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  • Abstract Number: 256 • 2016 ACR/ARHP Annual Meeting

    Muckle-Wells Syndrome in Chinese Adult Patients

    Di Wu, Min Shen and Xiaofeng Zeng, Rheumatology, Peking Union Medical College Hospital, Beijing, China

    Background/Purpose: Muckle-Wells syndrome (MWS) is a rare autoinflammatory disease, which is categorized as one of the three cryopyrin-associated periodic syndromes (CAPS). MWS is characterized by…
  • Abstract Number: 257 • 2016 ACR/ARHP Annual Meeting

    CNS Manifestations of Patients with Muckle-Wells Syndrome

    sara Sebnem KILIC1, sukru cekic2 and Juan Aróstegui3, 1Pedaitric Rheumatology, Prof Dr, Bursa, Turkey, 2Dr, Bursa, Turkey, 3Prof, Barcelona, Spain

    Background/Purpose: CAPS is a rare autoinflammatory disease associated with mutations in the NLRP3 gene that result in overactivation of the inflammasome, increased secretion of IL-1beta…
  • Abstract Number: 258 • 2016 ACR/ARHP Annual Meeting

    Unmet Psychosocial Needs in Patients with CAPS

    Jasmin B. Kuemmerle-Deschner1, Gabi Erbis1, Tetiana Sergiichuk1, Sandra Hansmann1, Iris Haug1 and Susanne Benseler2, 1Pediatrics, University Hospital Tuebingen, Tuebingen, Germany, 2Pediatrics, University of Calgary, Calgary, AB, Canada

    Background/Purpose: Cryopyrin-associated periodic syndrome (CAPS) is a rare autoinflammatory disease. Generalized manifestations like recurrent fever and fatigue and organ disease like reduced vision, hearing loss,…
  • Abstract Number: 259 • 2016 ACR/ARHP Annual Meeting

    Rituximab in Refractory Cardiac Sarcoidosis – Single Center Experience

    Megan Krause1, Leslie T. Cooper Jr.2, Panithaya Chareonthaitawee3 and Shreyasee Amin1, 1Rheumatology, Mayo Clinic, Rochester, MN, 2Cardiology, Mayo Clinic, Jacksonville, FL, 3Cardiology, Mayo Clinic, Rochester, MN

    Background/Purpose:   Cardiac sarcoidosis is a life threatening condition for which there is limited data to guide optimal steroid-sparing agents. B-cells have been reported to…
  • Abstract Number: 260 • 2016 ACR/ARHP Annual Meeting

    Risk of Cardiovascular Disease Among Patients with Sarcoidosis: A Population-Based Retrospective Cohort Study

    Patompong Ungprasert1, Cynthia S. Crowson2 and Eric L. Matteson1, 1Rheumatology, Mayo Clinic, Rochester, MN, 2Health Sciences Research, Mayo Clinic, Rochester, MN

    Risk of Cardiovascular Disease Among Patients With Sarcoidosis: A Population-Based Retrospective Cohort Study Background/Purpose: Several epidemiologic studies have demonstrated an increased incidence of atherosclerotic cardiovascular…
  • Abstract Number: 261 • 2016 ACR/ARHP Annual Meeting

    Sarcoidosis Is Associated with Increased Risk of Venous Thromboembolism: A Population-Based Study

    Patompong Ungprasert1, Cynthia S. Crowson2 and Eric L. Matteson1, 1Rheumatology, Mayo Clinic, Rochester, MN, 2Health Sciences Research, Mayo Clinic, Rochester, MN

    Sarcoidosis is Associated With Increased Risk of Venous Thromboembolism: A Population-Based Study Background/Purpose: Chronic inflammation has been increasingly recognized as a risk factor of venous…
  • Abstract Number: 262 • 2016 ACR/ARHP Annual Meeting

    Seasonal Variation in Incidence of Sarcoidosis: A Population-Based Study 1976-2013

    Patompong Ungprasert1, Cynthia S. Crowson2 and Eric L. Matteson1, 1Rheumatology, Mayo Clinic, Rochester, MN, 2Health Sciences Research, Mayo Clinic, Rochester, MN

    Seasonal Variation in Incidence of Sarcoidosis: A Population-Based Study 1976-2013 Background/Purpose:   Sarcoidosis is a multi-systemic disorder of unknown etiology. Studies of seasonal and regional…
  • Abstract Number: 263 • 2016 ACR/ARHP Annual Meeting

