Abstracts tagged "interstitial lung disease"

Abstract Number: 1648 • 2019 ACR/ARP Annual Meeting
Systemic Sclerosis Auto-antibody Profiles Predict Interstitial Lung Disease Onset but Not Progression
Boyang Zheng¹, Mianbo Wang ², Marvin Fritzler ³, May Choi ³, Murray Baron ⁴ and Marie Hudson ⁵, ¹Jewish General Hospital, McGill University, Montreal, QC, Canada, ²Lady Davis Institute, Jewish General Hospital, Montreal, QC, Canada, ³Cumming School of Medicine, University of Calgary, Calgary, AB, Canada, ⁴Jewish General Hospital, McGill University, Montreal, Canada, ⁵Jewish General Hospital, Lady Davis Institute for Medical Research, and Department of Medicine, McGill University, Montreal, QC, Canada
Background/Purpose: Interstitial lung disease (ILD) is frequent manifestation and a leading cause of mortality in systemic sclerosis (SSc). Some SSc-related autoantibodies (aAbs), especially anti-topoisomerase I…
Abstract Number: 2342 • 2019 ACR/ARP Annual Meeting
Association of Serum KL-6 Level and Change of Pulmonary Function in Interstitial Lung Disease of Rheumatoid Arthritis – Data from Prospective KOrean Rheumatoid Arthritis Interstitial Lung Disease (KORAIL) Study
Jeong Seok Lee¹, Young Im Yoon ², Eun Young Lee ³, Jung Yoon Choe ⁴, Hwa Jung Lee ⁵, Yong-Beom Park ⁶, Eun Ha Kang ⁷, You-Jung Ha ⁷, Yeon-Ah Lee ⁸ and Seong Hye Jang ⁹, ¹Korea Advanced Institute of Science and Technology, Daejeon, Republic of Korea, ²Seoul National University Hospital, Seoul, Republic of Korea, ³Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea, Seoul, Republic of Korea, ⁴Division of Rheumatology, Daegu Catholic University Medical Center, Daegu, Republic of Korea, ⁵DAEGU CATHOLIC UNIVERSITY, Daegu, Republic of Korea, ⁶Yonsei university college of medicine, severance hospital, Seodaemun-gu, Seoul-t'ukpyolsi, Republic of Korea, ⁷Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Republic of Korea, Seongnam, Republic of Korea, ⁸School of Medicine, Kyung Hee University, Seoul, Republic of Korea, ⁹Soonchunhyang University, Cheonan, Republic of Korea
Background/Purpose: Interstitial lung disease (ILD) is the most common lung disease among rheumatoid arthritis (RA) patients. Although the overall mortality rates for RA have decreased…
Abstract Number: 708 • 2019 ACR/ARP Annual Meeting
Improvement and Stabilization of Lung Function in Patients with SSc-ILD Treated with Nintedanib vs Placebo in a Randomized, Placebo-Controlled Phase III Trial: Proportions of Patients with FVC Changes Using Cutoffs Previously Proposed to Define Minimally Clinically Important Differences
Yannick Allanore¹, Dinesh Khanna ², Elizabeth Volkmann ³, Christian Stock ⁴, Martina Gahlemann ⁵, Nils Schoof ⁶, Oliver Distler ⁷ and Toby Maher ⁸, ¹Dept. of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France, ²Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor, ³University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, ⁴Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, Ingelheim am Rhein, Germany, ⁵Boehringer Ingelheim (Schweiz) GmbH, Basel, Switzerland, Basel, Switzerland, ⁶Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, ⁷Dept. of Rheumatology, University Hospital Zürich, Zürich, Switzerland, Zürich, Switzerland, ⁸National Heart and Lung Institute, Imperial College London, UK and National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London, UK, London, United Kingdom
Background/Purpose: Mean change in forced vital capacity percent predicted (FVC %pred) is a common endpoint in the assessment of treatment-related outcomes in patients with interstitial…
Abstract Number: 1649 • 2019 ACR/ARP Annual Meeting
