Frontiers in immunology · 2026

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

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

Аннотация

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.

Предметные рубрики MeSH

Humans Registries Scleritis Female Male Uveitis, Anterior Polychondritis, Relapsing Prognosis Syndrome Myelodysplastic Syndromes Skin Diseases, Genetic

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

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Аннотация

Предметные рубрики MeSH