    How Does Sarcoidosis Present in Spain? Characteristics at Diagnosis of 979 Patients from the Sarcogeas-SEMI Registry

    Pilar Brito-Zerón1,2, Belchin Kostov3, Marta Pérez de Lis4, Guadalupe Fraile5, Ricardo Gómez De La Torre6, María Roca Herrera7, Begoña De Escalante Yangüela8, Ana Alguacil9, Mercedes Pilar Perez Conesa10, Francisco Javier Rascón11, Jose Salvador Garcia Morillo12, Carlos Feijoo Massó13, Eva Fonseca Aizpuru14, Mariona Bonet15, Naya Faro Minguez16, Gloria De La Red Bellvis17, Eva Calvo Begueria18, Albert Gómez Lozano19, Enrique Peral Gutiérrez De Ceballos20, Jorge Francisco Gómez Cerezo21, Gracia Cruz Caparrós22, Patricia Perez Guerrero23, Sergio Rodríguez Fernández24, Alberto Gato Diez25, Neera Toledo Samaniego26, Miriam Akasbi27, Angel Robles28, Inmaculada Ojeda29, Maria José Vives30, María Penadés Vidal31, César Morcillo32, Moisés De Vicente33, Soledad Retamozo2,34, Lucio Pallarés11, Manuel Ramos-Casals35, Roberto Pérez-Alvarez4 and SARCOGEAS Registry, GEAS-SEMI, 1Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA- Sanitas, Barcelona., Bacelona, Spain, 2Laboratory of Systemic Autoimmune Diseases “Josep Font”, CELLEX, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Systemic Autoimmune Diseases, ICMID, Hospital Clinic, Barcelona, Barcelona, Spain, 3Primary Care Research Group, IDIBAPS, Centre d’Assistència Primària ABS Les Corts, CAPSE, Barcelona, Barcelona, Spain, 4Department of Internal Medicine, Hospital Alvaro Cunqueiro, Vigo, Vigo, Spain, 5Department of Internal Medicine, Hospital Ramón y Cajal, Madrid, Spain, Madrid, Spain, 6Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Oviedo, Spain, 7Department of Internal Medicine, Hospital Universitari Joan XXIII, Tarragona, Tarragona, Spain, 8Department of Internal Medicine, Hospital Clínico, Zaragoza, Zaragoza, Spain, 9Department of Internal Medicine, Hospital Virgen de la Salud, Toledo, Toledo, Spain, 10Department of Internal Medicine, Hospital Universitario Miguel Servet, Zaragoza, Zaragoza, Spain, 11Department of Internal Medicine, Hospital Son Espases. Palma de Mallorca, Palma de Mallorca, Spain, 12Department of Internal Medicine, Hospital Virgen del Rocio, Sevilla, Sevilla, Spain, 13Department of Internal Medicine, Hospital Parc Taulí, Sabadell, Sadabell, Spain, 14Department of Internal Medicine, Hospital de Cabueñes, Gijón, Gijón, Spain, 15Department of Internal Medicine, Althaia, Xarxa Assistencial de Manresa, Manresa, Spain, 16Department of Internal Medicine, Hospital Clínico San Cecilio, Granada, Granada, Spain, 17Department of Internal Medicine, Hospital de Santa Coloma de Gramanet, Barcelona, Barcelona, Spain, 18Department of Internal Medicine, Hospital General San Jorge, Huesca, Huesca, Spain, 19Department of Internal Medicine, Hospital de Santa Caterina, Girona, Girona, Spain, 20Department of Internal Medicine, Hospital Virgen Macarena, Sevilla, Sevilla, Spain, 21Department of Internal Medicine, Hospital Infanta Sofía, San Sebastián, San Sebastian, Spain, 22Department of Internal Medicine, Hospital de Poniente El Ejido, Almería, Almería, Spain, 23Department of Internal Medicine, Hospital Universitario Puerta del Mar, Cádiz, Cadiz, Spain, 24Department of Internal Medicine, Hospital da Barbanza, A Coruña, A Coruña, Spain, 25Department of Internal Medicine, Complejo Hospitalario Albacete, Albacete, Albacete, Spain, 26Department of Internal Medicine, Hospital Gregorio Marañón, Madrid, Madrid, Spain, 27Department of Internal Medicine, Hospital Infanta Leonor, Madrid, Madrid, Spain, 28Department of Internal Medicine, Hospital La Paz, Madrid, Madrid, Spain, 29Department of Internal Medicine, Hospital Valle del Guadiato, Córdoba, Cordoba, Spain, 30Department of Internal Medicine, Parc Sanitari San Joan de Déu, San Boi de Llobregat, Barcelona, Spain, 31Department of Internal Medicine, Hospital De Manises, Valencia, Valencia, Spain, 32Department of Medicine, Hospital CIMA-Sanitas, Barcelona, Barcelona, Spain, 33Department of Internal Medicine, Hospital Nuestra Señora del Prado, Talavera, Talavera, Spain, 34Rheumatology Unit, Hospital Privado Centro Médico de Córdoba, Argentina, Córdoba, Argentina, 35Laboratory of Systemic Autoimmune Diseases “Josep Font”, CELLEX, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Systemic Autoimmune Diseases, ICMID, Hospital Clinic, Barcelona, Spain, Barcelona, Spain

