Results 61 to 70 of about 1,455 (196)

Severe Adult HLH/MAS With SPTCL‐Like Panniculitis: A Phenotype‐Guided, Resource‐Adapted Therapeutic Strategy Without Cytotoxic Therapy

Clinical Case Reports, Volume 14, Issue 5, May 2026.
Graphical abstract illustrating the phenotype‐guided therapeutic approach in severe adult HLH/MAS with SPTCL‐like panniculitis, demonstrating clinical and biochemical response following sequential treatment with therapeutic plasma exchange, intravenous immunoglobulin, and cyclosporine.
Hatem Mousa Taha, Omar Marouf, Khaled Hatem Taha   +2 more
wiley   +1 more source

Efficacy and safety of conventional disease-modifying antirheumatic drugs in VEXAS syndrome: real-world data from the international AIDA network [PDF]

Background: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an adult-onset autoinflammatory condition resulting in severe, often treatment-refractory inflammation.
Araujo, Olga, Ayumi Kawakami-Campos, Perla, Baggio, Chiara, Balistreri, Alberto, BATU AKAL, EZGİ DENİZ, Beecher, Mark, Bindoli, Sara, Bixio, Riccardo, Bocchia, Monica, Caggiano, Valeria, Callisto, Alicia, Campochiaro, Corrado, Cantarini, Luca, Cauli, Alberto, Conticini, Edoardo, Cordeiro, Rafael Alves, Crisafulli, Francesca, D'Agostino, Maria Antonietta, Dagna, Lorenzo, De Paulis, Amato, de-la-Torre, Alejandra, Fabiani, Claudia, Franceschini, Franco, Frassi, Micol, Frediani, Bruno, Gavioli, Francesco, Giardini, Henrique A. Mayrink, Gomez-Caverzaschi, Veronica, Gonzalez-Garcia, Andres, Guaracha-Basanez, Guillermo Arturo, Gurnari, Carmelo, Hernandez-Rodriguez, Jose, Hinojosa-Azaola, Andrea, Hissaria, Pravin, Iannone, Florenzo, Italian Soc Rheumatology SIR, Italian Soc Rheumatology SIR, Jahnz-Rozyk, Karina, La Torre, Francesco, Leone, Flavia, Lopalco, Giuseppe, Martin-Nares, Eduardo, Montecucco, Carlomaurizio, Monti, Sara, Mormile, Ilaria, Pena-Rodriguez, Mercedes, Piga, Matteo, Ragab, Gaafar, Ruiz-Irastorza, Guillermo, Sbalchiero, Jessica, Sfriso, Paolo, Sota, Jurgen, Soto-Peleteiro, Adriana, Tomelleri, Alessandro, Torres-Ruiz, Jiram, Triggianese, Paola, TUFAN, ABDURRAHMAN, VASİ, İBRAHİM, Viapiana, Ombretta, Vitale, Antonio, Vitetta, Rosetta, Wiesik-Szewczyk, Ewa   +60 more
core   +3 more sources

Navigating through uncertainty—Experience from the UK national VEXAS MDT

British Journal of Haematology, Volume 208, Issue 4, Page 1306-1313, April 2026.
Summary The objective of this study was to describe the establishment, structure and influence of the United Kingdom national multidisciplinary team (MDT) for vacuoles, E1 enzyme, X‐linked, autoinflammatory, somatic (VEXAS) syndrome and to assess its clinical outputs and perceived value among participating clinicians.
Daniel Pietsch, Austin Kulasekararaj, Sinisa Savic, on behalf of VEXNET‐UK MDT, Adam Al‐Hakim, Tanya Basu, Catherine Cargo, Onima Chowdhry, James Galloway, Eiphyu Htut, Stephen Jolles, Arvind Kaul, Helen Lachmann, Calman A. MacLennan, Anoop Mistry, Elspeth Payne, James Poulter, Farzana Rahman, Manoj Raghavan, Rachel Tattersall, Roochi Trikha, Villyn Yong, Taryn Youngstein, Anna Babb, Celia Beynon, Sarah Bingham, Nuno Borges, Jenny Bosworth, Carlos Campani, Marian Chan, Shikha Chattree, Niall Conlon, Robert Corser, Elena Ganendra, Paraskevi Gkreka, William Gordon, Elisabeth Grey‐Davies, Joanna Haughton, Simona Huica, Pawel Kaczmarek, Alison Laing, Areti Makrygeorgou, Susanna Mathew, Jill Mccormick, Muhammad Mohsin, Vidhya Murthy, Sateesh Nagumantry, Ognjenka Savanovic‐Abel, Shaun Smale, Alex Sternberg, Rosemary Waller, Sarah Westbury   +51 more
wiley   +1 more source

