Imaging pearls of pediatric Behçet’s disease

Topçuoğlu, Osman Melih; Topcuoğlu, Elif Dilara; Altay, Cetin Murat; Genç, Sinan

doi:10.1016/j.ejrad.2017.06.016

Cited by 9 publications

(19 citation statements)

References 50 publications

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“…L a enfermedad de Behçet (EB) es una patología inflamatoria crónica, recurrente y multisistémica, incluida dentro de las vasculitis, de etiología no precisada [1][2][3][4][5][6][7][8][9] . Se caracteriza por comprometer vasos de tamaño grande, mediano y pequeño afectando tanto el territorio arterial como el venoso [1][2][3][4][5][6][7] .…”

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“…Esta enfermedad se ha descrito principalmente en países mediterráneos, con mayor incidencia en Turquía [1][2][3][4][5][6][7][8][9][10][11][12] . En Chile es muy infrecuente, existiendo a la fecha sólo 51 casos publicados [8][9][10][11] .…”

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“…En Chile es muy infrecuente, existiendo a la fecha sólo 51 casos publicados [8][9][10][11] . Suele presentarse entre los 20-30 años, sin predominio por sexos, describiéndose en los hombres una presentación más precoz y severa [1][2][3][4][5][6][7]12 .…”

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“…El diagnóstico de la EB se realiza por sus ma-nifestaciones clínicas, debido a que no existen marcadores genéticos ni de laboratorio específicos [1][2][3][4][5]7 . Las lesiones más características son la aparición recurrente de úlceras aftosas orales y genitales asociado a compromiso cutáneo y ocular, con uveítis a repetición [1][2][3][4][5][7][8][9][10] . En el 2014, el Grupo Internacional de Estudio de la Enfermedad de Behçet agregó a los criterios diagnósticos clásicos el compromiso vascular y neurológico, obteniendo una sensibilidad y especificidad de 96% y 91%, respectivamente 1,5,7 .…”

unclassified

“…Las lesiones más características son la aparición recurrente de úlceras aftosas orales y genitales asociado a compromiso cutáneo y ocular, con uveítis a repetición [1][2][3][4][5][7][8][9][10] . En el 2014, el Grupo Internacional de Estudio de la Enfermedad de Behçet agregó a los criterios diagnósticos clásicos el compromiso vascular y neurológico, obteniendo una sensibilidad y especificidad de 96% y 91%, respectivamente 1,5,7 . Estos dos últimos sistemas se ven afectados con mayor frecuencia en países occidentales, permitiendo con su incorporación a los criterios, facilitar el diagnóstico en zonas de baja incidencia de EB 8,9,12 .…”

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Hemoptisis como manifestación inicial de la enfermedad de Behçet. Casos clínicos

Abarca

Santamarina²,

Vergara

et al. 2018

Rev. méd. Chile

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Arterial involvement in Behçet's disease (BD) is less common than venous lesions. The most commonly affected arteries are: the aorta, lower extremity arteries, mesenteric, femoral, coronary, renal, subclavian and pulmonary arteries. The rupture of pulmonary arteries is the main cause of death of patients with EB and the presence of aneurysms is a bad prognostic factor. We report two patients with arterial involvement in BD. A 14 years old male presenting with hemoptysis lasting three days. A chest computed tomography showed an aneurismal dilatation of the right interlobar artery, bilateral intramural thrombi and alveolar hemorrhage. A right lobar pulmonary resection was performed but hemoptysis recurred. Suspecting a BD, prednisone was started and hemoptysis subsided. A 42 years old male presenting with chest pain and hemoptysis. A chest CT scan showed thrombi in pulmonary veins and anticoagulant therapy was started. Two months later he was admitted again due to a massive hemoptysis. The CT scan showed aneurisms in pulmonary arteries, mural thrombi and pulmonary infarction. With the diagnosis of BD, methylprednisolone, followed by prednisone and cyclophosphamide were used, with a good clinical response.

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Hemoptisis como manifestación inicial de la enfermedad de Behçet. Casos clínicos

Abarca

Santamarina²,

Vergara

et al. 2018

Rev. méd. Chile

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Childhood Cerebral Vasculitis

et al. 2022

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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.

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Pediatric rheumatology in Turkey

Adroviç

Kasapçopur

2019

Rheumatol Int

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Imaging pearls of pediatric Behçet’s disease

Cited by 9 publications

References 50 publications

Hemoptisis como manifestación inicial de la enfermedad de Behçet. Casos clínicos

Hemoptisis como manifestación inicial de la enfermedad de Behçet. Casos clínicos

Childhood Cerebral Vasculitis

Pediatric rheumatology in Turkey

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