ORIGINAL RESEARCH article
Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders: Autoinflammatory Disorders
Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1605714
This article is part of the Research TopicClinical and Immunological Phenotypic Characterization to better understand Pathogenesis and Response to Therapies in Systemic Autoimmune DiseasesView all articles
Assessing disease phenotypes in Behçet's syndrome: insights from a multiple correspondence analysis
Provisionally accepted- 1Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Tuscany, Italy
- 2Department of Medical Biotechnology, University of Siena, Siena, Italy
- 3Institute of Management, Scuola Superiore Sant'Anna, Pisa, Italy, Pisa, Italy
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Behçet's syndrome (BS) is a rare systemic vasculitis. Clinical manifestations in BS are frequently clustered rather than discrete, and the concept that distinct clinical phenotypes may exist in BS has recently emerged. The aim of the present work was to identify and analyze the disease phenotypes in a mono-centric historical cohort of BS patients. A total of 202 patients with BS diagnosis followed-up at the Behçet Clinic of the Azienda Ospedaliero-Universitaria Pisana were identified, and demographics, clinical and therapeutical data were retrospectively collected. Pairwise correlation among variables was evaluated by means of Pearson or Spearman correlation coefficient. A multiple correspondence analysis was performed to investigate the possible phenotypes resulting from the different patterns of associations among the demographic and clinical variables.Most of the patients were females (67%), Caucasian (92%) and HLA-B51 carriers (65.5%). Mean age at disease onset was 30.06±11.39 years and oral ulcers (OU) and genital ulcers (GU) were the most common manifestations (96% and 61%, respectively). According to bivariate correlation analysis significant positive correlations were observed between skin lesions and both OU (p=0.005) and arthritis (p=0.014), as well as pathergy (p=0.001), gastrointestinal (GI) symptoms (p=0.001) and other involvement (fever and serositis) (p=0.015). Neurological involvement was significantly and positively associated with ocular lesions (p=0.0114), GI symptoms (p=0.030), pathergy (rho=0.147, p=0.037) and vein thromboses (p=0.037). Despite the high heterogeneity, four disease phenotypes emerged from the MCA: A) male Caucasians with greater age at onset and at diagnosis than the median values, with OU and GU, skin lesions, erythema nodosum (EN), arthritis and GI symptoms; B) co-existence of benign subset and pathergy; C) orchitis/epididymitis associated with neurological involvement and ocular lesions; D) GI symptoms plus endoscopic lesions, large vessel disease (both arterial and venous) and other involvement.This study provides valuable insights into the possible BS clinical phenotypes, and the results partially agree with previous association studies on European and extra-European cohorts.Observational comparative studies are warranted to assess the response of tailored phenotypesbased therapeutic approaches.
Keywords: Behcet Syndrome, Multicorrespondence analysis (MCA), Disease phenotypes, Historical cohort study, Clinical profiles
Received: 03 Apr 2025; Accepted: 26 May 2025.
Copyright: © 2025 Talarico, Di Cianni, Sulis, Marinello, Lorenzoni and Mosca. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Rosaria Talarico, Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, 56126, Tuscany, Italy
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