Results 231 to 240 of about 698,939 (333)
Expert Perspective: Diagnosis and Treatment of Castleman Disease
Arthritis &Rheumatology, EarlyView.Castleman disease (CD) is a major diagnostic challenge for rheumatologists. Unicentric CD (UCD) involves one enlarged lymph node region, whereas multicentric CD (MCD) involves multiple enlarged lymph node regions. Both UCD and MCD may exhibit a wide range of symptoms that overlap with other immune‐mediated conditions.Luke Y. C. Chen, Lu Zhang, David C. Fajgenbaum +2 morewiley +1 more sourceLoss of Tripartite Motif–Containing Protein 21 and UVB‐Induced Systemic Inflammation by Regulating DNA‐Sensing Pathways
Arthritis &Rheumatology, EarlyView.Objective
Patients with systemic lupus erythematosus (SLE) experience photosensitivity, with exposure to UVB light driving lupus flares and triggering symptoms like joint pain, fatigue, and cutaneous lesions. Although the mechanism(s) linking UVB exposure to systemic effects are unclear, type I interferons (IFNs) are known to play a role.Gantsetseg Tumurkhuu, Graziela Perri, Richard Moore, Lihong Huo, Arati Naveen Kumar, Richard Ainsworth, Trinitee Oliver, Gabriela De Los Santos, David Gibb, Jessica Carriere, Jeong Min Yu, Rachel Abuav, Lindsy Forbess, Daniel J. Wallace, Mariko Ishimori, Wonwoo Shon, Christian Stehlik, Caroline A. Jefferies +17 morewiley +1 more sourceAmerican College of Rheumatology Guidance Statement for Diagnosis and Management of VEXAS Developed by the International VEXAS Working Group Expert Panel
Arthritis &Rheumatology, EarlyView.Objective
Vacuoles E1 enzyme X‐linked autoinflammatory somatic syndrome (VEXAS) is a recently identified rare genetic disorder associated with somatic mutations in the UBA1 gene. VEXAS presents with a combination of inflammatory and hematologic manifestations, leading to increased morbidity and mortality.Arsene Mekinian, Sophie Georgin‐Lavialle, Marcela A. Ferrada, Sinisa Savic, Matthew J. Koster, Olivier Kosmider, Thibault Comont, Mael Heilblig, Juan I. Arostegui, Annmarie Bosco, Rim Bourguiba, Katherine R. Calvo, Catherine Cargo, Chiara Cattaneo, François Chasset, Henrique Coelho, Corrado Campochiaro, Francesca Crisafulli, Stephanie Ducharme‐Benard, Raquel Faria, Franco Franceschini, Micol Frassi, Emma M. Groarke, Carmelo Gurnari, Yervand Hakobyan, Yvan Jamilloux, Ciprian Jurcut, Yohei Kirino, Austin Kulasekararaj, Hiroyoshi Kunimoto, Lauren M. Madigan, Heřman F. Mann, Chiara Marvisi, Marcin Milchert, Sara Morais, Katja Sockel, Francesco Muratore, Hideaki Nakajima, Mrinal M. Patnaik, Luísa Regadas, Marie Robin, Abraham Rutgers, Carlo Salvarani, Anthony M. Sammel, Joerg Seebach, Pierre Sujobert, Alessandro Tomelleri, Geoffrey Urbanski, Frédéric Vandergheynst, Romana Vieira, David S. Viswanatha, Ewa Więsik‐Szewczyk, Elisa Diral, Benjamin Terrier, Bhavisha A. Patel, Pierre Fenaux, Peter C. Grayson, David B. Beck, on behalf of the International VEXAS working group, and with endorsement of EuroBloodNet, the European Reference Network in Rare Hematological Diseases, Heřman Mann, Benjamin Terrier, François Chasset, Sophie Georgin Lavialle, Alessandro Tomelleri, Campochiaro Corrado, Carlo Salvarani, Francesca Crisafulli, Franco Franceschini, Micol Frassi, Yohei Kirino, Ewa Więsik‐Szewczyk, Marcin Milchert, Raquel Faria, Ciprian Jurcut, Joerg Seebach, Sinisa Savic, David Beck, Lauren Madigan, Matthew Koster, Patnaik Mrinal, Olivier Kosmider, Pierre Sujobert, Juan I. Arostegui, Catherine Cargo, David Viswanatha, Yervand Hagopian, Mael Heilblig, Pierre Fenaux, Thibault Comont, Bruno Alessandro, Chiara Cattaneo, Elisa Diral, Sara Morais, Austin Kulasekarara, Emma Groarke, Katherine Calvo, Patel Bhavisha, Anthony Sammel, Arsene Mekinian, Benjamin Terrier, Marie Robin, Sophie Georgin Lavialle, Katja Sockel, Yvan Jamilloux, Carmelo Gurnari, Henrique Coelho, Romana Vieira, Rim Bourguiba, Marcela Ferrada, Peter Grayson +111 morewiley +1 more sourceEpidermal Interferon‐κ Drives Cutaneous Lupus‐Like Lesions, Photosensitivity, and Systemic Autoimmunity In Vivo
Arthritis &Rheumatology, EarlyView.Objective
Keratinocyte‐derived interferon (IFN) κ is chronically overexpressed in human nonlesional systemic lupus erythematosus (SLE) skin. Recent evidence suggests that epidermal signals instruct the immune system in SLE, but whether epidermal IFNκ alone is sufficient to drive lupus phenotypes has not been investigated.Benjamin Klein, Deborah J. Colesa, Yiqing Gao, Patrick A. O'Brien, Lin Zhang, Lori Lowe, Kelsey E. McNeely, Nguyen Thi Kim Nguyen, Svenja Henning, Mehrnaz Gharaee‐Kermani, Jeffrey B. Hodgin, Jacob W. S. Martens, Johann E. Gudjonsson, Celine C. Berthier, J. Michelle Kahlenberg +14 morewiley +1 more source