Mycophenolate Mofetil and Cyclophosphamide Improve Health-Related Quality of Life in Patients with Systemic Sclerosis Who Participated in SLS II
Elizabeth Volkmann¹, Donald Tashkin ², Michael Roth ³, Holly LeClair ², Philip Clements ², Daniel Furst ⁴, Robert Elashoff ² and Dinesh Khanna ⁵, ¹University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, ²University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, ³University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, ⁴University of California, Los Angeles, CA, ⁵Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor
Background/Purpose: Interstitial lung disease (ILD) is the leading cause of morbidity and mortality in systemic sclerosis (SSc). The majority of studies investigating novel therapies for…
Abstract Number: 2344 • 2019 ACR/ARP Annual Meeting
Efficacy of Mycophenolate Mofetil in the Treatment of Rheumatoid Arthritis Associated Interstitial Lung Disease
Jonathan Amos¹, Jennifer Kendall ¹, Robyn Moran ², Megan Krause ³, Paul Schmidt ⁴, Chase Hall ⁵, Mark Hamblin ⁶ and Mehrdad Maz ⁷, ¹University of Kansas Medical Center, Division of Rheumatology, KANSAS CITY, KS, ²University of Kansas Medical Center, Kansas City, KS, ³University of Kansas, KANSAS CITY, KS, ⁴University of Kansas Medical Center, Division of Rheumatology, Kansas City, ⁵University of Kansas Medical Center, Division of Pulmonary and Critical Care, KANSAS CITY, ⁶University of Kansas Medical Center, Division of Pulmonary and Critical Care, Kansas City, KS, ⁷The University of Kansas Medical Center, KANSAS CITY, KS
Background/Purpose: Interstitial lung disease (ILD) as an extra-articular manifestation of rheumatoid arthritis (RA) leads to significant morbidity and mortality. As data is limited, this study…
Abstract Number: 712 • 2019 ACR/ARP Annual Meeting
Safety and Tolerability of Nintedanib in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease in the SENSCIS Trial: Subgroup Analysis Based on Demographic Characteristics
Oliver Distler¹, Kristin Highland ², Maureen Mayes ³, Masataka Kuwana ⁴, Lesley Saketkoo ⁵, Madelon Vonk ⁶, Michael Kreuter ⁷, Laura Hummers ⁸, Ute von Wangenheim ⁹, Martina Gahlemann ¹⁰, Veronika Kohlbrenner ¹¹, Margarida Alves ¹², Emmanuelle Clerisme-Beaty ¹² and Arata Azuma ¹³, ¹Dept. of Rheumatology, University Hospital Zürich, Zürich, Switzerland, Zürich, Switzerland, ²Cleveland Clinic, Cleveland, Ohio, USA, Cleveland, OH, ³Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, Texas, USA, Houston, TX, ⁴Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, Bunkyo-ku, Tokyo, Japan, ⁵New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans; Tulane University School of Medicine, University Medical Center – Comprehensive Pulmonary Hypertension Center, USA, New Orleans, ⁶Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands, Nijmegen, Gelderland, Netherlands, ⁷Center for Interstitial and Rare Lung Diseases, Pneumology, Thoraxklinik, University of Heidelberg, Member of the German Center for Lung Research, Germany, Germany, Germany, ⁸Johns Hopkins University School of Medicine, Division of Rheumatology, Baltimore, MD, USA, Baltimore, MD, ⁹Boehringer Ingelheim Pharma GmbH & Co. KG, Biberach an der Riss, Germany, Biberach an der Riss, ¹⁰Boehringer Ingelheim (Schweiz) GmbH, Basel, Switzerland, Basel, Switzerland, ¹¹Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, CT, USA, Ridgefield, ¹²Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, Ingelheim am Rhein, Germany, ¹³Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan, Tokyo, Japan
Background/Purpose: In the SENSCIS trial in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD), the adverse events associated with nintedanib were manageable for most patients…
