Dabrowski A, Droszcz W
Department of General Medicine and Pulmonology, Medical University, ul. Banacha 1, Warsaw, Poland.
Med Sci Monit. 2000 Jan-Feb;6(1):151-7.
In observed, during 15 years group, of 18 patients with established Wegener's granulomatosis WG diagnosis, important is period of time from onset of the manifestations to WG diagnosis--very diversified, between 3 to 96 months and heterogenerosity of clinical manifestations during establishing of the diagnosis. Separated from clinical and histopathological features in most of the patients the presence of the titre of antineutrophil cytoplasmic antibody (ANCA) in blood serum examinations was established--75% among 16 observed patients in whom such examination was possible to perform. There was no significant correlation between ANCA titre and prognosis of the WG course. Most of the patients developed upper respiratory tract, ears, lungs and kidney disease involvement. Cyclophosphamide (CF) and prednisolone (P) treatment improves clinical remission in most of the patients (88.8%) and possibility of the course of the disease control. However often recurrences are observed (50%) and side effects due to the long term CF and P treatment (60%). 38.8% of patients achieve long term remission of the manifestations. 3 deaths of WG patients were observed during that period. Patients require during treatment close collaboration and careful periodical control of treatment.
在观察的15年组中，18例已确诊韦格纳肉芽肿（WG）的患者，从症状出现到确诊WG的时间间隔很重要，差异很大，为3至96个月，且确诊过程中临床表现具有异质性。在大多数患者中，除了临床和组织病理学特征外，血清检查中还发现了抗中性粒细胞胞浆抗体（ANCA）滴度，在16例可进行此类检查的观察患者中，75%检测到该抗体。ANCA滴度与WG病程的预后之间无显著相关性。大多数患者出现上呼吸道、耳部、肺部和肾脏疾病受累。环磷酰胺（CF）和泼尼松龙（P）治疗可使大多数患者（88.8%）临床缓解，并有可能控制疾病进程。然而，常观察到复发（50%）以及长期CF和P治疗引起的副作用（60%）。38.8%的患者实现了症状的长期缓解。在此期间观察到3例WG患者死亡。患者在治疗期间需要密切合作并进行仔细的定期治疗监测。
