Ocular Manifestations in Juvenile Behçet's Disease: A Registry-Based Analysis from the AIDA Network.

Autoinflammatory diseases Behçet’s disease Paediatric ophthalmology Rare disease registries Retinal vasculitis Uveitis

Journal

Ophthalmology and therapy
ISSN: 2193-8245
Titre abrégé: Ophthalmol Ther
Pays: England
ID NLM: 101634502

Informations de publication

Date de publication:
30 Mar 2024
Historique:
received: 08 01 2024
accepted: 20 02 2024
medline: 2 4 2024
pubmed: 2 4 2024
entrez: 2 4 2024
Statut: aheadofprint

Résumé

This study aims to characterize ocular manifestations of juvenile Behçet's disease (jBD). This was a registry-based observational prospective study. All subjects with jBD from the Autoinflammatory Diseases Alliance (AIDA) Network BD Registry showing ocular manifestations before 18 years were enrolled. We included 27 of 1000 subjects enrolled in the registry (66.7% male patients, 45 affected eyes). The median (interquartile range [IQR]) age at ocular involvement was 14.2 (4.7) years. Uveitis affected 91.1% of eyes (anterior 11.1%, posterior 40.0%, panuveitis 40.0%), retinal vasculitis 37.8% and other manifestations 19.8%. Later onset (p = 0.01) and male predominance (p = 0.04) characterized posterior involvement. Ocular complications occurred in 51.1% of eyes. Patients with complications had earlier onset (p < 0.01), more relapses (p = 0.02) and more prolonged steroidal treatment (p = 0.02). The mean (standard deviation [SD]) central macular thickness (CMT) at the enrolment and last visit was 302.2 (58.4) and 293.3 (78.2) μm, respectively. Fluorescein angiography was pathological in 63.2% of procedures, with a mean (SD) Angiography Scoring for Uveitis Working Group (ASUWOG) of 17.9 (15.5). At the last visit, ocular damage according to the BD Overall Damage Index (BODI) was documented in 73.3% of eyes. The final mean (SD) best corrected visual acuity (BCVA) logMAR was 0.17 (0.47) and blindness (BCVA logMAR < 1.00 or central visual field ≤ 10°) occurred in 15.6% of eyes. At multivariate regression analysis, human leukocyte antigen (HLA)-B51 + independently predicted a + 0.35 change in the final BCVA logMAR (p = 0.01), while a higher BCVA logMAR at the first assessment (odds ratio [OR] 5.80; p = 0.02) independently predicted blindness. The results of this study may be leveraged to guide clinical practice and future research on this rare sight-threatening condition.

Identifiants

DOI: 10.1007/s40123-024-00916-z PMID: 38563868
pubmed: 38563868
doi: 10.1007/s40123-024-00916-z
pii: 10.1007/s40123-024-00916-z
doi:

Types de publication

Journal Article

Langues

eng

Informations de copyright

© 2024. The Author(s).

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Auteurs

Carla Gaggiano (C)

Rheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.

Abdurrahman Tufan (A)

Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Gazi University, Ankara, Turkey.

Silvana Guerriero (S)

Department of Ophthalmology and Otolaryngology, University of Bari, Bari, Italy.

Gaafar Ragab (G)

Rheumatology and Clinical Immunology Unit, Department of Internal Medicine, Faculty of Medicine, Cairo University, Giza, Egypt.
Faculty of Medicine, New Giza University, Giza, Egypt.

Jurgen Sota (J)

Rheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.

Stefano Gentileschi (S)

Rheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.

Stefania Costi (S)

Unit of Pediatric Rheumatology, Azienda Socio-Sanitaria Territoriale (ASST) Gaetano Pini-Centro Specialistico Ortopedico Traumatologico (CTO), Milan, Italy.

Ibrahim A Almaghlouth (IA)

Rheumatology Unit, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
College of Medicine Research Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia.

Andrea Hinojosa-Azaola (A)

Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.

Samar Tharwat (S)

Rheumatology and Immunology Unit, Internal Medicine Department, Mansoura University, Mansoura, Egypt.

Petros P Sfikakis (PP)

First Department of Propaedeutic Internal Medicine, Medical School, Laiko General Hospital [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory, and Autoimmune Diseases (RITA) Center], National and Kapodistrian University of Athens, Athens, Greece.
Joint Academic Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Athens, Greece.

Giuseppe Lopalco (G)

Rheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari, Bari, Italy.

Matteo Piga (M)

Rheumatology Unit, Department of Medical Sciences, University and AOU of Cagliari, Cagliari, Italy.

Giovanni Conti (G)

Pediatric Nephrology and Rheumatology Unit, Azienda Ospedaliero Universitaria (AOU) G Martino, Messina, Italy.

George Fragoulis (G)

First Department of Propaedeutic Internal Medicine, Medical School, Laiko General Hospital [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory, and Autoimmune Diseases (RITA) Center], National and Kapodistrian University of Athens, Athens, Greece.
Joint Academic Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Athens, Greece.

Angela Mauro (A)

Department of Biomedical and Clinical Sciences, Fatebenefratelli Hospital, Università di Milano, Milan, Italy.
Pediatric Rheumatology Unit, Department of Childhood and Developmental Medicine, Fatebenefratelli-Sacco Hospital, Milan, Italy.

Ezgi D Batu (ED)

Department of Pediatric Rheumatology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Seza Ozen (S)

Department of Pediatric Rheumatology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Maria Tarsia (M)

Clinical Pediatrics, Department of Molecular Medicine and Development, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy.

Francesco La Torre (F)

Department of Pediatrics, Giovanni XXIII Pediatric Hospital, University of Bari, Bari, Italy.

Perla A Kawakami-Campos (PA)

Department of Ophthalmology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.

Antonio Vitale (A)

Rheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.

Valeria Caggiano (V)

Rheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.

Riza C Kardaş (RC)

Division of Rheumatology, Department of Internal Medicine, Gazi University Hospital, Ankara, Turkey.

Gian Marco Tosi (GM)

Ophthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy.

Bruno Frediani (B)

Rheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.

Tadej Avčin (T)

Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University of Ljubljana and University Medical Centre Ljubljana [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Ljubljana, Slovenia.

José Hernández-Rodríguez (J)

Department of Autoimmune Diseases, Hospital Clínic of Barcelona [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.

Luca Cantarini (L)

Rheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy. cantariniluca@hotmail.com.
ORCID: 0000-0002-7352-1275

Claudia Fabiani (C)

Ophthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy.

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