Ophthalmic involvement in VEXAS syndrome and its influence on mortality: insights from the international AIDA network registry - Summary - MDSpire

Ophthalmic involvement in VEXAS syndrome and its influence on mortality: insights from the international AIDA network registry

  • By

  • Jurgen Sota

  • Andrea Hinojosa-Azaola

  • Eduardo Martín-Nares

  • Paolo Sfriso

  • Sara Bindoli

  • Pravin Hissaria

  • Mark Beecher

  • José Hernández-Rodríguez

  • Verónica Gómez-Caverzaschi

  • Micol Frassi

  • Francesca Crisafulli

  • Lorenzo Dagna

  • Corrado Campochiaro

  • Serena Bugatti

  • Alessandra Milanesi

  • Guillermo Ruiz-Irastorza

  • Adriana Soto-Peleteiro

  • Matteo Piga

  • Ombretta Viapiana

  • Abdurrahman Tufan

  • Ertugrul Cagri Bolek

  • Paola Triggianese

  • Marcella Prete

  • Jessica Sblachiero

  • Valeria Caggiano

  • Antonio Vitale

  • Henrique Ayres Mayrink Giardini

  • Giuseppe Lopalco

  • Fabrizio Conti

  • Paolo Moscato

  • Chiara Cardamone

  • Ewa Wiesik-Szewczy

  • Andrés González-García

  • Amato De Paulis

  • Rosetta Vitetta

  • Perla Ayumi Kawakami-Campos

  • Alessandra Brancaleoni

  • Andrea Mercanti

  • Alessandra Renieri

  • Monica Bocchia

  • Gaafar Ragab

  • Vishali Gupta

  • Alejandra de-la-Torre

  • Bruno Frediani

  • Carmelo Gurnari

  • Luca Cantarini

  • Claudia Fabiani

  • June 19, 2026

  • 0 min

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Objective:

To detail the ophthalmic involvement in patients with VEXAS syndrome and assess its association with mortality.

Approach:
    Key Findings:
    • Ophthalmic inflammation occurred in 47.7% of patients, often present at disease onset.
    • Anterior uveitis and anterior diffuse scleritis were the most common forms of ocular involvement.
    • Ophthalmic involvement was significantly associated with relapsing polychondritis (p = 0.014).
    • Patients with ocular involvement had a higher mortality rate (RR 5.87, p = 0.016; OR 3.72, p = 0.026).
    • 66.7% of patients with ophthalmic involvement showed full or partial response to glucocorticosteroids.
    Interpretation:

    Ophthalmic involvement in VEXAS syndrome is common.

    Limitations:
    • The study is based on a relatively small cohort of 86 patients.
    • Data on the onset age of ophthalmic involvement was missing for some patients.
    Conclusion:

    Ophthalmic inflammation is a significant aspect of VEXAS syndrome.

Original Source(s)

Ophthalmic involvement in VEXAS syndrome and its influence on mortality: insights from the international AIDA network registry

  • by Jurgen Sota, Andrea Hinojosa-Azaola, Eduardo Martín-Nares, Paolo Sfriso, Sara Bindoli, Pravin Hissaria, Mark Beecher, José Hernández-Rodríguez, Verónica Gómez-Caverzaschi, Micol Frassi, Francesca Crisafulli, Lorenzo Dagna, Corrado Campochiaro, Serena Bugatti, Alessandra Milanesi, Guillermo Ruiz-Irastorza, Adriana Soto-Peleteiro, Matteo Piga, Ombretta Viapiana, Abdurrahman Tufan, Ertugrul Cagri Bolek, Paola Triggianese, Marcella Prete, Jessica Sblachiero, Valeria Caggiano, Antonio Vitale, Henrique Ayres Mayrink Giardini, Giuseppe Lopalco, Fabrizio Conti, Paolo Moscato, Chiara Cardamone, Ewa Wiesik-Szewczy, Andrés González-García, Amato De Paulis, Rosetta Vitetta, Perla Ayumi Kawakami-Campos, Alessandra Brancaleoni, Andrea Mercanti, Alessandra Renieri, Monica Bocchia, Gaafar Ragab, Vishali Gupta, Alejandra de-la-Torre, Bruno Frediani, Carmelo Gurnari, Luca Cantarini, Claudia Fabiani

  • June 19, 2026

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