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Article Dans Une Revue Chest Année : 2022

Pleuropulmonary manifestations of VEXAS syndrome

1 PHERE (UMR_S_1152 / U1152) - Physiopathologie et Epidémiologie des Maladies Respiratoires
2 CHU Saint-Antoine [AP-HP]
3 LIRIC - Lille Inflammation Research International Center - U 995
4 CHU Angers - Centre Hospitalier Universitaire d'Angers
5 Service de médecine interne et maladies infectieuses [Bordeaux]
6 CHU Strasbourg - Centre Hospitalier Universitaire [Strasbourg]
7 CHU Pitié-Salpêtrière [AP-HP]
8 Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Ponchaillou]
9 UB - Université de Bordeaux
10 CHU Bordeaux
11 IMoPA - Ingénierie Moléculaire et Physiopathologie Articulaire
12 CHRU Nancy - Centre Hospitalier Régional Universitaire de Nancy
13 Hôpital Cochin [AP-HP]
14 Hôpital de la Croix-Rousse [CHU - HCL]
15 AP-HP - Hopital Saint-Louis [AP-HP]
16 Centre Hospitalier Saint Jean de Perpignan
17 Centre Hospitalier Agen-Nérac
18 CHRU Tours - Centre Hospitalier Régional Universitaire de Tours
19 HEGP - Hôpital Européen Georges Pompidou [APHP]
20 CHRU Montpellier - Centre Hospitalier Régional Universitaire [Montpellier]
21 CHRU Besançon - Centre Hospitalier Régional Universitaire de Besançon
22 UT3 - Université Toulouse III - Paul Sabatier
23 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
24 CHU Dijon
25 CH Rochefort - Centre Hospitalier de Rochefort
26 CHU Estaing [Clermont-Ferrand]
27 Hôpital universitaire Robert Debré [Reims]
28 CH Vienne - Centre Hospitalier de Vienne Lucien Hussel
29 Centre hospitalier de Saint-Nazaire
30 TIMONE - Hôpital de la Timone [CHU - APHM]
31 Centre Hospitalier de Bourg en Bresse
32 CHU Tenon [AP-HP]
Arsène Mekinian
Alexis Mathian
Marie Kostine
  • Fonction : Auteur
Maxime Samson
  • Fonction : Auteur
Vivien Guillotin
  • Fonction : Auteur
Rim Bourguiba
  • Fonction : Auteur

Résumé

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.

Dates et versions

hal-04498358 , version 1 (11-03-2024)

Identifiants

Citer

Raphael Borie, Marie-Pierre Debray, Alexis F. Guedon, Arsène Mekinian, Louis Terriou, et al.. Pleuropulmonary manifestations of VEXAS syndrome. Chest, 2022, Chest, 163 (3), pp.575-585. ⟨10.1016/j.chest.2022.10.011⟩. ⟨hal-04498358⟩
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