Article
Pleuropulmonary Manifestations of Vacuoles, E1 Enzyme, X-Linked, Autoinflammatory, Somatic (VEXAS) Syndrome.
Chest - 1 Mar 2023
Borie Raphael, Debray Marie Pierre, Guedon Alexis F, Mekinian Arsene, Terriou Louis, Lacombe Valentin, Lazaro Estibaliz, Meyer Aurore, Mathian Alexis, Ardois Samuel, Vial Guillaume, Moulinet Thomas, Terrier Benjamin, Jamilloux Yvan, Heiblig Mael, Bouaziz Jean-David, Zakine Eve, Outh Roderau, Groslerons Sylvie, Bigot Adrien, Flamarion Edouard, Kostine Marie, Henneton Pierrick, Humbert Sebastien, Constantin Arnaud, Samson Maxime, Bertrand Nadine Magy, Biscay Pascal, Dieval Celine, Lobbes Herve, Jeannel Juliette, Servettaz Amelie, Adelaide Leo, Graveleau Julie, de Sainte-Marie Benjamin, Galland Joris, Guillotin Vivien, Duroyon Eugénie, Templé Marie, Bourguiba Rim, Georgin Lavialle Sophie, Kosmider Olivier, Audemard-Verger Alexandra
Abstract excerpt
BACKGROUND: The vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a newly identified autoinflammatory disorder related to somatic UBA1 mutations. Up to 72% of patients may show lung involvement. RESEARCH QUESTION: What are the pleuropulmonary manifestations in VEXAS syndrome? STUDY DESIGN AND METHODS: One hundred fourteen patients were included in the French cohort of VEXAS syndrome...
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