Abstract Number: 1654 • 2019 ACR/ARP Annual Meeting
Esophageal Dilation and Other Clinical Factors Associated with Pulmonary Function Decline in Patients with Systemic Sclerosis
Kimberly Showalter¹, Aileen Hoffmann ², Carrie Richardson ³, Kimberly Koloms ², Gerald Rouleau ², David Aaby ², Julia (Jungwha) Lee ⁴, Jane Dematte ², Rishi Agrawal ², Xiaoping Wu ⁵, Rowland Chang ⁴ and Monique Hinchcliff ⁶, ¹Hospital for Special Surgery, New York, NY, ²Northwestern University Feinberg School of Medicine, Chicago, ³Rush University, Chicago, ⁴Northwestern University Feinberg School of Medicine, Chicago, IL, ⁵New York Presbyterian/Weill Cornell, New York, ⁶Yale University, Section of Rheumatology, Allergy and Immunology, New Haven, CT
Background/Purpose: Prior work has shown that Scl-70 positive autoantibody status and baseline c-reactive protein (CRP) are associated with more rapid pulmonary function decline in patients…
Abstract Number: 2345 • 2019 ACR/ARP Annual Meeting
Abatacept in Rheumatoid Arthritis with Interstitial Lung Disease: A Retrospective Multicenter Study of 263 Patients
C. Fernández-Díaz¹, Rafael Melero ², Javier Loricera ³, Santos Castañeda ⁴, Francisco Ortiz-Sanjuán ⁵, I. Casafont-Solé ⁶, Carmen carrasco-Cubero ⁷, A. Juan-Mas ⁸, Raquel Almodovar-Gonzalez ⁹, S. Rodriguez-Garcia ¹⁰, R. Castellanos ¹¹, O. Maiz-Alonso ¹², C. Aguilera-Cros ¹³, I. Cabezas-rodriguéz ¹⁴, EC Cervantes ¹⁵, M. Moreno ¹⁶, L. Arboleya ¹⁷, C. Montagut ¹⁸, Patricia Carreira ¹⁹, C. Ojeda-Garcia ²⁰, Gema Bonilla ²¹, T. Perez-Sandoval ²², P. Vela ²³, JL Andreu ²⁴, Susana Romero-Yuste ²⁵, A. Urruticoechea-Arana ²⁶, E. Salgado-Pérez ²⁷, Cristina Hidalgo ²⁸, Javier Narváez ²⁹, E. Raya ³⁰, MJ. Moreno-Ramos ³¹, P. Morales-Garrido ³², L. Pérez-alba ³³, Carlos Fernández-López ³⁴, Ignacio Villa ³⁵, Noelia Álvarez-Rivas ³⁶, Juan María Blanco-Madrigal ³⁷, JR Jimenez-Aberasturi ³⁸, A. Perez-Linaza ³⁹, N. Del-val-del-amo ⁴⁰, Sabela Fernández ⁴¹, A. García-valle ⁴², C.A. Peralta-Ginés ⁴³, A. Garcia-Aparicio ⁴⁴, L. Exposito-pérez ⁴⁵, N. Mena-Vazquez ⁴⁶, R. López-Sánchez ⁴⁷, B. Garcia-magallon ⁴⁸, Miguel Angel González-Gay ⁴⁹ and Ricardo Blanco ⁵⁰, ¹HU Vinalopo, Madrid, Madrid, Spain, ²Complexo Hospitalario Universitario de Vigo, Vigo, Galicia, Spain, ³Rheumatology Department. Hospital Marqués de Valdecilla, Santander, Cantabria, Spain, ⁴Rheumatology Department, Hospital Universitario de la Princesa, IIS-Princesa, Madrid, Spain, Madrid, Spain, ⁵Hospital Universitario y Politécnico La Fe, Valencia, Comunidad Valenciana, Spain, ⁶HU German Trias i pujol, BAacelona, ⁷CHU de Badajoz, Badajoz, Spain, ⁸H Son Llázer, Palma de Mallorca, Islas Baleares, Spain, ⁹Hospital Universitario Fundación Alcorcón, Madrid, Spain, ¹⁰HU. Clinic, Barcelona, ¹¹Fundacio Clinic. Hospital Clinic de Barcelona, Barcelona, Spain, ¹²HU Donostia, Donostia, Spain, ¹³hospital virgen del rocio, Sevilla, Spain, ¹⁴HU. Río Hortega, Valladolid, Spain, ¹⁵HU Santiago de compostela, Santiago, ¹⁶Parc Tauli Hospital Universitari, Sabadell, Spain, ¹⁷Hospital Central Asturias, OViedo, ¹⁸HCU Valladolid, Valladolid, Spain, ¹⁹Hospital Universitario 12 de Octubre, Madrid, Spain, ²⁰HU Virgen de la Macarena, Sevilla, ²¹Rheumatology Department, Hospital Universitario La Paz, Madrid, Spain, Madrid, Spain, ²²HU León, León, Spain, ²³Hospital General Universitario de Alicante, Alicante, Comunidad Valenciana, Spain, ²⁴HU Puerta del Hierro, Madrid, ²⁵Complejo Hospitalario Universitario Pontevedra, Pontevedra, Galicia, Spain, ²⁶HU Can Misses, Ibiza, Spain, ²⁷C.H Ourense, Ourense, ²⁸Hospital Clínico Universitario de Salamanca, Salamanca, Castilla