Figueiredo Sandra, Leal Laurentino Mendes, Morais António, Magalhães Adriana, Oliveira Teresa, Hespanhol Venceslau, Dias Carlos, Fernandes Gabriela
Serviço de Pneumologia, Hospital de São João (EPE), Porto.
Rev Port Pneumol. 2009 Sep-Oct;15(5):929-35. doi: 10.1016/s0873-2159(15)30187-2.
Wegener granulomatosis is a rare systemic idiopathic disease characterized by involvement of small vessels - medium and small arteries, venules, arterioles and ocasionally large arteries. This disease has predilection for the upper and lower respiratory tract and the kidney, with granulomatous inflamation and necrosis. Clinical manifestations and organ involvement of the disease vary widely. Early diagnosis and treatment may lead to a full recovery. Without treatment, Wegener's granulomatosis can be fatal. The authors present a case of a 33 year-old female, with severe disease, but with good outcome, after adequate diagnosis and treatment.
韦格纳肉芽肿病是一种罕见的系统性特发性疾病,其特征是小血管受累——中小动脉、小静脉、小动脉,偶尔也累及大动脉。这种疾病好发于上、下呼吸道和肾脏,伴有肉芽肿性炎症和坏死。该疾病的临床表现和器官受累情况差异很大。早期诊断和治疗可能会实现完全康复。未经治疗,韦格纳肉芽肿病可能会致命。作者报告了一例33岁女性患者,病情严重,但经过充分诊断和治疗后预后良好。