Results 31 to 40 of about 954 (158)

Development of a Disease Activity Index for the Assessment of VEXAS Syndrome (VEXAS‐DAI)

Arthritis Care &Research, EarlyView.
Objective Vacuoles, E1 enzyme, X‐linked, autoinflammatory, somatic syndrome (VEXAS) syndrome is characterized by a complex spectrum of inflammatory and hematologic manifestations. Clinical research to identify effective therapies is urgently needed but is hindered by the lack of validated outcome measures.
Kevin Byram, Heřman Mann, Danielle Hammond, Onima Chowdhury, Sinisa Savic, Yohei Kirino, Carmelo Gurnari, Maël Heiblig, Thibault Comont, Arsène Mekinian, Mrinal M. Patnaik, Lachelle D. Weeks, Gary Ho, Adam Al‐Hakim, Scott Goldberg, Marcela A. Ferrada, Sophie Georgin‐Lavialle, Peter C. Grayson, Emma M. Groarke, Bhavisha Patel, Megan Sullivan, Sarah A. Buckley, Bryan G. Harder, Raman Garcha, David B. Beck, Matthew J. Koster   +25 more
wiley   +1 more source

Think VEXAS: a case report of Vexas syndrome

Journal of Rare Diseases
AbstractVEXAS syndrome is a rare auto-inflammatory disorder characterized by heterogeneous inflammatory and hematologic features. First identified in 2020, it predominantly affects men over the age of 50. Clinical manifestations commonly include recurrent fever, weight loss, skin lesions, and diverse inflammatory presentations across multiple organ ...
T. Najdi, S. Karaa
openaire   +2 more sources

Improving Diagnosis and Clinical Management of Acquired Systemic Autoinflammatory Diseases

Journal of Inflammation Research, 2022
Adam Al-Hakim,1 Anoop Mistry,1 Sinisa Savic1,2 1Department of Clinical Immunology and Allergy, St James’s University Hospital, Leeds, UK; 2Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UKCorrespondence: Sinisa ...
Al-Hakim A, Mistry A, Savic S
doaj  

Therapeutic Outcomes in VEXAS Syndrome: A Multicenter Comparative Cohort of Allogeneic Hematopoietic Stem Cell Transplantation and Hypomethylating Agents

American Journal of Hematology, Volume 101, Issue 9, Page 2166-2176, September 2026.
ABSTRACT Hypomethylating agents (HMA) and allogeneic hematopoietic stem cell transplantation (alloHSCT) have both demonstrated remissions in VEXAS; however, comparative data is lacking. We conducted a multicenter, retrospective analysis of 66 patients diagnosed with VEXAS syndrome treated with HMA (n = 35) or alloHSCT (n = 31). Baseline characteristics
Saubia Fathima, Margaret Mei‐Kay Wong, Jesus Gonzalez‐Lugo, Susan M. Geyer, Ali Alsugair, Maria Sirenko, Kimberly J. Langer, Terra L. Lasho, Christy Finke, Jun Choi, Maher Abdul‐Hay, Gary Ho, Mark R. Litzow, Aasiya Matin, Urshila Durani, Mehrdad Hefazi, William J. Hogan, Mithun V. Shah, Aref Al‐Kali, Kebede H. Begna, Naseema Gangat, Antoine N. Saliba, Ronald S. Go, Tariq Kewan, Gabriel Bartoo, Jade Kutzke, Kristen McCullough, Kenneth J. Warrington, Megan Sullivan, Kaaren K. Reichard, Horatiu Olteanu, Hemant Murthy, Talha Badar, Yael Kusne, Jeanne Palmer, Saurabh Chhabra, Nathan Punwani, Mary Riwes, Joseph P. McGuirk, Elizabeth F. Krakow, Amelia Langston, Taxiarchis Kourelis, David Dingli, James Foran, Matthew J. Koster, Mrinal M. Patnaik, David B. Beck, Hassan B. Alkhateeb, Abhishek A. Mangaonkar   +48 more
wiley   +1 more source

Case report: VEXAS syndrome and literature review

Frontiers in Hematology
VEXAS syndrome (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) is a novel disorder first described in 2020. Patients are diagnosed by identifying a somatic mutation of the ubiquitin-like modifier-activating enzyme 1 (UBA1) gene.
Can Jones, Can Jones, Stanislav Ivanov, Pablo Ferraro, Pablo Ferraro, Souhad Younes, Souhad Younes, Hugo Fernandez   +7 more
doaj   +1 more source

