TY - JOUR
T1 - Ophthalmic involvement in VEXAS syndrome and its influence on mortality
T2 - insights from the international AIDA network registry
AU - Sota, Jurgen
AU - Hinojosa-Azaola, Andrea
AU - Martín-Nares, Eduardo
AU - Sfriso, Paolo
AU - Bindoli, Sara
AU - Hissaria, Pravin
AU - Beecher, Mark
AU - Hernández-Rodríguez, José
AU - Gómez-Caverzaschi, Verónica
AU - Frassi, Micol
AU - Crisafulli, Francesca
AU - Dagna, Lorenzo
AU - Campochiaro, Corrado
AU - Bugatti, Serena
AU - Milanesi, Alessandra
AU - Ruiz-Irastorza, Guillermo
AU - Soto-Peleteiro, Adriana
AU - Piga, Matteo
AU - Viapiana, Ombretta
AU - Tufan, Abdurrahman
AU - Bolek, Ertugrul Cagri
AU - Triggianese, Paola
AU - Prete, Marcella
AU - Sblachiero, Jessica
AU - Caggiano, Valeria
AU - Vitale, Antonio
AU - Giardini, Henrique Ayres Mayrink
AU - Lopalco, Giuseppe
AU - Conti, Fabrizio
AU - Moscato, Paolo
AU - Cardamone, Chiara
AU - Wiesik-Szewczy, Ewa
AU - González-García, Andrés
AU - De Paulis, Amato
AU - Vitetta, Rosetta
AU - Kawakami-Campos, Perla Ayumi
AU - Brancaleoni, Alessandra
AU - Mercanti, Andrea
AU - Renieri, Alessandra
AU - Bocchia, Monica
AU - Ragab, Gaafar
AU - Gupta, Vishali
AU - de-la-Torre, Alejandra
AU - Frediani, Bruno
AU - Gurnari, Carmelo
AU - Cantarini, Luca
AU - Fabiani, Claudia
N1 - Publisher Copyright:
Copyright © 2026 Sota, Hinojosa-Azaola, Martín-Nares, Sfriso, Bindoli, Hissaria, Beecher, Hernández-Rodríguez, Gómez-Caverzaschi, Frassi, Crisafulli, Dagna, Campochiaro, Bugatti, Milanesi, Ruiz-Irastorza, Soto-Peleteiro, Piga, Viapiana, Tufan, Bolek, Triggianese, Prete, Sblachiero, Caggiano, Vitale, Giardini, Lopalco, Conti, Moscato, Cardamone, Wiesik-Szewczy, González-García, De Paulis, Vitetta
PY - 2026
Y1 - 2026
N2 - Orbital inflammation is the most common presentation of VEXAS, with any orbital structure potentially affected. Non-sight-threatening and uncomplicated anterior non-granulomatous uveitis and anterior diffuse scleritis follow in frequency, along with episcleritis. Ophthalmic involvement was significantly associated with relapsing polychondritis (p = 0.014), with an increased chance of a fatal outcome (RR 5.87, p = 0.016) and independently predicts a higher mortality rate (OR 3.72, p = 0.026). Treatment of ophthalmic involvement showed full or partial response to glucocorticosteroids alone in 66.7% of cases. Ophthalmic involvement is common in VEXAS syndrome and signals a poorer prognosis in terms of mortality, highlighting the need for close monitoring.
AB - Orbital inflammation is the most common presentation of VEXAS, with any orbital structure potentially affected. Non-sight-threatening and uncomplicated anterior non-granulomatous uveitis and anterior diffuse scleritis follow in frequency, along with episcleritis. Ophthalmic involvement was significantly associated with relapsing polychondritis (p = 0.014), with an increased chance of a fatal outcome (RR 5.87, p = 0.016) and independently predicts a higher mortality rate (OR 3.72, p = 0.026). Treatment of ophthalmic involvement showed full or partial response to glucocorticosteroids alone in 66.7% of cases. Ophthalmic involvement is common in VEXAS syndrome and signals a poorer prognosis in terms of mortality, highlighting the need for close monitoring.
UR - https://www.scopus.com/pages/publications/105044506572
UR - https://www.scopus.com/pages/publications/105044506572#tab=citedBy
U2 - 10.3389/fimmu.2026.1709085
DO - 10.3389/fimmu.2026.1709085
M3 - Research Article
C2 - 42404884
AN - SCOPUS:105044506572
SN - 1664-3224
VL - 17
SP - 1709085
JO - Frontiers in Immunology
JF - Frontiers in Immunology
ER -