    Background/Purpose: To characterize the main epidemiological, clinical and radiological features at presentation of sarcoidosis in a large multicenter cohort from Southern Europe. Methods: In January…
  • Abstract Number: 264 • 2016 ACR/ARHP Annual Meeting

    Ethnicity-Related Differences in the Clinical Presentation of Sarcoidosis in Spain (SARCOGEAS-SEMI Registry)

    Roberto Pérez-Alvarez1, Soledad Retamozo2,3, Belchin Kostov4, Andrés González García5, Carmen Yllera Gutiérrez6, Miguel López Dupla7, Esperanza Bueno Juana8, Ana Alguacil9, Juan Escobedo Palau10, Francisco Javier Rascón11, Jose Salvador Garcia Morillo12, Carles Tolosa Vilella13, Eva Fonseca Aizpuru14, Mariona Bonet15, Naya Faro Minguez16, Anna Sánchez Biosca17, Ana Belén Madroñero18, Cristina Soler I Ferrer19, Enrique Peral Gutiérrez De Ceballos20, Jorge Francisco Gómez Cerezo21, Gracia Cruz Caparrós22, Patricia Perez Guerrero23, Sergio Rodríguez Fernández24, Alberto Gato Diez25, Blanca Pinilla26, Miriam Akasbi27, Angel Robles28, Inmaculada Ojeda29, María José Vives30, María Penadés Vidal31, Moisés De Vicente32, César Morcillo33, Lucio Pallarés11, Pilar Brito-Zerón3,34 and SARCOGEAS Registry, GEAS-SEMI, 1Department of Internal Medicine, Hospital Alvaro Cunqueiro, Vigo, Vigo, Spain, 2Rheumatology Unit, Hospital Privado Centro Médico de Córdoba, Postgraduate Career of Rheumatology Catholic University of Córdoba, Fundación para las Ciencias Biomédicas de Córdoba (FUCIBICO), Cordoba, Argentina, 3Laboratory of Systemic Autoimmune Diseases “Josep Font”, CELLEX, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Systemic Autoimmune Diseases, ICMID, Hospital Clinic, Barcelona, Barcelona, Spain, 4Primary Care Research Group, IDIBAPS, Centre d’Assistència Primària ABS Les Corts, CAPSE, Barcelona, Barcelona, Spain, 5Department of Internal Medicine, Hospital Ramón y Cajal, Madrid, Madrid, Spain, 6Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Oviedo, Spain, 7Department of Internal Medicine, Hospital Universitari Joan XXIII, Tarragona, Tarragona, Spain, 8Department of Internal Medicine, Hospital Clínico, Zaragoza, Zaragoza, Spain, 9Department of Internal Medicine, Hospital Virgen de la Salud, Toledo, Toledo, Spain, 10Department of Internal Medicine, Hospital Universitario Miguel Servet, Zaragoza, Zaragoza, Spain, 11Department of Internal Medicine, Hospital Son Espases. Palma de Mallorca, Palma de Mallorca, Spain, 12Department of Internal Medicine, Hospital Virgen del Rocio, Sevilla, Sevilla, Spain, 13Department of Internal Medicine, Hospital Parc Taulí, Sabadell, Sadabell, Spain, 14Department of Internal Medicine, Hospital de Cabueñes, Gijón, Gijón, Spain, 15Department of Internal Medicine, Althaia, Xarxa Assistencial de Manresa, Manresa, Spain, 16Department of Internal Medicine, Hospital Clínico San Cecilio, Granada, Granada, Spain, 17Department of Internal Medicine, Hospital de Santa Coloma de Gramenet Barcelona, Barcelona, Spain, 18Department of Internal Medicine, Hospital General San Jorge, Huesca, Huesca, Spain, 19Department of Internal Medicine, Hospital de Santa Caterina, Girona, Girona, Spain, 20Department of Internal Medicine, Hospital Virgen Macarena, Sevilla, Sevilla, Spain, 21Department of Internal Medicine, Hospital Infanta Sofía, San Sebastián, San Sebastian, Spain, 22Department of Internal Medicine, Hospital de Poniente El Ejido, Almería, Almería, Spain, 23Department of Internal Medicine, Hospital Universitario Puerta del Mar, Cádiz, Cadiz, Spain, 24Department of Internal Medicine, Hospital da Barbanza, A Coruña, A Coruña, Spain, 25Department of Internal Medicine, Complejo Hospitalario Albacete, Albacete, Albacete, Spain, 26Department of Internal Medicine, Hospital Gregorio Marañón, Madrid, Madrid, Spain, 27Department of Internal Medicine, Hospital Infanta Leonor, Madrid, Madrid, Spain, 28Department of Internal Medicine, Hospital La Paz, Madrid, Madrid, Spain, 29Department of Internal Medicine, Hospital Valle del Guadiato, Córdoba, Cordoba, Spain, 30Department of Internal Medicine, Parc Sanitari San Joan de Déu, San Boi de Llobregat, Barcelona, Spain, 31Department of Internal Medicine, Hospital De Manises, Valencia, Valencia, Spain, 32Department of Internal Medicine, Hospital Nuestra Señora del Prado, Talavera, Talavera, Spain, 33Department of Medicine, Hospital CIMA-Sanitas, Barcelona, Barcelona, Spain, 34Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA- Sanitas, Barcelona., Bacelona, Spain