Case Report: Coexistence of Multiple Myeloma and Auricular Chondritis in VEXAS Syndrome

Frontiers in Immunology, 2022
Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is an inflammatory disorder caused by somatic UBA1 variants, which are sometimes associated with hematological disorders, including myelodysplastic syndrome (MDS).
Haruki Matsumoto, Yuya Fujita, Masahiko Fukatsu, Takayuki Ikezoe, Kohei Yokose, Tomoyuki Asano, Naomi Tsuchida, Naomi Tsuchida, Naomi Tsuchida, Ayaka Maeda, Shuhei Yoshida, Honami Hashimoto, Jumpei Temmoku, Naoki Matsuoka, Makiko Yashiro-Furuya, Shuzo Sato, Mai Murakami, Hidenori Sato, Chiharu Sakuma, Kazumasa Kawashima, Norshalena Shakespear, Yuri Uchiyama, Yuri Uchiyama, Hiroshi Watanabe, Yohei Kirino, Naomichi Matsumoto, Kiyoshi Migita   +26 more
doaj   +1 more source

Ruxolitinib Pharmacokinetics and Exposure–Toxicity Relationship in Hematologic Malignancies and Immune‐Mediated Diseases: A Prospective Observational Study

Clinical Pharmacology &Therapeutics, Volume 120, Issue 3, Page 753-761, September 2026.
Ruxolitinib pharmacokinetics (PK) has been characterized in clinical trials but remains poorly documented in real‐world practice. This project aimed to investigate ruxolitinib PK in routine clinical practice, identify factors driving its variability, and explore exposure–response relationships to assess the potential role of therapeutic drug monitoring.
Jérémie Tachet, Sara C. Meyer, Francesco Grandoni, Emmanuel Haefliger, Monika Nagy‐Hulliger, Jörg P. Halter, Jakob R. Passweg, David Haefliger, Carine Bardinet, Laurent A. Decosterd, Monia Guidi, François R. Girardin   +11 more
wiley   +1 more source

An Italian cartography of VEXAS‐related thrombosis

HemaSphere, Volume 10, Issue 8, August 2026.
Abstract Thrombotic events (TEs) occur in up to 40% of patients with vacuoles, E1 enzyme, X‐linked, autoinflammatory, and somatic (VEXAS) syndrome, but data on its clinical‐genomics features and anticoagulation strategies are limited. To gain more insight into this, we conducted a two‐step study evaluating the prevalence and outcome of TE in VEXAS ...
Giorgia Ranucci, Maria Rosaria Pascale, Vittorio Forte, Elisa Diral, Corrado Campochiaro, Jacqueline Ferrari, Mariachiara Cuccaro, Chiara Cattaneo, Francesca Crisafulli, Lucia Cardillo, Valeria Renola, Elisa Meddi, Marianna Velocci, Elisa Casciani, Francesca Romano, Pellegrino Musto, Serafina Barbato, Giorgia Battipaglia, Marco Frigeni, Erika Morsia, Arianna Savi, Cristina Papayannidis, Elena Crisà, Laura Cicconi, Isabella Capodanno, Alfonso Fiumarella, Antonio Pierini, Massimo Breccia, Giovanni Orsolini, Giulia Rivoli, Sara Bindoli, Davide Firinu, Antonio Vitale, Bruno Fattizzo, Monica Bocchia, Luca Cantarini, Chiara Pinton, Gabriele Todisco, Nicola Toschi, Paola Triggianese, Adriano Venditti, Maria Teresa Voso, Carmelo Gurnari   +42 more
wiley   +1 more source

A case of VEXAS syndrome presenting with unusual bone marrow granulomas: a diagnostic dilemma

BMC Rheumatology, 2023
Background VEXAS is a recently described inflammatory disease caused by mutations in the UBA1 gene. Symptoms are diverse and include fevers, cartilaginous inflammation, lung inflammation, vasculitis, neutrophilic dermatoses, and macrocytic anemia ...
Khiem T. Vu, Rachel M. Wolfe, Jonathan E. Lambird, Danielle L. V. Maracaja   +3 more
doaj   +1 more source

A patient with VEXAS syndrome presenting with complete ophthalmoplegia

European Journal of Case Reports in Internal Medicine
Introduction: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently identified, somatic mutation-driven, autoinflammatory syndrome primarily affecting older males.
James Krzowski, Luke Harvey, Emily Mitchell, Vivek Rajagopal   +3 more
doaj   +1 more source