y Leon, Spain, ²⁹Rheumatology Department, Hospital Universitario de Bellvitge, Barcelona, Spain, Barcelona, Catalonia, Spain, ³⁰Hospital San Cecilio, Granada, Andalucia, Spain, ³¹HU Arrixaca, Murcia, Spain, ³²Hospital San Cecilio, Granada, Spain, ³³HU San Cecilio, Granada, Spain, ³⁴Servicio de Reumatología. Instituto de Investigación Biomédica de A Coruña (INIBIC). Complexo Hospitalario Universitario de A Coruña (CHUAC), Sergas. Universidade da Coruña (UDC). As Xubias, 15006. A Coruña, España, A Coruña, Spain, ³⁵Hospital de Sierrallana, Torrelavega, Cantabria, Spain, ³⁶Hospital Universitario Lucus Augusti, Lugo, Galicia, Spain, ³⁷Rheumatology Unit, Basurto University Hospital, Bilbao, Spain, Bilbao, Spain, ³⁸HU Araba, Vitoria, Spain, ³⁹HU Puerta del Mar, Cadiz, ⁴⁰HU Navarra, Pamplona, Spain, ⁴¹Hospital Universitario San Agustín, Avilés, Asturias, Spain, ⁴²Rheumatology Department. Hospital Rio Carrión, Palencia, Spain, ⁴³HU Lozano Blesa, Zaragoza, ⁴⁴H Virgen de la salud, Toledo, Spain, ⁴⁵HU Canarias, Tenerife, Spain, ⁴⁶HU Malaga., Malaga, ⁴⁷Hospital Negrin, Tenerife, ⁴⁸H Huesca, HUesca, ⁴⁹Universidad de Cantabria and IDIVAL, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ⁵⁰Rheumatology, Hospital Universitario Marques de Valdecilla, Santander, Spain
Background/Purpose: Interstitial Lung Disease (ILD) is a severe extraarticular manifestation of rheumatoid arthritis (RA).Interstitial lung disease (ILD) associated with Rheumatoid Arthritis (RA) has a poor…
Abstract Number: 737 • 2019 ACR/ARP Annual Meeting
The MUC5B Promoter Variant Does Not Predict Outcomes in Systemic Sclerosis-related Interstitial Lung Disease
Elizabeth Volkmann¹, Donald Tashkin ², Michael Roth ³, Ning Li ², Grace Kim ², Jonathan Goldin ², Maureen Mayes ⁴, Julio Charles ⁵, Philip Clements ², Daniel Furst ⁶, Dinesh Khanna ⁷, Robert Elashoff ² and Shervin Assassi ⁴, ¹University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, ²University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, ³University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, ⁴Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, Texas, USA, Houston, TX, ⁵University of Texas, Houston, Houston, ⁶University of California, Los Angeles, CA, ⁷Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor
Background/Purpose: Interstitial lung disease (ILD) affects the majority of patients with systemic sclerosis (SSc). The disease course of ILD varies among SSc patients and no…
Abstract Number: 1656 • 2019 ACR/ARP Annual Meeting
Health-Related Quality of Life in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD): Impact of Lung Function on Patient-Reported Outcomes in a Randomized Phase III Trial
Michael Kreuter ¹, Anna Maria Hoffmann-Vold ², Marco Matucci-Cerinic ³, Lesley Saketkoo ⁴, Kristin Highland⁵, Hilary Wilson ⁶, Margarida Alves ⁷, Nils Schoof ⁶ and Toby Maher ⁸, ¹Center for Interstitial and Rare Lung Diseases, Pneumology, Thoraxklinik, University of Heidelberg, Member of the German Center for Lung Research, Germany, Germany, Germany, ²Department of Rheumatology, Oslo University Hospital, Oslo, Norway, Oslo, Norway, ³University of Florence, Department of Medicine, Florence, Italy, Florence, Italy, ⁴New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans; Tulane University School of Medicine, University Medical Center – Comprehensive Pulmonary Hypertension Center, USA, New Orleans, ⁵Cleveland Clinic, Cleveland, Ohio, USA, Cleveland, OH, ⁶Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, ⁷Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, Ingelheim am Rhein, Germany, ⁸National Heart and Lung Institute, Imperial College London, UK and National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London, UK, London, United Kingdom
