Behçet’s syndrome: distinct features in 344 pediatric and adult patients in a non-endemic region

Ricardo Machado¹, Thales Souza², Matheus França³, Vinicius Matias⁴, Sylvia Farhat¹, Carolina Siqueira⁴, Maria Carolina Santos⁴, Lia Steuer¹, Luisa Forero¹, Rafael Bassara Macedo⁵, Thiago Freitas⁶, barbara Bayeh⁷, Carolina Ejnisman⁸, Pedro Araujo⁹, Rafael Cordeiro¹⁰, Fabio Specian⁵, Verena Balbi¹, Nadia Emi Aikawa¹¹, Katia Kozu¹, Clovis Artur Silva¹², Lucia Maria Campos¹³, Adriana Elias¹⁴ and Henrique Giardini⁶, ¹Instituto da Criança e do Adolescente, Hospital das Clinicas, Sao Paulo, Sao Paulo, Brazil, ²Faculdade de Medicina da Universidade de Brasilia, Brasilia, Distrito Federal, Brazil, ³Instituto da Criança e do Adolescente, Hospital das Clinicas, University of Sao Paulo Medical School, Sao Paulo, Sao Paulo, Brazil, ⁴Faculdade de Ciências Medicas da Santa Casa de Sao Paulo, Sao Paulo, Sao Paulo, Brazil, ⁵Rheumatology Division, Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo (USP), Sao Paulo, Sao Paulo, Brazil, ⁶Rheumatology Division, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil, São Paulo, Brazil, ⁷Hospital das Clínicas, University of Sao Paulo Medical School, Sao Paulo, Sao Paulo, Brazil, ⁸Rheumatology Division, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil, Sao Paulo, Brazil, ⁹Universidade de São Paulo, Sao Paulo, São Paulo, Brazil, ¹⁰Rheumatology Division, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil, São Paulo, Sao Paulo, Brazil, ¹¹Rheumatology Division and Pediatric Rheumatology Unit, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil, Sao Paulo, São Paulo, Brazil, ¹²University of São Paulo, São Paulo, São Paulo, Brazil, ¹³Instituto da Criança e do Adolescente, São Paulo, São Paulo, Brazil, ¹⁴Pediatric Rheumatology Unit, Instituto da Criança e do Adolescente, Hospital das Clinicas, University of Sao Paulo Medical School, São Paulo, São Paulo, Brazil

Meeting: ACR Convergence 2025

Keywords: autoimmune diseases, Behçet's Syndrome, Diagnostic criteria, Pediatric rheumatology

Session Information

Date: Tuesday, October 28, 2025

Title: (2524–2546) Vasculitis – Non-ANCA-Associated & Related Disorders Poster III

Session Type: Poster Session C

Session Time: 10:30AM-12:30PM

Background/Purpose: Behçet’s syndrome (BS) is a chronic inflammatory disorder characterized by a heterogeneous clinical presentation, occurring primarily in young adults and very rarely in children and adolescents. In addition, there are few studies that compared childhood-onset BS (cBS) and adult-onset BS (aBS), especially in non-endemic regions. Therefore, the objectives of the present study were to evaluate demographic, clinical and therapeutic data in cBS and aBS.

Methods: Medical records of 344 patients [99 cBS (≤ 18 years-old) and 245 aBS] from three tertiary Brazilian Rheumatology centers were reviewed in order to compare clinical and therapeutic features. cBS and aBS were classified according to International Study Group and/or the International Criteria for Behçet’s Disease.

Results: The median age at diagnosis was 11(0.5-17) years in cBS and 30(18-76) years in aBS. The median follow-up time was significantly reduced in cBS compared to aBD [7(0-23) vs. 10(0-27) years, p=0.014], with similar female sex in both groups (56% vs. 62%, p=0.14). Classification criteria were fulfilled in 82% of cBS and 100% of aBS patients. Fever (30% vs. 15%, p=0.002), gastrointestinal involvement (55% vs. 14%, p=0.003), and a family history of autoimmune diseases (22% vs. 16%, p=0.001) were significantly higher in cBS compared to aBS. In contrast, genital ulcers (67% vs. 81%, p=0.01), cutaneous lesions (47% vs. 68%, p=0.001), including pseudofolliculitis (34% vs. 53%, p=0.001) and erythema nodosum (24% vs. 36%, p=0.03), uveitis (32% vs. 44%, p=0.03), corticosteroids (68% vs. 88%, p < 0.001), azathioprine (56% vs. 70%, p=0.01), cyclosporine (9% vs. 22%, p=0.004), cyclophosphamide (19% vs. 34%, p=0.001), and intravenous immunoglobulin use (9% vs. 18%, p=0.03) were significantly lower in the former group. Frequencies of oral and cutaneous ulcers, articular or central nervous system involvement, retinal vasculitis, colchicine, mycophenolate mofetil, and methotrexate use were similar in both groups (p >0.05).

Conclusion: cBS was rarely observed in our non-endemic region, potentially due to under recognition, as it often presented with nonspecific symptoms such as fever and gastrointestinal involvement. The broader spectrum of symptoms in aBS patients might account for the more extensive use of immunosuppressive agents.

Disclosures: R. Machado: None; T. Souza: None; M. França: None; V. Matias: None; S. Farhat: None; C. Siqueira: None; M. Santos: None; L. Steuer: None; L. Forero: None; R. Bassara Macedo: None; T. Freitas: None; b. Bayeh: None; C. Ejnisman: None; P. Araujo: None; R. Cordeiro: None; F. Specian: None; V. Balbi: None; N. Aikawa: Fundação de Amparo a Pesquisa do Estado de São Paulo (FAPESP) (2022/12925-8), 5, GlaxoSmithKlein(GSK), 5; K. Kozu: None; C. Silva: Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) (304984/2020-5), 5, Fundação de Amparo a Pesquisa do Estado de Sao Paulo (FAPESP) (2022/12925-8), 5, GlaxoSmithKlein(GSK), 5; L. Campos: None; A. Elias: None; H. Giardini: None.

To cite this abstract in AMA style:

Machado R, Souza T, França M, Matias V, Farhat S, Siqueira C, Santos M, Steuer L, Forero L, Bassara Macedo R, Freitas T, Bayeh b, Ejnisman C, Araujo P, Cordeiro R, Specian F, Balbi V, Aikawa N, Kozu K, Silva C, Campos L, Elias A, Giardini H. Behçet’s syndrome: distinct features in 344 pediatric and adult patients in a non-endemic region [abstract]. Arthritis Rheumatol. 2025; 77 (suppl 9). https://acrabstracts.org/abstract/behcets-syndrome-distinct-features-in-344-pediatric-and-adult-patients-in-a-non-endemic-region/. Accessed .

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