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

被引：2

作者：

Gaggiano, Carla ^{[1
,2
]}

Tufan, Abdurrahman ^{[3
]}

Guerriero, Silvana ^{[4
]}

Ragab, Gaafar ^{[5
,6
]}

Sota, Jurgen ^{[1
,2
]}

Gentileschi, Stefano ^{[1
,2
]}

Costi, Stefania ^{[7
]}

Almaghlouth, Ibrahim A. ^{[8
,9
]}

Hinojosa-Azaola, Andrea ^{[10
]}

Tharwat, Samar ^{[11
]}

Sfikakis, Petros P. ^{[12
,13
]}

Lopalco, Giuseppe ^{[14
]}

Piga, Matteo ^{[15
]}

Conti, Giovanni ^{[16
]}

Fragoulis, George ^{[12
,13
]}

Mauro, Angela ^{[17
,18
]}

Batu, Ezgi D. ^{[19
]}

Ozen, Seza ^{[19
]}

Tarsia, Maria ^{[20
,21
]}

La Torre, Francesco ^{[22
]}

Kawakami-Campos, Perla A. ^{[23
]}

Vitale, Antonio ^{[1
,2
]}

Caggiano, Valeria ^{[1
,2
]}

Kardas, Riza C. ^{[24
]}

Tosi, Gian Marco ^{[21
,25
]}

Frediani, Bruno ^{[1
,2
]}

Avcin, Tadej ^{[26
,27
]}

Hernandez-Rodriguez, Jose ^{[28
]}

Cantarini, Luca ^{[1
,2
]}

Fabiani, Claudia ^{[21
,25
]}

机构：

[1] Univ Siena, Dept Med Sci Surg & Neurosci, Rheumatol Unit, Viale Bracci 16, I-53100 Siena, Italy

[2] Azienda Ospedaliero Univ Senese, Policlin Le Scotte, European Reference Network ERN Rare Immunodeficien, Viale Bracci 16, I-53100 Siena, Italy

[3] Gazi Univ, Fac Med, Dept Internal Med, Div Rheumatol, Ankara, Turkiye

[4] Univ Bari, Dept Ophthalmol & Otolaryngol, Bari, Italy

[5] Cairo Univ, Fac Med, Dept Internal Med, Rheumatol & Clin Immunol Unit, Giza, Egypt

[6] New Giza Univ, Fac Med, Giza, Egypt

[7] Azienda Sociosanit Terr ASST Gaetano Pini, Ctr Specialist Ortoped Traumatol CTO, Unit Pediat Rheumatol, Milan, Italy

[8] King Saud Univ, Coll Med, Dept Med, Rheumatol Unit, Riyadh, Saudi Arabia

[9] King Saud Univ, Res Ctr, Coll Med, Riyadh, Saudi Arabia

[10] Inst Nacl Ciencias Med & Nutr Salvador Zubiran, Dept Immunol & Rheumatol, Mexico City, Mexico

[11] Mansoura Univ, Internal Med Dept, Rheumatol & Immunol Unit, Mansoura, Egypt

[12] Natl & Kapodistrian Univ Athens, Laiko Gen Hosp European Reference Network ERN Rare, Dept Propaedeut Internal Med 1, Athens, Greece

[13] Natl & Kapodistrian Univ Athens, Med Sch, Joint Acad Rheumatol Program, Athens, Greece

[14] Univ Bari, Dept Emergency & Organ Transplantat, Rheumatol Unit, Bari, Italy

[15] Univ & AOU Cagliari, Dept Med Sci, Rheumatol Unit, Cagliari, Italy

[16] Azienda Ospedaliero Universitaria AOU G Martino, Azienda Ospedaliero Universitaria AOU Martino G, Messina, Italy

[17] Univ Milan, Fatebenefratelli Hosp, Dept Biomed & Clin Sci, Milan, Italy

[18] Fatebenefratelli Sacco Hosp, Dept Childhood & Dev Med, Pediat Rheumatol Unit, Milan, Italy

[19] Hacettepe Univ, Fac Med, Dept Pediat Rheumatol, Ankara, Turkiye

[20] Univ Siena, Dept Mol Med & Dev, Clin Pediat, Siena, Italy

[21] Azienda Ospedaliero Univ Senese, European Reference Network ERN Rare Immunodeficien, Siena, Italy

[22] Univ Bari, Giovanni Pediat Hosp 23, Dept Pediat, Bari, Italy

[23] Inst Nacl Ciencias Med & Nutr Salvador Zubiran, Dept Ophthalmol, Mexico City, Mexico

[24] Gazi Univ Hosp, Dept Internal Med, Div Rheumatol, Ankara, Turkiye

[25] Univ Siena, Dept Med Surg & Neurosci, Ophthalmol Unit, Siena, Italy

[26] Univ Childrens Hosp, Univ Ljubljana, Dept Allergol Rheumatol & Clin Immunol, Ljubljana, Slovenia

[27] Univ Med Ctr Ljubljana, European Reference Network ERN Rare Immunodeficie, Ljubljana, Slovenia

[28] Univ Barcelona, Hosp Clin Barcelona, European Reference Network ERN Rare Immunodeficie, Inst Invest Biomed August Pi i Sunyer IDIBAPS, Barcelona, Spain

来源：

OPHTHALMOLOGY AND THERAPY | 2024年 / 13卷 / 06期

关键词：

Autoinflammatory diseases; Behcet's disease; Paediatric ophthalmology; Rare disease registries; Retinal vasculitis; Uveitis; PEDIATRIC BEHCETS-DISEASE; CLINICAL-FEATURES; ONSET; UVEITIS; STANDARDIZATION; ASSOCIATION; CRITERIA; HLA-B51; RISK; AGE;

D O I：

10.1007/s40123-024-00916-z

中图分类号：

R77 [眼科学];

学科分类号：

100212 ;

摘要：

Introduction: This study aims to characterize ocular manifestations of juvenile Beh & ccedil;et's disease (jBD). Methods: 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. Results: 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) mu m, 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 degrees) 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. Conclusions: The results of this study may be leveraged to guide clinical practice and future research on this rare sight-threatening condition.

引用

页码：1479 / 1498

页数：20

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