Ophthalmic involvement in VEXAS syndrome and its influence on mortality: insights from the international AIDA network registry - Scorecard - 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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Clinical Scorecard: Ocular Manifestations in VEXAS Syndrome and Their Impact on Mortality: Findings from the Global AIDA Network Registry

At a Glance

CategoryDetail
ConditionVEXAS Syndrome
Key MechanismsAcquired mutations on the UBA1 gene leading to systemic inflammation and hematologic disorders.
Target PopulationPatients with genetically confirmed VEXAS syndrome.
Care SettingMulticenter cohort study assessing ophthalmic involvement.

Key Highlights

  • Orbital inflammation is the most common ocular manifestation in VEXAS syndrome.
  • Ophthalmic involvement significantly predicts higher mortality rates (OR 3.72).
  • 66.7% of patients showed full or partial response to glucocorticosteroids.
  • Ophthalmic inflammation is associated with relapsing polychondritis (p = 0.014).
  • Fatal outcomes were more frequent in patients with ocular involvement (RR 5.87).

Guideline-Based Recommendations

Diagnosis

  • Ophthalmic involvement should be assessed in patients with VEXAS syndrome.

Management

  • Glucocorticosteroids are effective in treating ophthalmic inflammation.

Monitoring & Follow-up

  • Close monitoring of patients with ocular involvement is essential due to increased mortality risk.

Risks

  • Ophthalmic involvement is associated with a higher risk of fatal outcomes.

Patient & Prescribing Data

86 patients with VEXAS syndrome (83 males, 3 females).

Topical and/or systemic glucocorticosteroids showed a response in 76.4% of patients with uveitis and scleritis.

Clinical Best Practices

  • Involve ophthalmologists in the multidisciplinary management of VEXAS syndrome.
  • Consider the presence of ocular symptoms as a prognostic factor.

Related Resources & Content

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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