Background/Purpose: SSc-ILD is a major cause of morbidity and mortality in patients with systemic sclerosis. A subset of patients with SSc-ILD show a decline in…
Abstract Number: 2346 • 2019 ACR/ARP Annual Meeting
Interstitial Lung Abnormalities in Rheumatoid Arthritis Patients: Identifying Associated Risk Factors
Karina Bonfiglioli¹, Márcio Sawamura ², Renato Nakagawa ³, Fábio Arimura ³, Diana Rangel ³, Cleonice Bueno ⁴, Ana Cristina de Medeiros Ribeiro ¹, Carlos Carvalho ³, Eloisa Bonfa ⁵, Ronaldo Kairalla ³ and Leticia Kawano-Dourado ⁶, ¹Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Sao Paulo, Brazil, ²Division of Radiology, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Br, Sao Paulo, Sao Paulo, Brazil, ³Pulmonary Division, Heart Institute (InCor)- Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, BR, Sao Paulo, Sao Paulo, Brazil, ⁴Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, BR, Sao Paulo, Sao Paulo, Brazil, ⁵Rheumatology Division, Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo (HCFMUSP), Sao Paulo, Brazil., Sao Paulo, Sao Paulo, Brazil, ⁶University of Sao Paulo, Sao Paulo, Brazil
Background/Purpose: A wide spectrum of interstitial lung abnormalities (ILA) occurs in patients with rheumatoid arthritis (RA). This study characterized ILA in large single-center cohort, including…
Abstract Number: 740 • 2019 ACR/ARP Annual Meeting
Rituximab Rescue Therapy in Patients with Systemic Sclerosis or Other Connective Tissue Diseases and Refractory Interstitial Lung Disease
Magdalena Benad ¹, Dirk Koschel ¹, Kristine Herrmann ¹, Kristin Wiefel ¹, Alexander Kleymann ¹ and Martin Aringer², ¹University Medical Center and Faculty of Medicine TU Dresden, Dresden, Germany, ²Division of Rheumatology, Department of Medicine III, University Medical Center & Faculty of Medicine Carl Gustav Carus, TU Dresden, Dresden, Germany, Dresden, Germany
Background/Purpose: Interstitial lung disease (ILD) is a common and life-threatening organ manifestation of patients with various connective tissue diseases (CTDs), and with systemic sclerosis and…
Abstract Number: 1830 • 2019 ACR/ARP Annual Meeting
Short- and Long-term Morbidity and Mortality Outcomes of African American Patients with Systemic Sclerosis-Related Interstitial Lung Disease
Elizabeth Volkmann¹, Virginia Steen ², Ning Li ³, Michael Roth ⁴, Philip Clements ³, Dinesh Khanna ⁵, Daniel Furst ⁶, Shervin Assassi ⁷, Grace Kim ³, Jonathan Goldin ³, Robert Elashoff ³ and Donald Tashkin ³, ¹University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, ²Georgetown University, Washington, D.C., USA, Georgetown, ³University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, ⁴University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, ⁵Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor, ⁶University of California, Los Angeles, CA, ⁷Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, Texas, USA, Houston, TX
Background/Purpose: Observational studies have demonstrated that African American (AA) patients with systemic sclerosis (SSc) have a more unfavorable prognosis compared with non-AA. However, no studies…
Abstract Number: 2360 • 2019 ACR/ARP Annual Meeting
Safety of Biological DMARD in Patients with Interstitial Lung Disease from a Chilean Cohort of Patients with Rheumatoid Arthritis
Silvana Saavedra¹, Karen Vergara ², Claudia Hernandez ², Pedro Zamorano ², Annelise Goecke ², Luis Toro ² and FELIPE REYES ¹, ¹HOSPITAL CLINICO UNIVERSIDAD DE CHILE, Santiago, Chile, ²HOSPITAL CLINICO UNIVERSIDAD DE CHILE, Santiago, Region Metropolitana, Chile