    Background/Purpose: To evaluate the influence of ethnicity on the clinical presentation of sarcoidosis in a large multicenter cohort from Southern Europe. Methods: In January 2016,…
  • Abstract Number: 265 • 2016 ACR/ARHP Annual Meeting

    Characterization of Eye Sarcoidosis with or without Systemic Involvement: Application of Iwos Criteria in an Uveitis Unit

    Hiurma Sanchez-Perez1, Denisse Angel-Pereira2, Maria Garcia-Gonzalez3, Ivan Ferraz-Amaro4, Elisa Trujillo5, Maria-Jose Losada-Castilla6 and Beatriz Rodriguez Lozano7, 1Rheumatology, Rheumatology Division, Hospital Universitario de Canarias, La Laguna. Tenerife, Spain, 2Ophtalmology, Ophtalmology Division, Hospital Universitario de Canarias, La Laguna, Spain, 3Rheumatology, Hospital Universitario de Canarias, La Laguna, Tenerife, Spain, 4Rheumatology, Rheumatology Division, Hospital Universitario de Canarias, Tenerife, Spain, 5Rheumatology, Hospital Universitario de Canarias, La Laguna.Tenerife, Spain, 6Ophtalmology Division, Hospital Universitario de Canarias, La Laguna, Spain, 7Rheumatology, Rheumatology Department. Hospital Universitario de Canarias, S/C TENERIFE, Spain

    Background/Purpose: Sarcoidosis(Sa) eye involvement is the most common extrapulmonary manifestation and the initial presentation in 5-20% of cases. The definitive diagnosis is histopathological. The sensitivity(S)…
  • Abstract Number: 266 • 2016 ACR/ARHP Annual Meeting

    Diffuse Idiopathic Skeletal Hyperostosis and Obesity- Is There a Causal Relationship?

    Melissa Wang1, Mariko Ishimori2, Greg Kinney3, Irina Ianculsecu1, Elizabeth Regan4, Michael Weisman5 and Mark Goodarzi6, 1Cedars-Sinai Medical Center, Los Angeles, CA, 2Rheumatology, Cedars-Sinai Medical Center, Los Angeles, CA, 3Department of Epidemiology, Colorado School of Public Health, Aurora, CO, 4Medicine, National Jewish Health, Denver, CO, 5Rheumatology, Cedars-Sinai Medical Center, West Hollywood, CA, 6Division of Endocrinology, Cedars-Sinai Medical Center, Los Angeles, CA

    Background/Purpose: Diffuse Idiopathic Skeletal Hyperostosis (DISH) is an incompletely understood condition characterized by new bone formation affecting the spine. Despite reported links between DISH and…
  • Abstract Number: 267 • 2016 ACR/ARHP Annual Meeting

    Diffuse Idiopathic Skeletal Hyperostosis: Can We Identify Different Clinicoradiological Patterns?

