Abstracts tagged "Fibrosing syndromes"

Abstract Number: 0133 • ACR Convergence 2022
Retention Rate of Targeted Therapies in Erdheim-Chester Disease
Corrado Campochiaro¹, alessandro tomelleri¹, Francesco Pegoraro², Giovanni Galardi³, Nicola Farina⁴, Martina Mazzariol⁵, Francesco Catamerò⁶, Giacomo De Luca⁴, Giulio Cavalli⁴, Augusto Vaglio⁷ and Lorenzo Dagna⁸, ¹IRCCS San Raffaele Hospital. Vita-Salute San Raffaele University, Milano, Italy, ²Department of Health Sciences, University of Florence, Firenze, Italy, ³Department of Health Sciences, University of Florence, Florence, Italy, ⁴IRCCS San Raffaele Hospital - Vita-Salute San Raffaele University, Milano, Italy, ⁵University of Turin, Torino, Italy, ⁶Vita-Salute San Raffaele University, Milano, Italy, ⁷Azienda Ospedaliero Universitaria Meyer, Parna, Italy, ⁸IRCCS San Raffaele Hospital and Vita-Salute San Raffaele University, Milano, Italy
Background/Purpose: Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis with a broad clinical spectrum. The therapeutic landscape of ECD has remarkably changed over the last…
Abstract Number: 1056 • ACR Convergence 2022
Clinical Characterization of a Cohort of Patient with Sclerotic-type Chronic Graft-versus-host Disease with Fascial/joint Involvement
Cristina Hidalgo¹, Concepción Roman², Olga Compan³, Sonia Pastor Navarro⁴, Marta Ibañez⁵, Belen Miguel⁶, Maria Dolores Sanchez⁷, Lucía Pantoja⁸, Carlos Montilla⁵ and Lucía López-Corral², ¹Hospital Universitario Salamanca, Salamanca, Spain, ²Universitary Hospital, Salamanca, Spain, ³Complejo asistencial Universitario de Palencia, Carbajosa De La Sagrada, Spain, ⁴Hospital Universitario de Salamanca, Salamanca, Spain, ⁵Complejo asistencial Universitario de Salamanca, Salamanca, Spain, ⁶University Hospital, Salamanca, Spain, ⁷Universitary Hospital, Valladolid, Spain, ⁸Complejo Asistencial Hospital General, Segovia, Spain
Background/Purpose: Graft-versus-host disease (GVHD) is the most frequent complication after allogeneic hematopoietic cell transplantation (allo-HCT). Its chronic form usually involves a multisystemic syndrome that reflects…
Abstract Number: 1184 • ACR Convergence 2022
Intracellular Calcium Signals Regulate the Pro-fibrotic Phenotype of Scleroderma Fibroblasts via Calcium/calmodulin-dependent Kinase II
Sirapa Vichaikul¹, William Brodie², Megan Mattichak², Qi Wu², Dinesh Khanna² and Eliza Pei-Suen Tsou², ¹Michigan Medicine, Howell, MI, ²University of Michigan, Ann Arbor, MI
Background/Purpose: We performed an in-depth analysis of the involvement of histone reader bromodomain extra-terminal proteins (BETs) in scleroderma (SSc) fibrosis and showed that JQ1, a…
Abstract Number: 0280 • ACR Convergence 2021
Mast Cells Contribute to the Development of Lung Fibrosis via Inducing Myofibroblast Differentiation by TGF-β Production
Shinjiro Kaieda¹, Masaki Okamoto², Masaki Tominaga¹ and Tomoaki Hoshino¹, ¹Kurume University School of Medicine, Kurume, Japan, ²Kyushu Medical Center, Fukuoka, Japan
Background/Purpose: Idiopathic pulmonary fibrosis is an interstitial lung disease of unknown etiology. The most critical event in the evolution of fibrosis is the appearance of…
Abstract Number: 1437 • ACR Convergence 2021
Fat and Fibrosis: A Novel Developmental Gene in Systemic Sclerosis
