系统性小血管炎临床分析

目的总结系统性小血管炎的临床表现及其转归。方法对42例确诊为系统性小血管炎患者的临床资料进行回顾性分析。其中男24例,女18例;平均发病年龄（60.3±15.4）岁,60岁以上者共27例,占64.3%。分析其临床表现、实验室检查、影像学检查、治疗方法、疗效、并发症及转归等。结果发热在首诊症状以及主要临床表现中均占首位,分别占64.3%和85.7%,其它显著的症状包括乏力、消瘦等非特异性症状。最常受累的器官是肺和肾脏,主要症状分别为咳嗽、痰血、咯血和气急,占88.1%,以及蛋白尿和血尿,占81.0%;其中肾功能减退的患者占40.5%。92.9%的患者有3个以上脏器受累。35例患者血清抗中性粒细胞浆抗体（ANCA）阳性,其中核周型ANCA阳性24例,胞浆型ANCA阳性11例;4例血清ANCA阴性;3例无ANCA资料。94.6%的患者对于免疫抑制治疗敏感,完全缓解率和部分缓解率分别为37.8%和56.8%。共6例患者死亡,其中5例死于多器官功能障碍综合征合并严重感染,1例为急性消化道大出血。结论系统性小血管炎是一种以老年人群为主要发病对象的系统性疾病,大多数患者有全身症状,受累脏器多,诊断困难,借助ANCA或组织学检查可使多数患者得到确诊。绝大多数患者都对免疫抑制治疗反应良好,感染是主要死因之一。

Clinical analysis of systemic small-vessel vasculitis

Objective To summarize the clinical manifestations and outcome of patients diagnosed with systemic small vessel vasculitis. Methods The clinical data of 42 patients with systemic small-vessel vasculitis[24 male/18 female, the average age was（ 60.3 ±15.4）years]were retrospectively studied. 27 of these patients aged over 60 years （ account for 64.3 % ）. Their clinical presentations, laboratory data, imaging finding, therapies, complications, curative effects and prognosis were analyzed. Results Fever ranked the first symptom in either initial or major clinical presentations（64.3 % and 85. 7 %, respectively）. There were also noticeable nonspecifie symptoms like fatigue and body weight loss as well. The most organs frequently involved were lungs （common symptoms included cough, hemoptysis,and dyspena, which accounted for 88. 1%）and kidney（renal involvement was defined by proteinuria/ hematuria, which accounted for 81.0 % ）. Renal insufficiency was present in 40.5 % of all the patients. Totally 92.9 % of the patients had simultaneously over three organ systems involved. Antineutrophil cytoplasmic antibodies（ANCA） positivity was found for 35 patients（24 for perinuclear ANCA and 11 for cytoplasmic ANCA）;while 4 patients presented negative. The other 3 patients had no record for ANCA. 94. 5% patients responded sensitively to immunosuppressive therapy（complete and partial response rate was 37.80% and 56.8 %, respectively）. Six patients died and 5 of them were died of multiple organ dysfunction syndromes（MODS）along with severe infection and 1 for acute massive hemorrhage of gastrointestinal tract. Conclusion Systemic small-vessel vasculitis is an elderly predominance disease with the majority patients had constitutional symptoms. Although variable organ involvements make diagnosis a challenge,it could be confirmed through the tool of ANCA and/or histology. Most patients respond well to immunosuppressive therapy. Infection is the majority cause of their death.