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. 2026 Feb 4;65(2):keaf613.
doi: 10.1093/rheumatology/keaf613.

Risk of major organ involvement in Behçet's patients with mucocutaneous onset: data from the AIDA Network Registry

Antonio Vitale  1 , Francesco Gavioli  1 , Valeria Caggiano  1 , Jessica Sbalchiero  1 , Giuseppe Lopalco  2 , Gaafar Ragab  3   4 , Silvana Guerriero  5 , Ibrahim AlMaglouth  6 , Abdurrahman Tufan  7 , Roberto Giacomelli  8 , Haner Direskeneli  9 , Piero Ruscitti  10 , Gülen Hatemi  11 , Francesco Carubbi  12 , Ezgi Deniz Batu  13 , Seza Ozen  13 , Jurgen Sota  1 , Henrique Ayres Mayrink Giardini  14 , Micol Frassi  15 , Petros P Sfikakis  16 , Florenzo Iannone  2 , Maria Morrone  2 , Mahmoud Ghanema  3 , Moustafa Ali Saad  3 , Rosanna Dammacco  5 , Hamit Kucuk  7 , Riza Can Kardas  7 , Ibrahim Yahya Cakir  7 , Luca Navarini  8 , Fatma Alibaz Öner  9 , Gizem Sevik  9 , Martina Gentile  10 , Alican Karakoc  11 , Alessia Alunno  12 , Hulya Ercan Emreol  13 , Francesca Crisafulli  15 , George Fragoulis  16 , Francesco Ciccia  17 , Maissa Thabet  18 , Serena Bugatti  19 , Alessandra Milanesi  19 , Maria Sole Chimenti  20 , Benedetta Monosi  20 , Matteo Piga  21 , Alberto Floris  21 , Andrea Hinojosa-Azaola  22 , Guillermo Arturo Guaracha-Basañez  22 , Cecilia Beatrice Chighizola  23   24 , José Hernández-Rodríguez  25 , Marco Cattalini  26 , Marcello Govoni  27 , Ombretta Viapiana  28 , Adele Civino  29 , Daniela Opris-Belinski  30 , Carla Gaggiano  1 , Rosaria Talarico  31 , Annarita Giardina  32 , Giacomo Emmi  33   34 , Piercarlo Sarzi Puttini  35 , Maria Cristina Maggio  36 , Paola Parronchi  37 , Piero Portincasa  38 , Alejandra de-la-Torre  39 , Blanca Aguilar-Barrera  39 , Stefano Gentileschi  1 , Angela Mauro  40 , Gian Domenico Sebastiani  41 , Alma Nunzia Olivieri  42 , Ali Şahin  43 , Donato Rigante  44   45 , Emre Bilgin  46 , Emanuela Del Giudice  47 , Luciana Breda  48 , Amato De Paulis  49   50 , Alberto Lo Gullo  51 , Şükran Erten  52 , Samar Tharwat  53   54 , Lampros Fotis  55 , Armin Maier  56 , Antonella Insalaco  57 , Anastasios Karamanakos  58 , Alessandro Conforti  59 , Özgül Soysal Gündüz  60 , Abdelhfeez Moshrif  61 , Francesca Li Gobbi  62 , Alberto Balistreri  63 , Elena Bartoloni  64 , Patrizia Barone  65 , Serena Guiducci  66 , Andrés Gonzáles-García  67 , Inés Hernanz Rodriguez  68 , Giovanni Conti  69 , Annamaria Iagnocco  70 , Fatos Önen  71 , Sulaiman M Al-Mayouf  72 , Didar Ucar  73 , Bruno Frediani  1 , Claudia Fabiani  74 , Luca Cantarini  1 ; from the International AIDA (AutoInflammatory Diseases Alliance) Network
Affiliations

Risk of major organ involvement in Behçet's patients with mucocutaneous onset: data from the AIDA Network Registry

Antonio Vitale et al. Rheumatology (Oxford). .

Abstract

Objectives: The progression of Behçet's disease (BD) from a mucocutaneous-limited form to major organ involvement (MOI) represents a significant challenge. This study aims to identify patients without MOI at BD onset who are at increased risk of developing MOI in later stages.

Methods: Patients' data were drawn from the International AutoInflammatory Disease Alliance (AIDA) Network registry dedicated to BD.

Results: A total of 328 patients with exclusively mucocutaneous manifestations at BD onset were enrolled. Of these, 82 patients (25%) developed MOI over the entire follow-up period. Patients with minor oral aphthosis and no major oral aphthosis exhibited a reduced risk of developing MOI, with an odds ratio (OR) of 0.41 [95% confidence interval (95%CI): 0.22-0.79, P = 0.008]. Conversely, patients with both major and minor oral aphthosis had a significantly higher risk of developing MOI, with an OR of 12.76 (95%CI: 1.44-113, P = 0.02). Moreover, the development of MOI was associated with major oral aphthosis plus genital aphthosis (OR: 2.49, 95%CI: 1.1-5.6, P = 0.03), major oral aphthosis plus pseudofolliculitis (OR: 2.9, 95%CI: 1.15-7.4, P = 0.02) and major oral aphthosis plus both genital aphthosis and pseudofolliculitis (OR: 3.73, 95%CI: 1.22-11.4, P = 0.02). A positive family history for BD was associated with MOI (OR: 2.85, 95%CI: 1.08-7.58, P = 0.03).

Conclusion: A positive family history and the presence of major oral aphthosis combined with minor oral aphthosis, genital aphthosis or pseudofolliculitis are associated with MOI development in patients with mucocutaneous BD at onset.

Keywords: AIDA Network; Behçet’s disease; international registry; prognosis; rare disease; uveitis.

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