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Introduction <p>Behçet’s disease (BD) frequently arises with exclusively mucocutaneous involvement, but some patients will develop major organ involvement, including ocular inflammation. This study aims to assess the patients’ demographic and clinical characteristics that may be associated with the development of ocular involvement in patients with BD with exclusively mucocutaneous involvement in the early stages.</p> Methods <p>Patients’ data were collected in the International AutoInflammatory Disease Alliance (AIDA) Network registry dedicated to BD.</p> Results <p>A total of 328 patients with BD were enrolled, 36 (11%) of whom developed ocular involvement over time. The following variables were significantly associated with the development of ocular inflammation at binary logistic regression: positive family history for BD (OR 4.32, 95% CI 1.16–14.72, <i>p</i> = 0.02), Arab ethnicity (OR 3.6, 95% CI 1.3–9.9, <i>p</i> = 0.01), presence of major oral aphthous ulceration (OR 3.26, 95% CI 1.18–8.99, <i>p</i> = 0.02), pseudofolliculitis plus erythema nodosum (OR 4.58, 95% CI 1.33–15.7, <i>p</i> = 0.016); conversely, white patients/patients of European descent (OR 0.33, 95% CI 0.12–0.91, <i>p</i> = 0.03) and the presence of a low number (one to two) ulcers at genital aphthous flares (OR 0.002, 95% CI ~ 0–0.07, <i>p</i> = 0.0006) were protective against the occurrence of ocular inflammatory manifestations. Genital aphthosis with more than five concurrent ulcers was associated with eye involvement in Arab patients (OR 209.2, 95% CI 5.8–7497, p = 0.003).</p> Conclusions <p>Major oral aphthosis, genital aphthous attacks with more than five concurrent ulcers, erythema nodosum coexisting with pseudofolliculitis, a positive family history of BD, and Arab ethnicity are associated with a higher risk of ocular involvement when BD arises with the sole mucocutaneous involvement.</p>

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The Risk of Developing Ocular Involvement Among Behçet’s Disease Patients Presenting with Mucocutaneous Involvement at Disease Onset: Data from the International AIDA Network Behçet’s Disease Registry

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  • 发表日期：

    2025年11月8日

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  • Antonio Vitale,
  • Valeria Caggiano,
  • Jessica Sbalchiero,
  • Giuseppe Lopalco,
  • Gaafar Ragab,
  • Silvana Guerriero,
  • Ibrahim AlMaglouth,
  • Abdurrahman Tufan,
  • Roberto Giacomelli,
  • Haner Direskeneli,
  • Piero Ruscitti,
  • Gülen Hatemi,
  • Francesco Carubbi,
  • Ezgi Deniz Batu,
  • Seza Ozen,
  • Jurgen Sota,
  • Henrique Ayres Mayrink Giardini,
  • Micol Frassi,
  • Petros P. Sfikakis,
  • Federica Gatti,
  • Claudia Ammoscato,
  • Amina Maher,
  • Ayman Abdel-Monem Ahmed Mahmoud,
  • Rosanna Dammacco,
  • Hamit Kucuk,
  • Riza Can Kardas,
  • Ibrahim Yahya Cakir,
  • Fatma Alibaz Öner,
  • Gizem Sevik,
  • Martina Gentile,
  • Alican Karakoc,
  • Alessia Alunno,
  • Hulya Ercan Emreol,
  • Francesca Crisafulli,
  • Katerina Laskari,
  • Francesco Ciccia,
  • Maissa Thabet,
  • Naceur Feriel,
  • Serena Bugatti,
  • Alessandra Milanesi,
  • Maria Sole Chimenti,
  • Benedetta Monosi,
  • Matteo Piga,
  • Alberto Floris,
  • Francesco Gavioli,
  • Cecilia Beatrice Chighizola,
  • José Hernández-Rodríguez,
  • Marco Cattalini,
  • Marcello Govoni,
  • Ombretta Viapiana,
  • Adele Civino,
  • Daniela Opris-Belinski,
  • Carla Gaggiano,
  • Rosaria Talarico,
  • Annachiara Alemanno,
  • Annarita Giardina,
  • Giacomo Emmi,
  • Piercarlo Sarzi Puttini,
  • Maria Cristina Maggio,
  • Paola Parronchi,
  • Piero Portincasa,
  • Alejandra de-la-Torre,
  • Laura Daniela Rodríguez-Camelo,
  • Stefano Gentileschi,
  • Angela Mauro,
  • Gian Domenico Sebastiani,
  • Alma Nunzia Olivieri,
  • Ali Şahin,
  • Donato Rigante,
  • Emre Bilgin,
  • Emanuela Del Giudice,
  • Luciana Breda,
  • Amato De Paulis,
  • Alberto Lo Gullo,
  • Şükran Erten,
  • Samar Tharwat,
  • Lampros Fotis,
  • Armin Maier,
  • Antonella Insalaco,
  • Anastasios Karamanakos,
  • Alessandro Conforti,
  • Özgül Soysal Gündüz,
  • Abdelhfeez Moshrif,
  • Francesca Li Gobbi,
  • Stefania Costi,
  • Elena Bartoloni,
  • Patrizia Barone,
  • Serena Guiducci,
  • Andrés Gonzáles-García,
  • Inés Hernanz Rodriguez,
  • Giovanni Conti,
  • Annamaria Iagnocco,
  • Fatos Önen,
  • Sulaiman M. Al-Mayouf,
  • Didar Uçar,
  • Alberto Balistreri,
  • Bruno Frediani,
  • Luca Cantarini,
  • Claudia Fabiani

摘要

Introduction

Behçet’s disease (BD) frequently arises with exclusively mucocutaneous involvement, but some patients will develop major organ involvement, including ocular inflammation. This study aims to assess the patients’ demographic and clinical characteristics that may be associated with the development of ocular involvement in patients with BD with exclusively mucocutaneous involvement in the early stages.

Methods

Patients’ data were collected in the International AutoInflammatory Disease Alliance (AIDA) Network registry dedicated to BD.

Results

A total of 328 patients with BD were enrolled, 36 (11%) of whom developed ocular involvement over time. The following variables were significantly associated with the development of ocular inflammation at binary logistic regression: positive family history for BD (OR 4.32, 95% CI 1.16–14.72, p = 0.02), Arab ethnicity (OR 3.6, 95% CI 1.3–9.9, p = 0.01), presence of major oral aphthous ulceration (OR 3.26, 95% CI 1.18–8.99, p = 0.02), pseudofolliculitis plus erythema nodosum (OR 4.58, 95% CI 1.33–15.7, p = 0.016); conversely, white patients/patients of European descent (OR 0.33, 95% CI 0.12–0.91, p = 0.03) and the presence of a low number (one to two) ulcers at genital aphthous flares (OR 0.002, 95% CI ~ 0–0.07, p = 0.0006) were protective against the occurrence of ocular inflammatory manifestations. Genital aphthosis with more than five concurrent ulcers was associated with eye involvement in Arab patients (OR 209.2, 95% CI 5.8–7497, p = 0.003).

Conclusions

Major oral aphthosis, genital aphthous attacks with more than five concurrent ulcers, erythema nodosum coexisting with pseudofolliculitis, a positive family history of BD, and Arab ethnicity are associated with a higher risk of ocular involvement when BD arises with the sole mucocutaneous involvement.