Clinical rheumatology · 2021
Juvenile Behçet's disease: a tertiary center experience.
СтатьяНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Ekici Tekin Zahide, Çelikel Elif, Aydin Fatma, Kurt Tuba, Sezer Müge, Tekgöz Nilüfer, Karagöl Cüneyt, Coşkun Serkan, Kaplan Melike Mehveş, Çelikel Acar Banu
Аннотация
OBJECTIVES: Juvenile Behçet's disease is a rare and severe disease of childhood characterized by a chronic inflammatory vasculitis. The aim of the present study is reporting demographic, clinical and therapeutic outcomes of juvenile Behçet's disease in a tertiary center.
METHODS: The retrospective study included patients who were diagnosed Behçet's disease before 16 years. The demographic and clinical features, and diagnostic and therapeutic strategies of patients were recorded.
RESULTS: Seventy-two patients with jBD were included in this study; 32 were male (44.4%). Thirty (41.7%) patients had BD cases in their family. We observed oral ulceration (100%), genital ulceration (68.1%), joint involvement (36.1%) and cutaneous manifestations (34.7%) as the most common clinical findings, respectively. As severe organ involvements, 20.8% ocular, 18.1% vascular and 15.3% neurologic pathologies were seen. All patients had colchicine. Corticosteroid (20.8%) was used to treat severe cases and acute attacks. Azathioprine (23.6%) was the main immunosuppressive agent and cyclophosphamide (8.3%) was applied initially for life-threatening conditions with pulse methylprednisolone.
CONCLUSION: In this cohort, the prevalence of genital ulceration and family history was high, and we observed less ocular involvement, a few permanent neurological morbidities and no death. Key Points • In the present study, there were acceptable permanent neurological involvements as morbidity and no mortality. • It is important noticing and managing jBD in early phase in order to prevent the devastating results. • The awareness of jBD provides timely treatment of patients. • The positivity of family history and HLA B51 should alert the clinician about the incomplete cases.
METHODS: The retrospective study included patients who were diagnosed Behçet's disease before 16 years. The demographic and clinical features, and diagnostic and therapeutic strategies of patients were recorded.
RESULTS: Seventy-two patients with jBD were included in this study; 32 were male (44.4%). Thirty (41.7%) patients had BD cases in their family. We observed oral ulceration (100%), genital ulceration (68.1%), joint involvement (36.1%) and cutaneous manifestations (34.7%) as the most common clinical findings, respectively. As severe organ involvements, 20.8% ocular, 18.1% vascular and 15.3% neurologic pathologies were seen. All patients had colchicine. Corticosteroid (20.8%) was used to treat severe cases and acute attacks. Azathioprine (23.6%) was the main immunosuppressive agent and cyclophosphamide (8.3%) was applied initially for life-threatening conditions with pulse methylprednisolone.
CONCLUSION: In this cohort, the prevalence of genital ulceration and family history was high, and we observed less ocular involvement, a few permanent neurological morbidities and no death. Key Points • In the present study, there were acceptable permanent neurological involvements as morbidity and no mortality. • It is important noticing and managing jBD in early phase in order to prevent the devastating results. • The awareness of jBD provides timely treatment of patients. • The positivity of family history and HLA B51 should alert the clinician about the incomplete cases.