Nancy Wareing¹, Brian Skaug², Minghua Wu³, Scott Collum¹, Cory Wilson¹, Lucy Revercomb⁴, Marka Lyons⁵, Weizhen Bi⁶, Tingting Mills¹, Julio Charles⁵, Shervin Assassi¹ and Harry Karmouty-Quintana¹, ¹University of Texas McGovern Medical School at Houston, Houston, TX, ²University of Texas McGovern Medical School Houston, Houston, TX, ³University of Texas Health Science Center at Houston, Houston, TX, ⁴Rice University, Houston, TX, ⁵Division of Rheumatology, University of Texas McGovern Medical School at Houston, Houston, TX, ⁶McGovern Medical School at UTHealth, Houston, TX
Background/Purpose: Early loss of skin-associated adipose tissue and concomitant replacement by extracellular matrix is a hallmark of systemic sclerosis (SSc). However, the contribution of adipose…
Abstract Number: 0416 • ACR Convergence 2020
IgG4-related Retroperitoneal Fibrosis. Retrospective Cohort
Ernestina Angarola¹, Federico Jauk², Verónica Peuchot³, Maria Orlova¹, Melina Valeo², Javier Pollan¹ and Hernán Garcia Rivello², ¹Department of Internal Medicine, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina, Ciudad Autonoma de Buenos Aires, Ciudad Autonoma de Buenos Aires, Argentina, ²Department of Pathology, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina, Ciudad Autonoma de Buenos Aires, Ciudad Autonoma de Buenos Aires, Argentina, ³Internal Medicine Research Unit, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina, Ciudad Autonoma de Buenos Aires, Ciudad Autonoma de Buenos Aires, Argentina
Background/Purpose: The spectrum of IgG4-related disease (IgG4-RD) includes many diseases that were thought to be confined to a single organ, as the retroperitoneal fibrosis. Many…
Abstract Number: 0417 • ACR Convergence 2020
Different Immunophenotypes Characterized IgG4-Related Disease Clinical Phenotypes
Eduardo Martin-Nares¹, Janette Furuzawa-Carballeda¹, Ángel Alexis Priego-Ranero¹, Isela Chan-Campos¹, Gladys Sulikey Herrera-Noguera¹, Fidel López-Verdugo¹ and Gabriela Hernandez-Molina¹, ¹Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Distrito Federal, Mexico
Background/Purpose: Patients with IgG4-related disease (IgG4-RD) can be classified in clinical phenotypes which differ in terms of demographics, clinical and serological features. Whether there are…
Abstract Number: 0947 • ACR Convergence 2020
STING Gain-of-Function in Radio-resistant Cells Supports a Lymphocyte Dependent Auto-inflammatory Lung Disease
Kevin Gao¹, Mona Motwani¹, Ann Marshak-Rothstein² and Katherine Fitzgerald¹, ¹University of Massachusetts medical school, worcester, MA, ²University of Massachusetts medical school, Newton, MA
Background/Purpose: cGAS-STING is a cytosolic dsDNA sensing pathway whose regulation is vital to immune homeostasis. Pediatric patients with constitutively active STING mutations develop an autoinflammatory…
Abstract Number: 1913 • ACR Convergence 2020
Investigation of the Cellular Mechanism for Estradiol-Induced Dermal Fibrosis
DeAnna Baker Frost¹, Alisa Savchenko², Adeyemi Ogunleye³, Milton Armstrong² and Carol Feghali-Bostwick⁴, ¹The Medical University of South Carolina, Charleston, ²Medical University of South Carolina, Charleston, SC, ³University of North Carolina Chapel Hill, Chapel Hill, NC, ⁴The Medical University of South Carolina, Charleston, SC
Background/Purpose: Estradiol (E2), one of the three forms of estrogen, is pro-fibrotic in the skin because it positively influences ECM production in the dermis. In…

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