Pleuropulmonary Manifestations of VEXAS Syndrome
EUROPEAN RESPIRATORY JOURNAL（2022）
CHRU Lille | CHU Angers | CHRU Bordeaux | CHRU Strasbourg | Pitie Salpetriere | CHU Rennes | CH Bordeauxen BresseFerrand | CHU Nancy | Cochin | CHU Lyon | CH Perpignan | HEGP | CHU Bordeaux | CHu Montpellier | CHU Besancon | CHU Toulouse | CHU Dijon | Ch Rochefort | Clin Mutualiste | CH Clermond Ferrand | CHU Reims | CH Vienne | Ch St Nazaire | CHU Marseille | CH Bourg BresseFerrand | Tenon
Abstract
VEXAS (Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic) syndrome is a new autoinflammatory syndrome related to somatic UBA1 mutations. Up to 72% may present specific pulmonary manifestation, yet poorly reported. The objective of this work is to describe pulmonary manifestation in VEXAS syndrome and its impact in prognosis. 114 patients with VEXAS syndrome were included in the French cohort between November 2020 and June 2021. Most patients were males (95%), with a median age of 67.4 years at the onset of symptoms. Forty-five had a specific pulmonary involvement of VEXAS syndrome after adjudication. 44% complained of dyspnea 40% of cough, 6% required oxygen. Main clinical features did not differ between patients with pulmonary pulmonary involvement and those without: skin lesion (72% vs 87%), fever (67% vs 53%) or chondritis (38 vs 35%). Median CRP (105 vs 50 mg/L) and platelets count (222 vs 173 g/L) were increased in the patients with pulmonary manifestations. Nature the UBA1 mutation and prevalence of myelodysplasia (44% vs 52%) did not differ. All patients presented interstitial lung disease (ground glass opacities 87%, consolidation 49%, reticulation 38%, septal lines 51%) with 3 different pattern individualized (unspecific, OP like or CHF like) and 53.3% a pleural effusion. All patients were improved with prednisone but usually require>20 mg/day of prednisone, ruxolitinib (n=9) showing promising results. The median survival did not differ between the patients with pulmonary involvement (135.6 months) and those without (116 months). Pulmonary manifestations are frequent in VEXAS syndrome but rarely at the forefront. Outcome is favorable with prednisone and does not lead to pulmonary fibrosis.
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Key words
Orphan diseases,Bronchoalveolar lavage,Immunology
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