Pleuropulmonary Manifestations of Vacuoles, E1 Enzyme, X-Linked, Autoinflammatory, Somatic (VEXAS) Syndrome

被引：41

作者：

Borie, Raphael ^{[1
,9
]}

Debray, Marie Pierre ^{[2
,9
]}

Guedon, Alexis F. ^{[3
]}

Mekinian, Arsene ^{[3
]}

Terriou, Louis ^{[12
]}

Lacombe, Valentin ^{[13
]}

Lazaro, Estibaliz ^{[14
]}

Meyer, Aurore ^{[16
]}

Mathian, Alexis ^{[4
]}

Ardois, Samuel ^{[18
]}

Vial, Guillaume ^{[19
]}

Moulinet, Thomas ^{[21
]}

Terrier, Benjamin ^{[5
]}

Jamilloux, Yvan ^{[22
]}

Heiblig, Mael ^{[22
]}

Bouaziz, Jean-David ^{[7
]}

Zakine, Eve ^{[7
]}

Outh, Roderau ^{[23
]}

Groslerons, Sylvie ^{[24
]}

Bigot, Adrien ^{[11
]}

Flamarion, Edouard ^{[10
]}

Kostine, Marie ^{[20
]}

Henneton, Pierrick ^{[25
]}

Humbert, Sebastien ^{[26
]}

Constantin, Arnaud ^{[27
,28
]}

Samson, Maxime ^{[29
]}

Bertrand, Nadine Magy ^{[26
]}

Biscay, Pascal ^{[15
]}

Dieval, Celine ^{[30
]}

Lobbes, Herve ^{[31
]}

Jeannel, Juliette ^{[17
]}

Servettaz, Amelie ^{[32
]}

Adelaide, Leo ^{[33
]}

Graveleau, Julie ^{[34
]}

de Sainte-Marie, Benjamin ^{[35
]}

Galland, Joris ^{[36
]}

Guillotin, Vivien ^{[14
]}

Duroyon, Eugenie ^{[6
]}

Temple, Marie ^{[6
]}

Bourguiba, Rim ^{[8
]}

Lavialle, Sophie Georgin ^{[8
]}

Kosmider, Olivier ^{[6
]}

Audemard-Verger, Alexandra ^{[11
]}

机构：

[1] Hop Bichat Claude Bernard, Serv Pneumol, Paris, France

[2] Hop Bichat Claude Bernard, Serv Radiol, Paris, France

[3] Hop St Antoine, Serv Med Interne, Paris, France

[4] Hop La Pitie Salpetriere, Serv Med Interne 2, Paris, France

[5] Hop Cochin, Serv Med Interne, Paris, France

[6] Hop Cochin, Lab Hematol, Paris, France

[7] Hop St Louis, Serv Dermatol, Paris, France

[8] Hop Tenon, AP HP, Serv Med Interne, Paris, France

[9] Univ Paris, INSERM, U1152, Paris, France

[10] Univ Paris Cite, Hop Europeen Georges Pompidou, AP HP Ctr, Serv Med Interne, Paris, France

[11] CHU Bretonneau, Serv Med Interne & Immunol Clin, Tours, France

[12] CHRU Lille, Serv Med Interne, Lille, France

[13] CHU Angers, Serv Med Interne & Immunol Clin, Angers, France

[14] CHU Bordeaux, Hop Haut Eveque, Med Interne & Malad Infect, Pessac, France

[15] Clin Mutualiste Pessac Med Interne, Pessac, France

[16] CHU Strasbourg, Nouvel Hop Civil, Serv Immunol Clin & Med Interne, Strasbourg, France

[17] CHU Strasbourg, Nouvel Hop Civil, Serv Med Interne, Strasbourg, France

[18] Hop Pontchaillou, Serv Med Interne & Immunol Clin, Renne, France

[19] Hop St Andre, Med Interne & Immunol Clin, Bordeaux, France

[20] CHU Bordeaux, Serv Rhumatol, Bordeaux, France

[21] Univ Lorraine, Dept Med Interne & Immunol Clin, CHU Nancy, IMoPA,CNRS,UMR 7365, Nancy, France

[22] Ctr Hosp Lyon Sud, Hop La Croix Rousse, Serv Med Interne, Hematol, Lyon, France

[23] CHG Perpignan, Serv Med Interne, Perpignan, France

[24] CH Agen Nerac, CHU Serv Med Interne, Agen, France

[25] CHU Montpellier, Serv Med Vasc, Montpellier, France

[26] CHU Besancon, Serv Med Interne, Besancon, France

[27] Pierre Paul Riquet, Univ Hosp, Dept Rheumatol, Toulouse, France

[28] Toulouse III Paul Sabatier Univ, Toulouse, France

[29] CHU Dijon, Serv Med Interne & Immunol Clin, Dijon, France

[30] CHU Rochefort, Serv Med Interne, Rochefort, France

[31] CHU Clermont Ferrand, Hop Estaing, Serv Med Interne, Clermont Ferrand, France

[32] CHU Reims, Serv Med Interne, Malad Infect, Immunol Clin, Reims, France

[33] CHU Lucien Hussel, Serv Med Interne, Vienne, France

[34] CHU St Nazaire, Serv Med Interne, St Nazaire, France

[35] Hop Timone Marseille, Med Interne, Marseille, France

[36] Ctr Hosp Bourg En Bresse, Serv Med Interne, Bourg En Bresse, France

来源：

CHEST | 2023年 / 163卷 / 03期

关键词：

interstitial lung disease; janus kinase inhibitors; myelodysplasia; organizing pneumonia; pleural effusion; PREVALENCE;

D O I：

10.1016/j.chest.2022.10.011

中图分类号：

R4 [临床医学];

学科分类号：

1002 ; 100602 ;

摘要：

BACKGROUND: The vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a newly identified autoinflammatory disorder related to somatic UBA1 muta-tions. 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 multi-disciplinary 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 predni-sone, 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.CHEST 2023; 163(3):575-585

引用

页码：575 / 585

页数：11

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