Pediatric Behçet’s disease - clinical aspects and current concepts

Yıldız, Mehmet; Köker, Oya; Adroviç, Amra; Şahin, Sezgin; Barut, Kenan; Kasapçopur, Özgür

doi:10.5152/eurjrheum.2019.19121

Cited by 24 publications

(32 citation statements)

References 112 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The pathergy phenomenon is a nonspecific hypersensitivity reaction to trauma. It can be performed by puncturing the flexor aspect of forearm skin with a 20-gauge needle, and the test is considered positive if an indurated erythematous pustule develops at the site of trauma within 24 to 48 h ( 1 ). It should be kept in mind that the positive pathergy test is not pathognomonic to BD.…”

Section: Clinical Findingsmentioning

confidence: 99%

“…Although BD can affect all sizes and types of vessels, venous system involvement is more common during the course of the disease ( 1 ). Vascular involvement in children is reported as 1.8–21%, and the most common vascular involvement in BD is lower extremity venous thrombosis ( 21 , 58 , 65 , 68 , 72 ).…”

Section: Clinical Findingsmentioning

confidence: 99%

“…Behçet's disease, which is a multi-systemic vasculitis that can involve vessels of all sizes and types, was first described by Hulusi Behçet with the triad of oral aphthae, genital aphthae, and uveitis ( 1 ). While the term of “pediatrics BD” describes cases diagnosed in childhood, “juvenile BD” refers to cases that were diagnosed in adulthood but whose first symptoms started before the 16 years of age ( 68 ).…”

Section: Definitions and Classification Criteriamentioning

confidence: 99%

“…Behçet's Disease (BD) is a systemic vasculitis with unique geographic distribution around the historical silk road. It is firstly defined as a disease causing recurrent oral and genital aphthae with uveitis by Hulusi Behçet ( 1 ). With the expanding knowledge about the genetic basis, etiopathogenesis and clinical findings, now we all know that BD has more extensive findings than the definition of Hulusi Behçet.…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Pediatric Behçet's Disease

et al. 2021

Self Cite

View full text Add to dashboard Cite

Behçet's Disease (BD) is a systemic vasculitis firstly described as a disorder causing aphthous lesion in oral and genital mucosae and uveitis. The disease has an extremely unique distribution characterized by the highest incidence in communities living along the historical Silk road. Although our understanding of the etiopathogenesis of BD has expanded over time, there are still lots of unidentified points in the underlying mechanisms of the disease. The accepted opinion in the light of the current knowledge is that various identified and/or unidentified infectious and/or environmental triggers can take a role as a trigger in individuals with genetic susceptibility. Although the disease usually develops in young adulthood, it is reported that about 15–20% of all Behçet's patients develop in childhood. Pediatric BD differs from adult BD not only with the age of onset but also in the frequency and distribution of clinical findings, disease severity and outcome. While gastrointestinal system involvement, neurological findings, arthralgia and positive family history are more common in children, genital lesions and vascular lesions are more common in adult patients. In addition, a better disease outcome with lower severity score and activity index has been reported in children. The diagnosis of the disease is made according to clinical findings. It can be challenging to diagnose the disease due to the absence of a specific diagnostic test, and the long time interval from the first finding of the disease to the full-blown disease phenotype in pediatric cases. Therefore, many classification criteria have been proposed so far. The widely accepted ones are proposed by the International Study Group. The new sets of classification criteria which is the only one for pediatric BD were also developed for pediatric cases by the PEDBD group. The primary goal for the treatment is preventing the organ damages by suppressing the ongoing inflammation and forestalling the disease flares. The treatment of the BD can be onerous due to its multisystemic nature and a multidisciplinary approach is essential for the management of the patients. In this review article, the definition, clinical findings, epidemiology, etiopathogenesis, and treatment will be discussed.

show abstract

Section: Clinical Findingsmentioning

confidence: 99%

Section: Clinical Findingsmentioning

confidence: 99%

Section: Definitions and Classification Criteriamentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Pediatric Behçet's Disease

et al. 2021

Self Cite

View full text Add to dashboard Cite

show abstract

“…La enfermedad de Behçet (EB) es una vasculitis sistémica que puede afectar a venas y arterias de diferente calibre [1][2][3] . En clínica suele manifestarse con úlceras orales y genitales recurrentes, en relación con síntomas oculares.…”

Section: Introductionunclassified

Enfermedad de Behçet en pediatría. Experiencia en un hospital de tercer nivel

Giacaman¹,

Mir-Perello²,

Lpez-Garca³

et al. 2021

MCUT

View full text Add to dashboard Cite

Introducción: El diagnóstico de la enfermedad de Behçet (EB) es clínico y en los niños puede ser un desafío dadas la baja prevalencia y la aparición insidiosa de los síntomas, lo que lleva en la mayor parte de los casos a un retraso del diagnóstico. Objetivo: Describir las características clínicas de los pacientes menores de 16 años con sospecha de EB en seguimiento en un hospital de tercer nivel. Material y métodos: Se realizó un estudio descriptivo de corte transversal que incluyó a todos los pacientes bajo seguimiento por sospecha de

show abstract

Childhood Cerebral Vasculitis

et al. 2022

View full text Add to dashboard Cite

Cerebral vasculitis is increasingly recognized as a common cause of pediatric arterial stroke. It can present with focal neurological deficits, psychiatric manifestations, seizures, and encephalopathy. The etiopathogenesis of childhood cerebral vasculitis (CCV) is multifactorial, making an inclusive classification challenging. In this review, we describe the common and uncommon CCV with a comprehensive discussion of etiopathogenesis, the role of various imaging modalities, and advanced techniques in diagnosing CCV. We also highlight the implications of relevant clinical, laboratory, and genetic findings to reach the final diagnosis. Based on the clinicoradiological findings, a stepwise diagnostic approach is proposed to facilitate CCV diagnosis and rule out potential mimics. Identification of key clinical manifestations, pertinent blood and cerebrospinal fluid results, and evaluation of central nervous system vessels for common and disease-specific findings will be emphasized. We discuss the role of magnetic resonance imaging, MR angiography, and vessel wall imaging as the imaging investigation of choice, and reservation of catheter angiography as a problem-solving tool. We emphasize the utility of brain and leptomeningeal biopsy for diagnosis and exclusion of imitators and masqueraders.

show abstract

Pediatric Behçet’s disease - clinical aspects and current concepts

Cited by 24 publications

References 112 publications

Pediatric Behçet's Disease

Pediatric Behçet's Disease

Enfermedad de Behçet en pediatría. Experiencia en un hospital de tercer nivel

Childhood Cerebral Vasculitis

Contact Info

Product

Resources

About