⁶Rheumatology Division, Escola Paulista de Medicina - Universidade Federal de São Paulo, Sao Paulo, Brazil
Background/Purpose: : Increased neutrophil activation has been previously shown in Behçet’s disease (BD) patients and it is unclear whether neutrophil activation occurs constitutively or if…
Abstract Number: 2614 • 2013 ACR/ARHP Annual Meeting
Plasma Of Active Behçet´s Disease Increases Oxidative Metabolism Profile Of Normal and Patients Phagocytes
Sandro F. Perazzio^1,2, Paulo Vitor Soeiro Pereira³, Alexandre W.S. Souza^4,5, Antonio Condino-Neto³ and Luis Eduardo C. Andrade⁶, ¹Rheumatology, Escola Paulista de Medicina - Universidade Federal de São Paulo, Sao Paulo, Brazil, ²Fleury Medicine and Health, Sao Paulo, Brazil, ³Immunology, ICB IV - Universidade de São Paulo, São Paulo, Brazil, ⁴University Medical Center Groningen, Groningen, Netherlands, ⁵Rheumatology Div/Dept of Med, Escola Paulista de Medicina - Universidade Federal de São Paulo, Sao Paulo, Brazil, ⁶Rheumatology Division, Escola Paulista de Medicina - Universidade Federal de São Paulo, Sao Paulo, Brazil
Background/Purpose: Behçet’s disease (BD) exhibits several features suggestive of neutrophil hiperactivity. It is unclear whether neutrophil activation occurs constitutively or if it is secondary to…
Abstract Number: 2173 • 2013 ACR/ARHP Annual Meeting
The Interleukin-1 Receptor Antagonist Anakinra Is Effective In The Treatment Of Undifferentiated Periodic Fever Syndromes
Daniella M. Schwartz¹, Runsheng Wang², Karyl Barron³, Daniel L. Kastner⁴ and Amanda K. Ombrello⁵, ¹Rheumatology fellowship and training branch, National Institutes of Health, National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, ²NIH/NIAMS, Rheumatology fellowship and training branch, Bethesda, MD, ³Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, ⁴Inflammatory Disease Section, National Human Genome Research Institute, Bethesda, MD, ⁵National Human Genome Research Institute,National Institute of Health, Bethesda, MD
Background/Purpose: The autoinflammatory diseases are a group of disorders associated with dysregulation of the innate immune system. They are characterized by recurrent episodes of fever…
Abstract Number: 1781 • 2013 ACR/ARHP Annual Meeting
Clinical Variation In Children With Mutations In MEFV
Karyl Barron¹, Amanda K. Ombrello², Ivona Aksentijevich³, Anne Jones³ and Daniel L. Kastner³, ¹Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, ²National Human Genome Research Institute,National Institute of Health, Bethesda, MD, ³Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD
Background/Purpose: As a referral clinic for children with periodic fevers, we see a wide variety of clinical presentations. While genetic testing is usually helpful in…
Abstract Number: 1783 • 2013 ACR/ARHP Annual Meeting
Canakinumab Treatment In Active Hyper-IgD With Periodic Fever Syndrome
Juan Ignacio Aróstegui¹, Inmaculada Calvo², Jordi Anton³, Angel Robles⁴, Jordi Yagüe¹, Ruth Viana⁵, Lillian Tseng⁶ and Ken Abrams⁶, ¹Immunology Department, Hospital Clinic Barcelona, Barcelona, Spain, ²Hospital La Fe, Valencia, Spain, ³Rheumatology, Hospital Sant Joan de Deu, Barcelona, Spain, ⁴Hospital La Paz, Madrid, Spain, ⁵Medical Department, Novartis Farmacéutica, S.A., Barcelona, Spain, ⁶Novartis Pharmaceuticals Corporation, East Hanover, NJ
Background/Purpose: Hyper-IgD with periodic fever syndrome (HIDS) is a recessively inherited autoinflammatory disease due to biallelic MVK mutations. It is characterized by 4-6 days-long acute…

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