英文摘要 |
Objective: Wegener's granulomatosis (WG) is a rare disease in Taiwan and data on the disease course and outcome is limited. We analyzed the clinical characteristics of patients with WG in Taiwan in order to facilitate improved case recognition and management in the future. Methods: A retrospective study was conducted by chart review. Patients with WG diagnosed between 1985 and 2005 at Taichung Veteran's General Hospital were included. Their clinical features, disease course, and laboratory findings were analyzed. Results: The patient group consisted of 5 males and 7 females. The mean age at diagnosis was 55.4 years. The most common manifestation at diagnosis was rhinosinusitis (100%). During follow-up, the most common involved organ/system was the upper respiratory tract (100%), followed by lungs (75%) and kidneys (50%). Serum antineutrophil cytoplasmic antibodies (ANCA) were assessed in 11 of the 12 patients, and were positive in all patients with a cytoplasmic pattern. All 12 patients achieved at least partial remission after treatment, but 58% of patients (7/12) experienced one or more relapses. The overall mortality rate was 50%, and the leading cause of death was bacterial pneumonia. Conclusion: WG in Taiwan usually affects middle-aged adults, and nearly always involves the upper respiratory tract in the early stage of disease. Patients in this study are characterized by high morbidity and mortality, as well as high rates of seropositivity for cytoplasmic-AN CA. A high index of suspicion is needed in diagnosis of this rare and complex disease. In patients with clinical features and pathological findings that are highly suggestive of WG, seropositivity for cytoplasmic-ANCA could help support the diagnosis. |