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Juvenile angio-Behçet’s disease: report and brain MRI findings of 3 cases
Abstract
Background: Behçet’s Disease (BD) is a vasculitis of unknown origin; it is characterized by recurrent mouth and genital ulcerations, uveitis and diverse systemic manifestations. It is very rare in children. Vascular tropism is mainly characterized by phlebothrombosis; arterial involvement is less frequent.
Case presentations: We report here three cases of juvenile angio-Behçet in two boys aged 11 and 16 years-old and a 14 year-old girl. All three children were admitted for a newly-diagnosed BD characterized by multiple, migrating and recurring phlebothromboses, treated with anticoagulants and corticosteroids and requiring cyclophosphamide pulses, along with a severe uveitis in one patient, having required the addition of azathioprine, with favorable outcome. Complications such as pulmonary embolism and Budd-Chiari syndrome were present in case 3, which improved under immunosuppressants. In order to prevent future thrombosis, anticoagulants were maintained for long periods as well as imunosuppressants. Magnetic Resonance Imaging (MRI) of the brain revealed subclinical findings in the 3 cases.
Conclusions: Development of venous thrombosis in juvenile BD cases should not be overlooked and special attention is required for these cases in order to improve their disease outcome. Performing advanced radiologic investigations is useful to detect subclinical cases and delineate the extent of affection. Prognosis remains variable but often bad, depending on the presence of vascular, ocular and neurological complications.
Keywords: Juvenile angio-Behçet, Phlebothrombosis, MRI brain, Rare disease, Immunosuppressants
Case presentations: We report here three cases of juvenile angio-Behçet in two boys aged 11 and 16 years-old and a 14 year-old girl. All three children were admitted for a newly-diagnosed BD characterized by multiple, migrating and recurring phlebothromboses, treated with anticoagulants and corticosteroids and requiring cyclophosphamide pulses, along with a severe uveitis in one patient, having required the addition of azathioprine, with favorable outcome. Complications such as pulmonary embolism and Budd-Chiari syndrome were present in case 3, which improved under immunosuppressants. In order to prevent future thrombosis, anticoagulants were maintained for long periods as well as imunosuppressants. Magnetic Resonance Imaging (MRI) of the brain revealed subclinical findings in the 3 cases.
Conclusions: Development of venous thrombosis in juvenile BD cases should not be overlooked and special attention is required for these cases in order to improve their disease outcome. Performing advanced radiologic investigations is useful to detect subclinical cases and delineate the extent of affection. Prognosis remains variable but often bad, depending on the presence of vascular, ocular and neurological complications.
Keywords: Juvenile angio-Behçet, Phlebothrombosis, MRI brain, Rare disease, Immunosuppressants
