دورية أكاديمية
Pleuropulmonary Manifestations of Vacuoles, E1 Enzyme, X-Linked, Autoinflammatory, Somatic (VEXAS) Syndrome.
| العنوان: | Pleuropulmonary Manifestations of Vacuoles, E1 Enzyme, X-Linked, Autoinflammatory, Somatic (VEXAS) Syndrome. |
|---|---|
| المؤلفون: | Borie R; Service de Pneumologie A, Hôpital Bichat, APHP, Paris, France; INSERM, Unité 1152, Université de Paris, Paris, France. Electronic address: raphael.borie@aphp.fr., Debray MP; Service de Radiologie, Hôpital Bichat, APHP, Paris, France; INSERM, Unité 1152, Université de Paris, Paris, France., Guedon AF; Service de Médecine Interne, Hôpital St. Antoine, APHP, Paris, France., Mekinian A; Service de Médecine Interne, Hôpital St. Antoine, APHP, Paris, France., Terriou L; Service de Médecine Interne, CHRU de Lille, France., Lacombe V; Service de Médecine Interne et Immunologie Clinique, CHU d'Angers, Angers, France., Lazaro E; Médecine Interne et Maladies Infectieuses, Hôpital Haut l'Evêque, CHU de Bordeaux, Pessac, France., Meyer A; Service d'Immunologie Clinique et Médecine Interne, Nouvel Hôpital Civil, CHU Strasbourg, Strasbourg, France., Mathian A; Service de Médicine Interne 2, Hôpital de la Pitié Salpêtrière, APHP, Paris, France., Ardois S; Service de Médecine Interne et Immunologie Clinique, Hôpital Pontchaillou, Renne, France., Vial G; Médecine Interne et Immunologie Clinique, Hôpital Saint André, CHU Bordeaux, Bordeaux, France., Moulinet T; Département de Médecine Interne et Immunologie Clinique, CHU Nancy, UMR 7365, IMoPA, University of Lorraine, CNRS, Nancy, France., Terrier B; Service de Médecine Interne, Hôpital Cochin, APHP, Paris, France., Jamilloux Y; Service de Médecine Interne, Hôpital de la Croix Rousse, Hématologie, Centre Hospitalier de Lyon Sud, Pierre Bénite, Lyon, France., Heiblig M; Service de Médecine Interne, Hôpital de la Croix Rousse, Hématologie, Centre Hospitalier de Lyon Sud, Pierre Bénite, Lyon, France., Bouaziz JD; Service de Dermatologie, Hopital St. Louis, APHP, Paris, France., Zakine E; Service de Dermatologie, Hopital St. Louis, APHP, Paris, France., Outh R; Service de Médecine Interne, CHG Perpignan, Perpignan, France., Groslerons S; CHU Service de Médecine Interne, CH Agen Nerac, Agen, France., Bigot A; Service de Médecine Interne et Immunologie Clinique, CHU Bretonneau, Tours, France., Flamarion E; Service de Médecine Interne, Hôpital Européen Georges Pompidou, APHP-Centre, Université de Paris Cité, Paris, France., Kostine M; Service de Rhumatologie, CHU Bordeaux, Bordeaux, France., Henneton P; Service de Médecine Vasculaire, CHU de Montpellier, Montpellier, France., Humbert S; Service de Médecine Interne, CHU de Besançon, Besançon, France., Constantin A; Department of Rheumatology, Pierre-Paul Riquet University Hospital, and Toulouse III-Paul Sabatier University, Toulouse, France., Samson M; Service de Médecine Interne et Immunologie Clinique, CHU de Dijon, Dijon, France., Bertrand NM; Service de Médecine Interne, CHU de Besançon, Besançon, France., Biscay P; Clinique Mutualiste Pessac Médecine Interne, Pessac, France., Dieval C; Service de Médecine Interne, CHU Rochefort, Rochefort, France., Lobbes H; Service de Médecine Interne, CHU de Clermont-Ferrand, Hôpital Estaing, Clermont-Ferrand, France., Jeannel J; Service de Médecine Interne, Nouvel Hôpital Civil, CHU Strasbourg, Strasbourg, France., Servettaz A; Service de Médecine Interne, Maladies Infectieuses, Immunologie Clinique, CHU de Reims, Reims, France., Adelaide L; Service de Médecine Interne, CHU Lucien Hussel, Vienne, France., Graveleau J; Service de Médecine Interne, CHU Saint-Nazaire, Saint-Nazaire, France., de Sainte-Marie B; Médecine Interne, Hôpital de la Timone-Marseille, Marseille, France., Galland J; Service de Médecine Interne, Centre Hospitalier de Bourg-en-Bresse, Bourg-en-Bresse, France., Guillotin V; Médecine Interne et Maladies Infectieuses, Hôpital Haut l'Evêque, CHU de Bordeaux, Pessac, France., Duroyon E; Laboratoire d'Hématologie, Hôpital Cochin, APHP, Paris, France., Templé M; Laboratoire d'Hématologie, Hôpital Cochin, APHP, Paris, France., Bourguiba R; Service de Médecine Interne, Hôpital Tenon, APHP, Paris, France., Georgin Lavialle S; Service de Médecine Interne, Hôpital Tenon, APHP, Paris, France., Kosmider O; Laboratoire d'Hématologie, Hôpital Cochin, APHP, Paris, France., Audemard-Verger A; Service de Médecine Interne et Immunologie Clinique, CHU Bretonneau, Tours, France. |
| مؤلفون مشاركون: | French VEXAS Group |
| المصدر: | Chest [Chest] 2023 Mar; Vol. 163 (3), pp. 575-585. Date of Electronic Publication: 2022 Oct 20. |
| نوع المنشور: | Journal Article; Research Support, Non-U.S. Gov't |
| اللغة: | English |
| بيانات الدورية: | Publisher: Elsevier Country of Publication: United States NLM ID: 0231335 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1931-3543 (Electronic) Linking ISSN: 00123692 NLM ISO Abbreviation: Chest Subsets: MEDLINE |
| أسماء مطبوعة: | Publication: 2016- : New York : Elsevier Original Publication: Chicago : American College of Chest Physicians |
| مواضيع طبية MeSH: | Vacuoles* , Pulmonary Fibrosis*/pathology, Male ; Humans ; Aged ; Female ; Prednisone ; Lung/diagnostic imaging ; Lung/pathology ; Syndrome ; Mutation |
| مستخلص: | 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. (Copyright © 2022 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.) |
| فهرسة مساهمة: | Investigator: J Haroche; Z Amoura; M Pha; M Hie; K Meghit; M Rondeau-Lutz; JC Weber Keywords: interstitial lung disease; janus kinase inhibitors; myelodysplasia; organizing pneumonia; pleural effusion |
| المشرفين على المادة: | VB0R961HZT (Prednisone) |
| SCR Disease Name: | VEXAS syndrome |
| تواريخ الأحداث: | Date Created: 20221022 Date Completed: 20230313 Latest Revision: 20230327 |
| رمز التحديث: | 20231215 |
| DOI: | 10.1016/j.chest.2022.10.011 |
| PMID: | 36272567 |
| قاعدة البيانات: | MEDLINE |
Full Text via ClinicalKey 
Full Text Finder | تدمد: | 1931-3543 |
|---|---|
| DOI: | 10.1016/j.chest.2022.10.011 |