Title :

Pleuropulmonary manifestations of VEXAS syndrome.

Author(s) :

Borie, Raphael [Auteur]
Physiopathologie et Epidémiologie des Maladies Respiratoires [PHERE (UMR_S_1152 / U1152)]
Debray, Marie-Pierre [Auteur]
Physiopathologie et Epidémiologie des Maladies Respiratoires [PHERE (UMR_S_1152 / U1152)]
Guedon, Alexis F. [Auteur]
CHU Saint-Antoine [AP-HP]
Mekinian, Arsène [Auteur]
CHU Saint-Antoine [AP-HP]
Terriou, Louis [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Lacombe, Valentin [Auteur]
Centre Hospitalier Universitaire d'Angers [CHU Angers]
Lazaro, Estibaliz [Auteur]
Service de médecine interne et maladies infectieuses [Bordeaux]
Meyer, Aurore [Auteur]
Centre Hospitalier Universitaire [Strasbourg] [CHU Strasbourg]
Mathian, Alexis [Auteur]
CHU Pitié-Salpêtrière [AP-HP]
Ardois, Samuel [Auteur]
Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Pontchaillou]
Vial, Guillaume [Auteur]
Université de Bordeaux [UB]
Moulinet, Thomas [Auteur]
Ingénierie Moléculaire et Physiopathologie Articulaire [IMoPA]
Terrier, Benjamin [Auteur]
Hôpital Cochin [AP-HP]
Jamilloux, Yvan [Auteur]
Hôpital de la Croix-Rousse [CHU - HCL]
Heiblig, Mael [Auteur]
Centre Hospitalier Lyon Sud [CHU - HCL] [CHLS]
Bouaziz, Jean-David [Auteur]
Hopital Saint-Louis [AP-HP] [AP-HP]
Zakine, Eve [Auteur]
Outh, Rodereau [Auteur]
Groslerons, Sylvie [Auteur]
Bigot, Adrien [Auteur]
Centre Hospitalier Régional Universitaire de Tours [CHRU Tours]
Flamarion, Edouard [Auteur]
Hôpital Européen Georges Pompidou [APHP] [HEGP]
Kostine, Marie [Auteur]
Centre Hospitalier Universitaire de Bordeaux [CHU Bordeaux]
Henneton, Pierrick [Auteur]
Centre Hospitalier Régional Universitaire [Montpellier] [CHRU Montpellier]
Humbert, Sebastien [Auteur]
Centre Hospitalier Régional Universitaire de Besançon [CHRU Besançon]
Constantin, Arnaud [Auteur]
Université Toulouse III - Paul Sabatier [UT3]
Samson, Maxime [Auteur]
CHU Dijon
Bertrand, Nadine Magy [Auteur]
Centre Hospitalier Régional Universitaire de Besançon [CHRU Besançon]
Biscay, Pascal [Auteur]
Université de Bordeaux [UB]
Dieval, Celine [Auteur]
Lobbes, Hervé [Auteur]
CHU Estaing [Clermont-Ferrand]
Jeannel, Juliette [Auteur]
Centre Hospitalier Universitaire [Strasbourg] [CHU Strasbourg]
Servettaz, Amélie [Auteur]
Hôpital universitaire Robert Debré [Reims] [CHU Reims]
Adelaide, Leo [Auteur]
Graveleau, Julie [Auteur]
Centre hospitalier de Saint-Nazaire
De Sainte-Marie, Benjamin [Auteur]
Hôpital de la Timone [CHU - APHM] [TIMONE]
Galland, Joris [Auteur]
Centre Hospitalier de Bourg en Bresse
Guillotin, Vivien [Auteur]
Centre Hospitalier Universitaire de Bordeaux [CHU Bordeaux]
Duroyon, Eugenie [Auteur]
Hôpital Cochin [AP-HP]
Templé, Marie [Auteur]
Hôpital Cochin [AP-HP]
Bourguiba, Rim [Auteur]
CHU Tenon [AP-HP]
Georgin Lavialle, Sophie [Auteur]
CHU Tenon [AP-HP]
Kosmider, Olivier [Auteur]
Hôpital Cochin [AP-HP]
Audemard-Verger, Alexandra [Auteur]
Centre Hospitalier Régional Universitaire de Tours [CHRU Tours]

Journal title :

Chest

Abbreviated title :

Chest

Volume number :

163

Pages :

575-585

Publication date :

2022-10-26

ISSN :

1931-3543

English keyword(s) :

interstitial lung disease
janus kinase inhibitors
myelodysplasia
organizing pneumonia
pleural effusion

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

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 ...
Show more >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 between November 2020 and May 2021. Each patient included in the study who had an available chest CT scan was discussed in an adjudication multidisciplinary team and classified as showing potentially pleuropulmonary-specific involvement of VEXAS syndrome or others. Results Fifty-one patients had a CT scan available for review and 45 patients (39%) showed pleuropulmonary abnormalities on chest CT scan that were considered related to VEXAS syndrome after adjudication. Most patients were men (95%) with a median age 67.0 years at the onset of symptoms. Among these 45 patients, 44% reported dyspnea and 40% reported cough. All 45 patients showed lung opacities on chest CT scan (including ground-glass opacities [87%], consolidations [49%], reticulation [38%], and septal lines [51%]) and 53% of patients showed pleural effusion. Most patients showed improvement with prednisone, but usually required > 20 mg/d. The main clinical and biological features as well the median survival did not differ between the 45 patients with pleuropulmonary involvement and the rest of the cohort, suggesting that the prevalence of pleuropulmonary involvement might have been underdiagnosed in the rest of the cohort. Interpretation Pulmonary manifestations are frequent in VEXAS syndrome, but rarely are at the forefront. The initial outcome is favorable with prednisone and does not seem to lead to pulmonary fibrosis.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

Université de Lille
Inserm
CHU Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Submission date :

2024-01-12T00:15:57Z
2024-03-11T10:44:32Z