Results 51 to 60 of about 940 (155)

VEXAS syndrome in a Moroccan patient: the story of a two-year diagnostic lag

European Journal of Case Reports in Internal Medicine
Background: VEXAS syndrome, also known as vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome, is a newly identified genetic condition characterised by a combination of autoinflammatory symptoms and myeloid dysplasia.
Abire Allaoui, Lilia Zizi, Halima Hadri, Doha Kabil, Ahmed Aziz Bousfiha, Abdelhamid Naitlho, Mohamed Adnaoui   +6 more
doaj   +1 more source

Candida oesophagitis as an Initial Manifestation of VEXAS Syndrome: Expanding Evidence for an Acquired Immunodeficiency Phenotype

eJHaem, Volume 7, Issue 4, August 2026.
Demi Rianne Vintges, Marina Weerheim, Faiz Karim   +2 more
wiley   +1 more source

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

VEXAS syndrome as a cause for multifocal, relapsing head and neck inflammation

Clinical Case Reports
Key Clinical Message VEXAS syndrome (vacuoles, E1 enzyme, X‐linked, autoinflammatory, somatic) is a novel autoinflammatory syndrome. We describe a case of VEXAS syndrome with upper airway and oral cavity involvement which are not well described in the ...
Aoife Heeney, Rachael Wu, Conall Fitzgerald, Nina Orfali, Nadim Akasheh, Conor Magee   +5 more
doaj   +1 more source

Schnitzler Syndrome as an Autoinflammatory Disease Driven by B‐Cell‐Specific Somatic MYD88 Mutation

Allergy, Volume 81, Issue 8, Page 2969-2971, August 2026.
Yuyi Zhou, Yoko Ueki, Naoya Iwata, Hiroshi Oue, Kentaro Kato, Mengyan Li, Kazushi Izawa, Tomoyasu Jo, Seishi Ogawa, Kenji Kabashima, Naotomo Kambe   +10 more
wiley   +1 more source

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

Distinct Pattern of Atypical Megakaryocytes in VEXAS Syndrome

International Journal of Laboratory Hematology, Volume 48, Issue 4, Page 713-714, August 2026.
Andrew Y. Sung, Jackson W. Douglas, Olga K. Weinberg, Sharon K. Germans, Weina Chen   +4 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

A clinical phenotype of VEXAS syndrome with pleural effusion, infiltrates, and systemic inflammation in a 76-year-old patient: a case report

Journal of Medical Case Reports
Introduction VEXAS syndrome, characterized by a UBA1 gene mutation, is a rare and severe systemic inflammatory disease predominantly affecting men. Since its initial description in 2020, it has been noted for its broad clinical phenotype and frequent ...
Melanie Berger, Falk Schumacher, Maximilian Wollsching-Strobel, Doreen Kroppen, Sarah B. Stanzel, Daniel S. Majorski, Kathrin Fricke, Ilka Plath, Wolfram Windisch, Maximilian Zimmermann   +9 more
doaj   +1 more source

Macrocytosis combined with monoclonal gammopathy identifies patients carrying VEXAS‐linked UBA1 mutations

HemaSphere, Volume 10, Issue 7, July 2026.
Verena Petzer, Wolfram Mayr, Eva Spoegler, Emina Jukic, Christoph Winkler, Verena Vogi, Normann Steiner, Christina Duftner, Andrea Griesmacher, Günter Weiss, Dominik Wolf, Florian Kocher, David Haschka   +12 more
wiley   +1 more source