PausJenssen Elizabeth S, Cockcroft Donald W
Division of Pulmonary Medicine, University of Saskatchewan, Saskatoon, Saskatchewan, Canada.
Ann Allergy Asthma Immunol. 2003 Jul;91(1):82-5. doi: 10.1016/S1081-1206(10)62064-9.
Wegener granulomatosis is a systemic vasculitis classically described as involving the upper and lower respiratory tracts together with glomerulonephritis. Its poor prognosis was marginally improved with the use of corticosteroids, and long-term remission was not achieved until the introduction of cytotoxic agents. Generalized or systemic Wegener granulomatosis has a worse prognosis than those limited to the respiratory tracts.
To report the case of an individual who we suspect has a 30-year history of Wegener granulomatosis treated only with prednisone.
A literature search was performed with PubMed using the keywords Wegener granulomatosis, survival, prognosis, and treatment.
Our patient is alive 32 years after her initial symptoms of vasculitis. She has been taking daily prednisone only for the majority of this time. Our clinical diagnosis of Wegener granulomatosis is based on the history and very high antineutrophil cytoplasmic antibody with the cytoplasmic pattern.
There may be variants of generalized Wegener granulomatosis that survive with less aggressive treatment.
韦格纳肉芽肿是一种系统性血管炎，经典描述为累及上、下呼吸道以及肾小球肾炎。使用皮质类固醇后其较差的预后仅略有改善，直到引入细胞毒性药物才实现长期缓解。广泛性或系统性韦格纳肉芽肿的预后比局限于呼吸道的情况更差。
报告一例我们怀疑有30年韦格纳肉芽肿病史且仅用泼尼松治疗的病例。
使用PubMed以韦格纳肉芽肿、生存、预后和治疗为关键词进行文献检索。
我们的患者在出现血管炎初始症状32年后仍然存活。在这段时间的大部分时间里，她仅每日服用泼尼松。我们对韦格纳肉芽肿的临床诊断基于病史以及非常高的胞浆型抗中性粒细胞胞浆抗体。
可能存在广泛性韦格纳肉芽肿的变异型，通过不太积极的治疗也能存活。