    Teresa Clavaguera1, Ramon Valls2 and Mari Carmen Rodriguez-Jimeno2, 1Unitat de Reumatologia, Hospital de Palamós, Girona, Spain, 2rheumatology, Hospital de Palamós, Palamós, Spain

    Background/Purpose:  Diffuse Idiopathic Skeletal Hyperostosis (DISH) was described based on vertebral radiological signs (Resnick). Subsequently, Utsinger presented other criteria that add extraspinal involvement that allowed…
  • Abstract Number: 268 • 2016 ACR/ARHP Annual Meeting

    Effect of Abatacept Treatment on T Cells in Muscle Tissue and Peripheral Blood in Polymyositis and Dermatomyositis Patients

    Quan Tang1, Daniel Ramsköld2, Olga Krystufkova3, Herman F Mann4, Cecilia Wick5, Maryam Dastmalchi6, Peter Brodin7, Vivianne Malmström8, Jiri Vencovsky9 and Ingrid E. Lundberg6, 1Department of Medicine, Rheumatology Unit, Department of Medicine, Karolinska University Hospital, Solna, Karolinska Institutet, Stockholm, Sweden, 2Department of Medicine, Rheumatology Unit, Karolinska University Hospital, Solna, Stockholm, Sweden, 3Institute of Rheumatology, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic, 41st Faculty of Medicine, Charles University, Prague, Prague, Czech Republic, 5Rheumatology Unit, Department of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden, 6Department of Medicine, Rheumatology Unit, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden, 7Science for Life Laboratory, Department of Medicine Solna, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden, 8Department of Medicine, Rheumatology Unit, Department of Medicine, Karolinska Institute, Karolinska University Hospital, Stockholm, Sweden, 9Pediatria II, Reumatologia, PRINTO, Istituto Giannina Gaslini, Genoa, Italy

    Background/Purpose:  Abatacept (CTLA4-Ig), a blocking agent for T cell co-stimulation, has been proven beneficial in several autoimmune diseases. The aim of the study was to…
  • Abstract Number: 269 • 2016 ACR/ARHP Annual Meeting

    The Immunoproteasomes Are Essential for Maintaining Myokine Production and MHC Class I Expression in Idiopathic Inflammatory Myopathies

    Salyan Bhattarai1, Khetam Ghannam1, Sabine Krause2, Olivier Benveniste3, Andreas Marg4, Gerjan de Bruin5, Bo-Tao Xin5, Herman S Overkleeft6, Simone Spuler7, Werner Stenzel7 and Eugen Feist7, 1Rheumatology and Clinical Immunology, Charité-Universitätsmedizin Berlin, Berlin, Germany, 2Friedrich Baur Institute, Ludwig Maximilians University, Munich, Germany, 3Pitié-Salpêtrière University Hospital, Paris, France, 4Muscle Research Unit, Experimental and Clinical Research Center, Charité-Universitätsmedizin Berlin, Berlin, Germany, 5Leiden Institute of Chemistry, Leiden University, Leiden, Netherlands, 6Leiden University, Leiden, Netherlands, 7Charité-Universitätsmedizin Berlin, Berlin, Germany

    Background/Purpose: Idiopathic inflammatory myopathies (IIMs) are muscle diseases, characterized by inflammatory infiltration and increased expression of MHC class I molecules on myofibers. Immunoproteasome, as a…
  • Abstract Number: 270 • 2016 ACR/ARHP Annual Meeting

    Retinoic Acid-Inducible Gene-I Increased in Peripheral CD3+T Lymphocytes of Patients with Dermatomyositis

    Lu Zhang1, Qisheng Xia2, Wenli Li3, Qinglin Peng4 and Guochun Wang1, 1Rheumatology, China-Japan Friendship Hospital, Beijing, China, 2Molecular Biology Labortary, China-Japan Friedship Hospital, Beijing, China, 3Rheumatology, China-Japan Friendship Hospital, Beiing, China, 4Rheuamtology, China-Japan Friendship Hospital, Beijing, China

    Background/Purpose: The study population included 26 treatment-naive DM patients from Department of Rheumatology at China-Japan Friendship Hospital between July 2015 and February 2016 who fulfilled…
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