American College of Rheumatology Guidance Statement for Diagnosis and Management of VEXAS Developed by the International VEXAS Working Group Expert Panel

Arthritis &Rheumatology, Volume 78, Issue 3, Page 509-522, March 2026.
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 Heiblig, 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 more
wiley   +1 more source

VEXAS syndrome: a Swiss national retrospective cohort study. [PDF]

VEXAS syndrome is a recently discovered monogenic auto-inflammatory disease caused by a somatic mutation in the UBA1 gene that manifests with rheumatologic and haematologic features.
Amstad, A., Balabanov, S., Blum, S., Bonadies, N., Bruecker, R., Canbek, A., Caratsch, L., Chitic, M., Chrysoula, M., Clottu, A., Coattrenec, Y., Comte, D., Efthymiou, A., Lötscher, F., Nilsson, J., Regli, D., Schreiber, C., Seebach, J., Seitz, L., Seitz, P., Vilinovszki, O., Wolff, L.   +21 more
core   +1 more source

VEXAS and Myelodysplastic Syndrome: An Interdisciplinary Challenge [PDF]

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently recognized systemic autoinflammatory disease caused by somatic mutations in hematopoietic progenitor cells.
Boyadzhieva, Zhivana, Jelas, Ivan, Kreutzinger, Virginie, Kübke, Jan Carl, le Coutre, Philipp, Oberender, Christian, Pankow, Anne, Schneider, Udo, Schröder, Sebastian, Stephan, Lars Uwe, Stintzing, Sebastian, Ziegeler, Katharina   +11 more
core   +1 more source

How I Manage Transplant Ineligible Patients with Myelodysplastic Neoplasms [PDF]

, 2022
Myelodysplastic neoplasms, formerly known as myelodysplastic syndromes (MDS), represent a group of clonal disorders characterized by a high degree of clinical and molecular heterogeneity, and an invariable tendency to progress to acute myeloid leukemia ...
Gurnari, Carmelo, Xie, Zhuoer, Zeidan, Amer M   +2 more
core   +1 more source

The VEXAS Syndrome: Uncontrolled Inflammation and Macrocytic Anaemia in a 77-Year-Old Male Patient

European Journal of Case Reports in Internal Medicine, 2021
The VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently described X-linked autoinflammatory condition caused by a somatic mutation of the UBA1 gene and characterized by an evolving phenotype. This includes inflammatory
Andreas Himmelmann, Rolf Brücker
doaj   +1 more source

A Case of VEXAS (Vacuoles, E1 Enzyme, X-Linked, Autoinflammatory, Somatic) Syndrome With Cardiac Involvement

Annals of Internal Medicine: Clinical Cases
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome was first described in late 2020. It occurs as the result of somatic mutations in the ubiquitin-activating enzyme UBA1.
Xiaocao Xu, Najiya Haque, Chiamaka Onah, Lauren Dudley   +3 more
doaj   +1 more source

Current status and prospects of diagnosis and treatment of VEXAS syndrome [PDF]

Zhenduanxue lilun yu shijian
VEXAS (Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently recognized autoinflammatory disease involving multiple systems, caused by somatic mutations in the ubiquitin-like modifier activating enzyme 1 (UBA1) gene.
QIAN Haozhou, CHANG Chunkang
doaj   +1 more source