Background/Purpose: Interstitial lung disease (ILD) is a common pulmonary manifestation of rheumatoid arthritis (RA) that may be related to the inflammatory process itself, to infectious…
Abstract Number: 796 • 2019 ACR/ARP Annual Meeting
Under Detection of Interstitial Lung Disease in Juvenile Systemic Sclerosis (jSSc) Utilizing Pulmonary Function Tests: Results from the Juvenile Scleroderma Inception Cohort
Ivan Foeldvari¹, Bernd Hinrichs ², Kathryn Torok ³, Maria José Santos ⁴, Ozgur Kasapcopur ⁵, Amra Adrovic ⁶, Valda Stanevicha ⁷, Flavio Sztajnbok ⁸, Maria Teresa Terreri ⁹, Ekaterina Alexeeva ¹⁰, Jordi Antón ¹¹, Maria Katsicas ¹², Vanessa Smith ¹³, Tadej Avcin ¹⁴, Rolando Cimaz ¹⁵, Mikhail Kostik ¹⁶, Thomas Lehman ¹⁷, Walter Sifuentes-Giraldo ¹⁸, Simone Appenzeller ¹⁹, Mahesh Janarthanan ²⁰, Monika Moll ²¹, Dana Nemcova ²², Dieneke Schonenberg ²³, Christina Battagliotti ²⁴, Lillemor Berntson ²⁵, Blanca Bica ²⁶, Juergen Brunner ²⁷, Patricia Costa Reis ²⁸, Despina Eleftheriou ²⁹, Liora Harel ³⁰, Gerd Horneff ³¹, Tilmann Kallinich ³², Dragana Lazarevic ³³, Kirsten Minden ³⁴, Susan Nielsen ³⁵, Farzana Nuruzzaman ³⁶, Anjali Patwardhan ³⁷, Yosef Uziel ³⁸ and Nicola Helmus ¹, ¹Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany, ²Asklepios Klinik Nord - Heidberg, Hamburg, Germany, ³UPMC Children's Hospital of Pittsburgh, Pittsburgh, ⁴Rheumatology department, Hospital Garcia de Orta, Almada, Portugal, ⁵Department of Pediatric Rheumatology, Istanbul University-Cerrahpasa, Istanbul, Turkey, Istanbul, Turkey, ⁶Cerrahpaşa Tıp Fakültesi, Istanbul, Turkey, ⁷Riga Stradins University, Riga, Latvia, ⁸Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil, ⁹Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil, ¹⁰National Medical Research Center of Children`s Health, Moscow, Russia, ¹¹Hospital Sant Joan de Déu, Barcelona, Spain, ¹²Hospital de Pediatria, Buenos Aires, Argentina, ¹³Dept. of Rheumatology, Ghent University Hospital, Ghent, Belgium; Dept. of Internal Medicine, Ghent University, Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium, Gent, Belgium, ¹⁴Ljubljana University Medical Centre, Ljubljana, Slovenia, ¹⁵University Hospital Meyer, Florence, Italy, ¹⁶Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia, ¹⁷Hospital for Special Surgery, New York, ¹⁸Hospital Universitario Ramón y Cajal, Madrid, Spain, ¹⁹UNICAMP Universidade Estadual de Campinas, Sao Paulo, Brazil, ²⁰Sri Ramachandra University, Chennai, India, ²¹University of Tuebingen, Tuebingen, Germany, ²²General University Hospital, Prague, Czech Republic, ²³Amsterdam UMC, Amsterdam, Netherlands, ²⁴Children's Hospital Dr. Orlando Alassia, Santa Fee, Argentina, ²⁵Uppsala University Hospital, Uppsala, Sweden, ²⁶Hospital Universitário Clementino Fraga Filho (HUCFF/UFRJ), Rio de Janeiro, Brazil, ²⁷Tirol Kliniken, Innsbruck, Austria, ²⁸Hospital de Santa Maria, Lisbon, Portugal, ²⁹Great Ormond Street Hospital, London, United Kingdom, ³⁰Tel Aviv University, Tel Aviv, Israel, ³¹Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany, ³²Charite, Berlin, Germany, ³³KC Niš, Niš, Serbia, ³⁴German Rheumatism Research Center and Charité University Medicine, Berlin, Germany, ³⁵Department of Pediatrics, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark., Copenhavn, Denmark, ³⁶Stony Brook Children's Hospital, Stony Brook, NY, ³⁷University of Missouri, Columbia, ³⁸Meir Medical Center, Kfar Saba, Israel
Background/Purpose: Juvenile systemic sclerosis(jSSc) is an orphan disease with a prevalence in around 3 in a million children[1]. Pulmonary involvement in jSSc occurs in approximately 40…

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