Details

Autor(en) / Beteiligte

• Vitale, Antonio
• Caggiano, Valeria
• Martin-Nares, Eduardo
• Frassi, Micol
• Dagna, Lorenzo
• Hissaria, Pravin
• Sfriso, Paolo
• Hernández-Rodríguez, José
• Ruiz-Irastorza, Guillermo
• Monti, Sara
• Tufan, Abdurrahman
• Piga, Matteo
• Giardini, Henrique A Mayrink
• Lopalco, Giuseppe
• Viapiana, Ombretta
• De Paulis, Amato
• Triggianese, Paola
• Vitetta, Rosetta
• de-la-Torre, Alejandra
• Fonollosa, Alex
• Caroni, Federico
• Sota, Jurgen
• Conticini, Edoardo
• Sbalchiero, Jessica
• Renieri, Alessandra
• Casamassima, Giulia
• Wiesik-Szewczyk, Ewa
• Yildirim, Derya
• Hinojosa-Azaola, Andrea
• Crisafulli, Francesca
• Franceschini, Franco
• Campochiaro, Corrado
• Tomelleri, Alessandro
• Callisto, Alicia
• Beecher, Mark
• Bindoli, Sara
• Baggio, Chiara
• Gómez-Caverzaschi, Verónica
• Pelegrín, Laura
• Soto-Peleteiro, Adriana
• Milanesi, Alessandra
• Vasi, Ibrahim
• Cauli, Alberto
• Antonelli, Isabele Parente de Brito
• Iannone, Florenzo
• Bixio, Riccardo
• Casa, Francesca Della
• Mormile, Ilaria
• Gurnari, Carmelo
• Fiorenza, Alessia
• Mejia-Salgado, Germán
• Kawakami-Campos, Perla Ayumi
• Ragab, Gaafar
• Ciccia, Francesco
• Ruscitti, Piero
• Bocchia, Monica
• Balistreri, Alberto
• Tosi, Gian Marco
• Frediani, Bruno
• Cantarini, Luca
• Fabiani, Claudia

Titel

Orbital/ocular inflammatory involvement in VEXAS syndrome: Data from the international AIDA network VEXAS registry

Ist Teil von

• Seminars in arthritis and rheumatism, 2024-06, Vol.66, p.152430-152430, Article 152430

Ort / Verlag

United States: Elsevier Inc

Erscheinungsjahr

2024

Quelle

Alma/SFX Local Collection

Beschreibungen/Notizen

• VEXAS syndrome is a recently described monogenic autoinflammatory disease capable of manifesting itself with a wide array of organs and tissues involvement. Orbital/ocular inflammatory manifestations are frequently described in VEXAS patients. The objective of this study is to further describe orbital/ocular conditions in VEXAS syndrome while investigating potential associations with other disease manifestations. In the present study, twenty-seven out of 59 (45.8 %) VEXAS patients showed an inflammatory orbital/ocular involvement during their clinical history. The most frequent orbital/ocular affections were represented by periorbital edema in 8 (13.6 %) cases, episcleritis in 5 (8.5 %) patients, scleritis in 5 (8.5 %) cases, uveitis in 4 (6.8 %) cases, conjunctivitis in 4 (6.8 %) cases, blepharitis in 3 (5.1 %) cases, orbital myositis in 2 (3.4 %) cases. A diagnosis of systemic immune-mediated disease was observed in 15 (55.6 %) cases, with relapsing polychondritis diagnosed in 12 patients. A significant association was observed between relapsing polychondritis and orbital/ocular involvement in VEXAS syndrome (Relative Risk: 2.37, 95 % C.I. 1.03–5.46, p = 0.048). Six deaths were observed in the whole cohort of patients after a median disease duration of 1.2 (IQR=5.35) years, 5 (83.3 %) of which showed orbital/ocular inflammatory involvement. In conclusion, this study confirms that orbital/ocular inflammatory involvement is a common finding in VEXAS patients, especially when relapsing polychondritis is diagnosed. This makes ophthalmologists a key figure in the diagnostic process of VEXAS syndrome. The high frequency of deaths observed in this study seems to suggest that patients with orbital/ocular involvement may require increased attention and more careful follow-up.