韦格纳肉芽肿病延误诊断原因分析——兼论其诊断标准的局限性

The analysis for the causes of delaying diagnosis of Wegener's granulomatosis-discussing limitations of diagnostic criteria for WG

目的分析韦格纳肉芽肿(WG)延误诊断的原因以期提高本病的诊断水平。方法通过计算机系统网上查询对北京大学人民医院1997—2007年住院收治并确诊为WG病的病例,同时查阅2003—2007年《中华风湿病学杂志》和《临床误诊误治杂志》中关于WG病误诊病例,进行回顾性分析。结果WG病可累及多个系统和器官,主要侵犯肺脏、肾脏及上呼吸道,也可侵犯耳部、眼部、腮腺、口腔等部位,首发症状及临床表现中有很大一部分为非三联征,易导致误诊误治。其c-ANCA阳性率高,病理表现多为坏死性肉芽肿炎症、炎细胞浸润的血管炎。结论WG的临床表现复杂多样,为多系统或器官损伤的症候群,症状不典型时易误诊,尤其是早期阶段。我们建议对WG病诊断标准进行必要的修改,以期做到早期诊断,早期治疗。

Objective To analyse the cause for delaying diagnosis of WG and improve the diagnosis level. Methods A retrospective study was done on patients who were admitted to the People＇s Hospital of Peking University and were diagnosed with WG from 1997 to 2007 ,by searching the computer system of the hospital for patients with WG,who were misdiagnosed and reported in Chinese Journal of Rheumatology and Clinical Misdiagnosis ＆ Mistherapy from 2003 to 2007. Results WG included multiple systems and organs ivolvement. The lung, kidney and upper airway were the most com- monly involved organs, and ear, eye, external salivary gland and oral cavity were also involved. Most initial symptom and clinical manifestation were not typical manifestation of WG trilogy, which led to misdiagnosis and mistreatment. Anti-neurophil cytoplasmic antibody （ANCN）positive rate was high. The most common pathologic features were necrotizing granulomatous inflammation and vasculitis. Conclusion Clinical manifestation of WG is complicated and it injures multiple system or organs. When the symptoms are atypical, it is easy to lead to misdiagnosis, especially in early stage. We suggest running requisite revision for diagnostic criteria of WG,in order to make early diagnosis and treatment of WG.