系统性小血管炎诊断情况调查

Investigation of current diagnostic situation of systemic small-vessel vasculitis in general hospital

目的了解综合性医院中系统性小血管炎的诊断现状,探讨该病确诊困难的原因。方法对本院确诊为系统性小血管炎的42例患者进行回顾,其中,男24例,女18例。分析其发病年龄、首发症状、疾病发展过程、从症状的发生到确诊所花费的时间及误诊情况等。结果患者发病年龄24～82岁,平均(60.3±15.4)岁,其中60岁以上者共27例。从首发症状到确诊时间7d～10年,中位数13个月。39例以各类待查诊断入院,13例在首次入院期间即获确诊,均为开展ANCA检测后确诊的患者;25例延迟诊断;4例属于误诊。39例进行了血清抗中性粒细胞浆抗体(ANCA)检测,其中35例ANCA阳性,包括核周型ANCA(P-ANCA)阳性24例和胞浆型ANCA(C-ANCA)阳性11例;4例ANCA阴性。20例有病理资料,17例呈阳性结果;其中3例在不同器官取材,3例在同一器官重复活检或手术。临床表现几乎涵盖了所有系统,确诊本病的前3个专科分别是肾内科、呼吸科和消化科。结论系统性小血管炎是一组好发于老年人的涉及多脏器的慢性疾病。血管炎的严重程度、活动性、病变部位等均可能影响其诊断的确立,误诊率较高。除临床表现外,ANCA和组织病理学检查以及临床医生的认知度是提高诊断率的关键。

Objective To investigate the current diagnostic situation of systemic small vessel vasculitis in gengral hospital,and to provide insight on the facts which might contribute to the diagnostic difficulties. Methods Data of all 42 patients[24 male/18 female, the average age was（60.3 ±15.4）years-lwhose accurate diagnosis was systemic small vessel vasculitis in this hospital were retrospectively studied. Their age of onset,initial presentations, misdiagnosis and the duration time of establishing the diagnosis were analyzed. Results The age of onset ranged from 24 to 82 years（mean 60. 3±15. 4 years）. Twenty-seven of these patients aged over 60 years. Most of them passed through a difficult period before diagnosis since the duration from symptom onset to diagnosis can range from 1 week to 10 years（median 13 months）. Thirty-nine patients were diagnosed as unknown origin diseases at the beginning of their admission. Thirteen patients obtained confirmation during their first hospitalization, all of them had admitted since ANCA program was launched. Four patients were misdiagnosed,and 25 cases were delayed diagnosis. Thirty-five patients out of 39 patients were found antineutrophil cytoplasmic antibodies（ANCA）positive（24 for perinuclear ANCA and 11 for cytoplasmic ANCA） ,while 4 patients present negative. Seventeen out of 20 patients got positive histopathological evidences. Three patients got tissues from different organs and another 3 patients underwent repeated biopsy or operations on the same organs. Their clinical manifestations almost covered all systems. The top three departments that often confirmed the diagnosis were department of nephrology, respiratory and gastroenterology, respectively. Conclusion The systemic small vessel vasculitis is a group of chronic multisystem disorders that mainly attacked elderly and usually involves an extended span of duration. The diagnosis might be influenced by the severity,activity,location of the damages. And the misdiagnosis rate is high. Despite the clinical findings,improvement of diagnosis relies on both ANCA test,histopathological examination and the cognition of clinicians.